Latest revision as of 01:41, 26 June 2024

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Section editor
	Sanjai Sharma, MD Sequoia Regional Cancer Center Visalia, CA, USA LinkedIn

12 regimens on this page 17 variants on this page

Guidelines

Given the rapid change in evidence in many areas of hematology/oncology, readers are encouraged to consider any guideline published 5+ years ago to be for historical purposes, only.

ESMO

2015: Robak et al. Hairy cell leukaemia: ESMO Clinical Practice Guidelines for diagnosis, treatment and follow-up PubMed

Hairy Cell Leukemia Foundation

2017: Grever et al. Consensus guidelines for the diagnosis and management of patients with classic hairy cell leukemia PubMed

NCCN

NCCN Guidelines - Hairy Cell Leukemia
- 2017: Wierda et al. Hairy Cell Leukemia, Version 2.2018, NCCN Clinical Practice Guidelines in Oncology PubMed

Untreated

Cladribine monotherapy

Regimen variant #1, 0.09 mg/kg/day x 7 days

Study	Dates of enrollment	Evidence
Saven et al. 1998	1986-1993	Non-randomized (RT)

Chemotherapy

Cladribine (Leustatin) 0.09 mg/kg/day IV continuous infusion over 7 days, started on day 1 (total dose: 0.63 mg/kg)

7-day course

Regimen variant #2, 0.1 mg/kg/day x 7 days

Study	Dates of enrollment	Evidence
Piro et al. 1990	1986-1989	Pilot, fewer than 20 pts
Tallman et al. 1996	1991-1995	Phase 2

Chemotherapy

Cladribine (Leustatin) 0.1 mg/kg/day IV continuous infusion over 7 days, started on day 1 (total dose: 0.7 mg/kg)

7-day course

Regimen variant #3, 0.12 mg/kg/day x 5 days

Study	Dates of enrollment	Evidence	Comparator	Comparative Efficacy
Robak et al. 1999	1991-1997	Phase 2
Robak et al. 2007	1998-2005	Phase 3 (C)	Cladribine; weekly	Did not meet primary endpoint of CR rate

Chemotherapy

Cladribine (Leustatin) 0.12 mg/kg IV over 2 hours once per day on days 1 to 5

5-day course

Regimen variant #4, 0.14 mg/kg/day x 5 days

Study	Dates of enrollment	Evidence	Comparator	Comparative Efficacy
Zenhäusern et al. 2009 (SAKK 32/98)	1998-2005	Phase 3 (C)	Cladribine; weekly	Did not meet primary endpoint of average leukocyte count within 6 weeks from randomization

Chemotherapy

Cladribine (Leustatin) 0.14 mg/kg SC once per day on days 1 to 5

5-day course

Regimen variant #5, 0.15 mg/kg/day x 5 days

Study	Dates of enrollment	Evidence
Damasio et al. 1998	NR in abstract	Phase 2

Chemotherapy

Cladribine (Leustatin) 0.15 mg/kg IV over 2 hours once per day on days 1 to 5

5-day course

References

Piro LD, Carrera CJ, Carson DA, Beutler E. Lasting remissions in hairy-cell leukemia induced by a single infusion of 2-chlorodeoxyadenosine. N Engl J Med. 1990 Apr 19;322(16):1117-21. link to original article contains dosing details in abstract PubMed
Tallman MS, Hakimian D, Rademaker AW, Zanzig C, Wollins E, Rose E, Peterson LC. Relapse of hairy cell leukemia after 2-chlorodeoxyadenosine: long-term follow-up of the Northwestern University experience. Blood. 1996 Sep 15;88(6):1954-9. link to original article contains dosing details in manuscript PubMed
1. Update: Chadha P, Rademaker AW, Mendiratta P, Kim B, Evanchuk DM, Hakimian D, Peterson LC, Tallman MS. Treatment of hairy cell leukemia with 2-chlorodeoxyadenosine (2-CdA): long-term follow-up of the Northwestern University experience. Blood. 2005 Jul 1;106(1):241-6. Epub 2005 Mar 10. link to original article contains dosing details in abstract PubMed content property of HemOnc.org
Damasio EE, Resegotti L, Masoudi B, Bruni R, Cerri R, Isaza A, Clavio M, Risso M, Rossi E, Spriano M, Truini M; Italian Group for the Hairy Cell Leukemia. Five day intermittent vs seven day continuous 2-chlorodeoxyadenosine infusion for the treatment of hairy cell leukemia: a study by Italian Group for the Hairy Cell Leukemia. Recenti Prog Med. 1998 Feb;89(2):68-73. PubMed
Saven A, Burian C, Koziol JA, Piro LD. Long-term follow-up of patients with hairy cell leukemia after cladribine treatment. Blood. 1998 Sep 15;92(6):1918-26. link to original article PubMed
Robak T, Błasińska-Morawiec M, Błoński J, Hellmann A, Hałaburda K, Konopka L, Kotlarek-Haus S, Potoczek S, Hansz J, Dmoszyńska A, Urasiński I, Zdziarska B, Dwilewicz-Trojaczek J, Hołowiecki J, Skotnicki AB. 2-chlorodeoxyadenosine (cladribine) in the treatment of hairy cell leukemia and hairy cell leukemia variant: 7-year experience in Poland. Eur J Haematol. 1999 Jan;62(1):49-56. link to original article PubMed
Robak T, Jamroziak K, Gora-Tybor J, Blonski JZ, Kasznicki M, Dwilewicz-Trojaczek J, Wiater E, Zdunczyk A, Dybowicz J, Dmoszynska A, Wojtaszko M, Zdziarska B, Calbecka M, Kostyra A, Hellmann A, Lewandowski K, Stella-Holowiecka B, Sulek K, Gawronski K, Skotnicki AB, Nowak W, Zawilska K, Molendowicz-Portala L, Kloczko J, Sokolowski J, Warzocha K, Seferynska I, Ceglarek B, Konopka L; PALG. Cladribine in a weekly versus daily schedule for untreated active hairy cell leukemia: final report from the Polish Adult Leukemia Group (PALG) of a prospective, randomized, multicenter trial. Blood. 2007 May 1;109(9):3672-5. Epub 2007 Jan 5. link to original article contains dosing details in manuscript PubMed
SAKK 32/98: Zenhäusern R, Schmitz SF, Solenthaler M, Heim D, Meyer-Monard S, Hess U, Leoncini L, Bargetzi M, Rufener B, Tobler A. Randomized trial of daily versus weekly administration of 2-chlorodeoxyadenosine in patients with hairy cell leukemia: a multicenter phase III trial (SAKK 32/98). Leuk Lymphoma. 2009 Sep;50(9):1501-11. link to original article contains dosing details in manuscript PubMed NCT00003746

Cladribine & Rituximab

Regimen

Study	Dates of enrollment	Evidence
Ravandi et al. 2011	2004-2009	Phase 2

Note: 28 days after administration of cladribine, bone marrow examination was repeated, and then rituximab was started.

Chemotherapy, first portion (course 1)

Cladribine (Leustatin) 5.6 mg/m² IV over 2 hours once per day on days 1 to 5

Supportive therapy, first portion (course 1)

All were administered at the discretion of the treating physician.

Targeted therapy, second portion (cycles 2 to 9)

Rituximab (Rituxan) 375 mg/m² IV once on day 1

28-day course, then 7-day cycle for 8 cycles

References

Ravandi F, O'Brien S, Jorgensen J, Pierce S, Faderl S, Ferrajoli A, Koller C, Challagundla P, York S, Brandt M, Luthra R, Burger J, Thomas D, Keating M, Kantarjian H. Phase 2 study of cladribine followed by rituximab in patients with hairy cell leukemia. Blood. 2011 Oct 6;118(14):3818-23. Epub 2011 Aug 5. link to original article contains dosing details in manuscript link to PMC article PubMed
1. Update: Chihara D, Kantarjian H, O'Brien S, Jorgensen J, Pierce S, Faderl S, Ferrajoli A, Poku R, Jain P, Thompson P, Brandt M, Luthra R, Burger J, Keating M, Ravandi F. Long-term durable remission by cladribine followed by rituximab in patients with hairy cell leukaemia: update of a phase II trial. Br J Haematol. 2016 Sep;174(5):760-6. Epub 2016 Jun 15. link to original article link to PMC article PubMed

Interferon alfa-2a monotherapy

Regimen

Study	Dates of enrollment	Evidence	Comparator	Comparative Efficacy
Grever et al. 1995 (SWOG 8691)	1986-1989	Phase 3 (C)	Pentostatin	Inferior RFS (co-primary endpoint)

Immunotherapy

Interferon alfa-2a (Roferon-A) 3,000,000 units SC once per day on days 1, 3, 5 (3 times per week)

7-day cycles

References

SWOG 8691: Grever M, Kopecky K, Foucar MK, Head D, Bennett JM, Hutchison RE, Corbett WE, Cassileth PA, Habermann T, Golomb H, Rai K, Eisenhauer E, Appelbaum F, Cheson BD. Randomized comparison of pentostatin versus interferon alfa-2a in previously untreated patients with hairy cell leukemia: an intergroup study. J Clin Oncol. 1995 Apr;13(4):974-82. link to original article contains dosing details in abstract PubMed
1. Update: Flinn IW, Kopecky KJ, Foucar MK, Head D, Bennett JM, Hutchison R, Corbett W, Cassileth P, Habermann T, Golomb H, Rai K, Eisenhauer E, Appelbaum F, Cheson B, Grever MR. Long-term follow-up of remission duration, mortality, and second malignancies in hairy cell leukemia patients treated with pentostatin. Blood. 2000 Nov 1;96(9):2981-6. link to original article PubMed

Pentostatin monotherapy

Regimen variant #1

Study	Dates of enrollment	Evidence
Spiers et al. 1987	1984-1987	Non-randomized

Chemotherapy

Pentostatin (Nipent) 5 mg/m² IV over 2 minutes once per day on days 1 & 2

14-day cycles, continued to the point of maximal response or treatment failure

Regimen variant #2

Study	Dates of enrollment	Evidence	Comparator	Comparative Efficacy
Grever et al. 1995 (SWOG 8691)	1986-1989	Phase 3 (E-RT-switch-ooc)	Interferon alfa-2a	Superior RFS (co-primary endpoint)

Note: patients who had complete response before 6 months were given 2 additional cycles of pentostatin, and then treatment was stopped. Patients with objective partial response after 6 months of treatment received an additional 6 months of additional therapy, until they had a complete response or progression of disease.

Chemotherapy

Pentostatin (Nipent) 4 mg/m² IV rapid injection once on day 1

Supportive therapy

1.5 liters of IV fluid hydration with every dose

14-day cycles (see note)

Dose and schedule modifications

Patients with performance status of 3 received an initial dose of 2 mg/m² IV rapid injection once on day 1, which would be increased in later cycles to 4 mg/m² IV rapid injection once on day 1 if patients had no adverse reactions.
Doses of pentostatin were held for patients who had an increase in the serum creatinine concentration greater than 20% above their baseline. Pentostatin was not given until renal function returned to baseline or if 24-hour creatinine clearance was greater than 50 mL/min/1.73m².

References

Spiers AS, Moore D, Cassileth PA, Harrington DP, Cummings FJ, Neiman RS, Bennett JM, O'Connell MJ; ECOG. Remissions in hairy-cell leukemia with pentostatin (2'-deoxycoformycin). N Engl J Med. 1987 Apr 2;316(14):825-30. link to original article contains dosing details in manuscript PubMed
1. Update: Cassileth PA, Cheuvart B, Spiers AS, Harrington DP, Cummings FJ, Neiman RS, Bennett JM, O'Connell MJ. Pentostatin induces durable remissions in hairy cell leukemia. J Clin Oncol. 1991 Feb;9(2):243-6. link to original article contains dosing details in abstract PubMed
SWOG 8691: Grever M, Kopecky K, Foucar MK, Head D, Bennett JM, Hutchison RE, Corbett WE, Cassileth PA, Habermann T, Golomb H, Rai K, Eisenhauer E, Appelbaum F, Cheson BD. Randomized comparison of pentostatin versus interferon alfa-2a in previously untreated patients with hairy cell leukemia: an intergroup study. J Clin Oncol. 1995 Apr;13(4):974-82. link to original article contains dosing details in manuscript PubMed
1. Update: Flinn IW, Kopecky KJ, Foucar MK, Head D, Bennett JM, Hutchison R, Corbett W, Cassileth P, Habermann T, Golomb H, Rai K, Eisenhauer E, Appelbaum F, Cheson B, Grever MR. Long-term follow-up of remission duration, mortality, and second malignancies in hairy cell leukemia patients treated with pentostatin. Blood. 2000 Nov 1;96(9):2981-6. link to original article contains dosing details in manuscript PubMed

Relapsed or refractory

Bendamustine & Rituximab (BR)

BR: Bendamustine, Rituximab

Regimen

Study	Dates of enrollment	Evidence
Burotto et al. 2013	NR	Pilot, fewer than 20 patients

Note: this was a dose-escalation study and the bendamustine dose was selected for further study based on absence of DLT.

Chemotherapy

Bendamustine 90 mg/m² IV once per day on days 1 & 2

Targeted therapy

Rituximab (Rituxan) 375 mg/m² IV once per day on days 1 & 15

28-day cycle for 6 cycles

References

Burotto M, Stetler-Stevenson M, Arons E, Zhou H, Wilson W, Kreitman RJ. Bendamustine and rituximab in relapsed and refractory hairy cell leukemia. Clin Cancer Res. 2013 Nov 15;19(22):6313-21. Epub 2013 Oct 28. link to original article link to PMC article contains dosing details in manuscript PubMed

Cladribine & Rituximab

Regimen

Study	Dates of enrollment	Evidence
Ravandi et al. 2011	2004-2009	Phase 2, fewer than 20 pts in this cohort

Note: 28 days after administration of cladribine, bone marrow examination was repeated, and then rituximab was started.

Chemotherapy, first portion (course 1)

Cladribine (Leustatin) 5.6 mg/m² IV over 2 hours once per day on days 1 to 5

Supportive therapy, first portion (course 1)

All were administered at the discretion of the treating physician.

Targeted therapy, second portion (cycles 2 to 9)

Rituximab (Rituxan) 375 mg/m² IV once on day 1

28-day course, then 7-day cycle for 8 cycles

References

Ravandi F, O'Brien S, Jorgensen J, Pierce S, Faderl S, Ferrajoli A, Koller C, Challagundla P, York S, Brandt M, Luthra R, Burger J, Thomas D, Keating M, Kantarjian H. Phase 2 study of cladribine followed by rituximab in patients with hairy cell leukemia. Blood. 2011 Oct 6;118(14):3818-23. Epub 2011 Aug 5. link to original article contains dosing details in manuscript link to PMC article PubMed
1. Update: Chihara D, Kantarjian H, O'Brien S, Jorgensen J, Pierce S, Faderl S, Ferrajoli A, Poku R, Jain P, Thompson P, Brandt M, Luthra R, Burger J, Keating M, Ravandi F. Long-term durable remission by cladribine followed by rituximab in patients with hairy cell leukaemia: update of a phase II trial. Br J Haematol. 2016 Sep;174(5):760-6. Epub 2016 Jun 15. link to original article link to PMC article PubMed

Interferon alfa-2a monotherapy

Regimen

Study	Dates of enrollment	Evidence
Quesada et al. 1986 (N2752)	1984-NR	Non-randomized (RT)

Note: This is of historic interest. It is not completely clear whether the cited study is the same as N2752, which is cited by the FDA in the package insert.

Immunotherapy

Interferon alfa-2a (Roferon-A)

References

N2752: Quesada JR, Hersh EM, Manning J, Reuben J, Keating M, Schnipper E, Itri L, Gutterman JU. Treatment of hairy cell leukemia with recombinant alpha-interferon. Blood. 1986 Aug;68(2):493-7. link to original article PubMed

Interferon alfa-2b monotherapy

Regimen

Study	Dates of enrollment	Evidence	Comparator	Comparative Toxicity
Quesada et al. 1984	NR	Pilot, fewer than 20 pts
Golomb et al. 1986	1983-1984	Non-randomized
Golomb et al. 1988	1985-1987	Randomized (E-RT-de-esc)	Inteferon alfa-2b x 18 mo	Seems to have lower incidence of fatigue

Note: This is of historic interest. Most patients had previously undergone splenectomy.

Immunotherapy

Interferon alfa-2b (Intron-A) 2,000,000 units SC three times per week

12-month course

References

Quesada JR, Reuben J, Manning JT, Hersh EM, Gutterman JU. Alpha interferon for induction of remission in hairy-cell leukemia. N Engl J Med. 1984 Jan 5;310(1):15-8. link to original article PubMed
Golomb HM, Jacobs A, Fefer A, Ozer H, Thompson J, Portlock C, Ratain M, Golde D, Vardiman J, Burke JS, Brady J, Bonnem E, Spiegel R. Alpha-2 interferon therapy of hairy-cell leukemia: a multicenter study of 64 patients. J Clin Oncol. 1986 Jun;4(6):900-5. link to original article PubMed
Golomb HM, Ratain MJ, Fefer A, Thompson J, Golde DW, Ozer H, Portlock C, Silber R, Rappeport J, Bonnem E, Spiegel R, Tensen L, Burke JS, Vardiman JW. Randomized study of the duration of treatment with interferon alfa-2B in patients with hairy cell leukemia. J Natl Cancer Inst. 1988 May 4;80(5):369-73. link to original article PubMed

Moxetumomab pasudotox monotherapy

Regimen

FDA-recommended dose

Study	Dates of enrollment	Evidence
Kreitman et al. 2018 (Study 1053)	2013-2016	Phase 2 (RT)

Targeted therapy

Moxetumomab pasudotox (Lumoxiti) 0.04 mg/kg IV over 30 minutes once per day on days 1, 3, 5

28-day cycle for up to 6 cycles

References

Study 1053: Kreitman RJ, Dearden C, Zinzani PL, Delgado J, Karlin L, Robak T, Gladstone DE, le Coutre P, Dietrich S, Gotic M, Larratt L, Offner F, Schiller G, Swords R, Bacon L, Bocchia M, Bouabdallah K, Breems DA, Cortelezzi A, Dinner S, Doubek M, Gjertsen BT, Gobbi M, Hellmann A, Lepretre S, Maloisel F, Ravandi F, Rousselot P, Rummel M, Siddiqi T, Tadmor T, Troussard X, Yi CA, Saglio G, Roboz GJ, Balic K, Standifer N, He P, Marshall S, Wilson W, Pastan I, Yao NS, Giles F. Moxetumomab pasudotox in relapsed/refractory hairy cell leukemia. Leukemia. 2018 Aug;32(8):1768-1777. Epub 2018 Jul 20. link to original article link to PMC article contains dosing details in abstract PubMed NCT01829711
1. Update: Kreitman RJ, Dearden C, Zinzani PL, Delgado J, Robak T, le Coutre PD, Gjertsen BT, Troussard X, Roboz GJ, Karlin L, Gladstone DE, Kuptsova-Clarkson N, Liu S, Patel P, Rotolo F, Mitry E, Pastan I, Giles F; Study 1053 investigators. Moxetumomab pasudotox in heavily pre-treated patients with relapsed/refractory hairy cell leukemia (HCL): long-term follow-up from the pivotal trial. J Hematol Oncol. 2021 Feb 24;14(1):35. link to original article link to PMC article PubMed

Rituximab monotherapy

Regimen

Study	Dates of enrollment	Evidence
Nieva et al. 2003	2000-2002	Phase 2

Targeted therapy

Rituximab (Rituxan) 375 mg/m² IV once per day on days 1, 8, 15, 22

Supportive therapy

Allopurinol (Zyloprim) 300 mg PO once per day on days 1 to 7

4-week course

References

Nieva J, Bethel K, Saven A. Phase 2 study of rituximab in the treatment of cladribine-failed patients with hairy cell leukemia. Blood. 2003 Aug 1;102(3):810-3. Epub 2003 Mar 27. link to original article contains dosing details in manuscript PubMed

Vemurafenib monotherapy

Regimen

Study	Dates of enrollment	Evidence	Efficacy
Tiacci et al. 2015 (MSKCC 12-200)	2013-2015	Phase 2	ORR: 96-100%
Tiacci et al. 2015 (HCL-PG01)	2013-2015	Phase 2	ORR: 96-100%

Note: a pooled analysis of two phase 2 trials is reported in Tiacci et al. 2015; they have slightly different durations - please see original text for details.

Targeted therapy

Vemurafenib (Zelboraf) 960 mg PO twice per day on days 1 to 28

28-day cycle for up to 4 cycles (HCL-PG01) or 6 cycles (MSKCC 12-200)

References

MSKCC 12-200: Tiacci E, Park JH, De Carolis L, Chung SS, Broccoli A, Scott S, Zaja F, Devlin S, Pulsoni A, Chung YR, Cimminiello M, Kim E, Rossi D, Stone RM, Motta G, Saven A, Varettoni M, Altman JK, Anastasia A, Grever MR, Ambrosetti A, Rai KR, Fraticelli V, Lacouture ME, Carella AM, Levine RL, Leoni P, Rambaldi A, Falzetti F, Ascani S, Capponi M, Martelli MP, Park CY, Pileri SA, Rosen N, Foà R, Berger MF, Zinzani PL, Abdel-Wahab O, Falini B, Tallman MS. Targeting mutant BRAF in relapsed or refractory hairy-cell leukemia. N Engl J Med. 2015 Oct 29;373(18):1733-47. Epub 2015 Sep 9. link to original article contains dosing details in manuscript link to PMC article PubMed NCT01711632
HCL-PG01: Tiacci E, Park JH, De Carolis L, Chung SS, Broccoli A, Scott S, Zaja F, Devlin S, Pulsoni A, Chung YR, Cimminiello M, Kim E, Rossi D, Stone RM, Motta G, Saven A, Varettoni M, Altman JK, Anastasia A, Grever MR, Ambrosetti A, Rai KR, Fraticelli V, Lacouture ME, Carella AM, Levine RL, Leoni P, Rambaldi A, Falzetti F, Ascani S, Capponi M, Martelli MP, Park CY, Pileri SA, Rosen N, Foà R, Berger MF, Zinzani PL, Abdel-Wahab O, Falini B, Tallman MS. Targeting mutant BRAF in relapsed or refractory hairy-cell leukemia. N Engl J Med. 2015 Oct 29;373(18):1733-47. Epub 2015 Sep 9. link to original article contains dosing details in manuscript link to PMC article PubMed EudraCT 2011-005487-13

Hairy cell leukemia variant (HCLv), all lines of therapy

Note: this is a distinct disease process from hairy cell leukemia, but will be included on this page for now given the similarities. We may create a dedicated page in the future.

Cladribine & Rituximab

Regimen

Study	Dates of enrollment	Evidence
Ravandi et al. 2011	2004-2009	Phase 2, fewer than 20 pts in this cohort

Note: 28 days after administration of cladribine, bone marrow examination was repeated, and then rituximab was started.

Chemotherapy, first portion (course 1)

Cladribine (Leustatin) 5.6 mg/m² IV over 2 hours once per day on days 1 to 5

Supportive therapy, first portion (course 1)

All were administered at the discretion of the treating physician.

Targeted therapy, second portion (cycles 2 to 9)

Rituximab (Rituxan) 375 mg/m² IV once on day 1

28-day course, then 7-day cycle for 8 cycles

References

Ravandi F, O'Brien S, Jorgensen J, Pierce S, Faderl S, Ferrajoli A, Koller C, Challagundla P, York S, Brandt M, Luthra R, Burger J, Thomas D, Keating M, Kantarjian H. Phase 2 study of cladribine followed by rituximab in patients with hairy cell leukemia. Blood. 2011 Oct 6;118(14):3818-23. Epub 2011 Aug 5. link to original article contains dosing details in manuscript link to PMC article PubMed
1. Update: Chihara D, Kantarjian H, O'Brien S, Jorgensen J, Pierce S, Faderl S, Ferrajoli A, Poku R, Jain P, Thompson P, Brandt M, Luthra R, Burger J, Keating M, Ravandi F. Long-term durable remission by cladribine followed by rituximab in patients with hairy cell leukaemia: update of a phase II trial. Br J Haematol. 2016 Sep;174(5):760-6. Epub 2016 Jun 15. link to original article link to PMC article PubMed

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-'''Use of this site is subject to you reading and agreeing with the terms set forth in the [[HemOnc.org_-_A_Hematology_Oncology_Wiki:General_disclaimer|disclaimer]].'''
+<span id="BackToTop"></span>
+<div class="noprint" style="background-color:LightGray; position:fixed; bottom:2%; right:0.25%; padding-left:5px; padding-right:5px; margin: 15px; opacity:0.8; border-style: solid; border-color:DarkGray; border-width: 1px">
-Is there a regimen missing from this list?  Would you like to share a different dosage/schedule or an additional reference for a regimen?  Have you noticed an error?  Do you have an idea that will help the site grow to better meet your needs and the needs of many others?  You are [[How_to_contribute|invited to contribute to the site]].
+[[#top|Back to Top]]
+</div>
+{{#lst:Editorial board transclusions|inhl}}
+{| class="wikitable" style="float:right; margin-right: 5px;"
+|-
+|<div style="background-color: #fee0d1; border: 1px solid #808000; padding: 5px; {{border-radius|16px}}" align="right"><font size="4"><b>{{#ask: [[-Has subobject::{{FULLPAGENAME}}]] |?Regimen |limit=10000|format=sum}} [[Tutorial#Regimens|regimens]] on this page</b></font></div>
+<div style="background-color: #deebf6; border: 1px solid #808000; padding: 5px; {{border-radius|16px}}"><font size="4"><b>{{#ask: [[-Has subobject::{{FULLPAGENAME}}]] |?Variant |limit=10000|format=sum}} [[Tutorial#Variants|variants]] on this page</b></font></div>
+|}
 {{TOC limit|limit=3}}
+=Guidelines=
+'''Given the rapid change in evidence in many areas of hematology/oncology, readers are encouraged to consider any guideline published 5+ years ago to be for historical purposes, only.'''
+==ESMO==
+*'''2015:''' Robak et al. [https://doi.org/10.1093/annonc/mdv200 Hairy cell leukaemia: ESMO Clinical Practice Guidelines for diagnosis, treatment and follow-up] [https://pubmed.ncbi.nlm.nih.gov/26269205/ PubMed]
+==Hairy Cell Leukemia Foundation==
+*'''2017:''' Grever et al. [https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5290982/ Consensus guidelines for the diagnosis and management of patients with classic hairy cell leukemia] [https://pubmed.ncbi.nlm.nih.gov/27903528/ PubMed]
+==NCCN==
+*[https://www.nccn.org/guidelines/guidelines-detail?category=1&id=1481 NCCN Guidelines - Hairy Cell Leukemia]
+**'''2017:''' Wierda et al. [https://doi.org/10.6004/jnccn.2017.0165 Hairy Cell Leukemia, Version 2.2018, NCCN Clinical Practice Guidelines in Oncology] [https://pubmed.ncbi.nlm.nih.gov/29118233/ PubMed]
 =Untreated=
-==Cladribine (Leustatin)==
+==Cladribine monotherapy {{#subobject:dbcbf4|Regimen=1}}==
-===Regimen #1, Tallman, et al. 1996===
+<div class="toccolours" style="background-color:#eeeeee">
-Level of Evidence:
+===Regimen variant #1, 0.09 mg/kg/day x 7 days {{#subobject:c4fe17|Variant=1}}===
-<span
+{| class="wikitable" style="width: 60%; text-align:center;"
-style="background:#EEEE00;
+!style="width: 33%"|Study
-padding:3px 6px 3px 6px;
+!style="width: 33%"|Dates of enrollment
-border-color:black;
+!style="width: 33%"|[[Levels_of_Evidence#Evidence|Evidence]]
-border-width:2px;
+|-
-border-style:solid;">Phase II</span>
+|[https://doi.org/10.1182/blood.V92.6.1918 Saven et al. 1998]
+|1986-1993
-*[[Cladribine (Leustatin)]] 0.1 mg/kg/day IV continuous infusion on days 1 to 7 (total dose per cycle: 0.7 mg/kg)
+|style="background-color:#91cf61"|Non-randomized (RT)
+|-
+|}
+<div class="toccolours" style="background-color:#b3e2cd">
+====Chemotherapy====
+*[[Cladribine (Leustatin)]] 0.09 mg/kg/day IV continuous infusion over 7 days, started on day 1 (total dose: 0.63 mg/kg)
+'''7-day course'''
+</div></div><br>
+<div class="toccolours" style="background-color:#eeeeee">
+===Regimen variant #2, 0.1 mg/kg/day x 7 days {{#subobject:c4fe60|Variant=1}}===
+{| class="wikitable sortable" style="width: 60%; text-align:center;"
+!style="width: 33%"|Study
+!style="width: 33%"|Dates of enrollment
+!style="width: 33%"|[[Levels_of_Evidence#Evidence|Evidence]]
+|-
+|[https://doi.org/10.1056/NEJM199004193221605 Piro et al. 1990]
+|1986-1989
+| style="background-color:#ffffbe" |Pilot, fewer than 20 pts
+|-
+|[https://doi.org/10.1182/blood.V88.6.1954.bloodjournal8861954 Tallman et al. 1996]
+|1991-1995
+|style="background-color:#91cf61"|Phase 2
+|-
+|}
+<div class="toccolours" style="background-color:#b3e2cd">
+====Chemotherapy====
+*[[Cladribine (Leustatin)]] 0.1 mg/kg/day IV continuous infusion over 7 days, started on day 1 (total dose: 0.7 mg/kg)
 '''7-day course'''
+</div></div><br>
-===Regimen #2, Zenhäusern, et al. 2009 (SAKK 32/98)===
+<div class="toccolours" style="background-color:#eeeeee">
-Level of Evidence:
+===Regimen variant #3, 0.12 mg/kg/day x 5 days {{#subobject:e3c2ec|Variant=1}}===
-<span
+{| class="wikitable sortable" style="width: 100%; text-align:center;"
-style="background:#00CD00;
+!style="width: 20%"|Study
-padding:3px 6px 3px 6px;
+!style="width: 20%"|Dates of enrollment
-border-color:black;
+!style="width: 20%"|[[Levels_of_Evidence#Evidence|Evidence]]
-border-width:2px;
+!style="width: 20%"|Comparator
-border-style:solid;">Phase III</span>
+!style="width: 20%"|[[Levels_of_Evidence#Comparative_efficacy|Comparative Efficacy]]
+|-
-*[[Cladribine (Leustatin)]] 0.14 mg/kg/day IV continuous infusion on days 1 to 5 (total dose per cycle: 0.7 mg/kg)
+|[https://doi.org/10.1111/j.1600-0609.1999.tb01114.x Robak et al. 1999]
+|1991-1997
+|style="background-color:#91cf61"|Phase 2
+| style="background-color:#d3d3d3" |
+| style="background-color:#d3d3d3" |
+|-
+|[https://doi.org/10.1182/blood-2006-08-042929 Robak et al. 2007]
+|1998-2005
+|style="background-color:#1a9851"|Phase 3 (C)
+|[[#Cladribine_monotherapy|Cladribine]]; weekly
+|style="background-color:#ffffbf"|Did not meet primary endpoint of CR rate
+|-
+|}
+<div class="toccolours" style="background-color:#b3e2cd">
+====Chemotherapy====
+*[[Cladribine (Leustatin)]] 0.12 mg/kg IV over 2 hours once per day on days 1 to 5
+'''5-day course'''
+</div></div><br>
+<div class="toccolours" style="background-color:#eeeeee">
+===Regimen variant #4, 0.14 mg/kg/day x 5 days {{#subobject:8929f9|Variant=1}}===
+{| class="wikitable sortable" style="width: 100%; text-align:center;"
+!style="width: 20%"|Study
+!style="width: 20%"|Dates of enrollment
+!style="width: 20%"|[[Levels_of_Evidence#Evidence|Evidence]]
+!style="width: 20%"|Comparator
+!style="width: 20%"|[[Levels_of_Evidence#Comparative_efficacy|Comparative Efficacy]]
+|-
+|[https://doi.org/10.1080/10428190903131755 Zenhäusern et al. 2009 (SAKK 32/98)]
+|1998-2005
+|style="background-color:#1a9851"|Phase 3 (C)
+|[[#Cladribine_monotherapy|Cladribine]]; weekly
+|style="background-color:#ffffbf"|Did not meet primary endpoint of average leukocyte count within 6 weeks from randomization
+|-
+|}
+<div class="toccolours" style="background-color:#b3e2cd">
+====Chemotherapy====
+*[[Cladribine (Leustatin)]] 0.14 mg/kg SC once per day on days 1 to 5
+'''5-day course'''
+</div></div><br>
+<div class="toccolours" style="background-color:#eeeeee">
+===Regimen variant #5, 0.15 mg/kg/day x 5 days {{#subobject:44f9c0|Variant=1}}===
+{| class="wikitable sortable" style="width: 60%; text-align:center;"
+!style="width: 33%"|Study
+!style="width: 33%"|Dates of enrollment
+!style="width: 33%"|[[Levels_of_Evidence#Evidence|Evidence]]
+|-
+|[https://pubmed.ncbi.nlm.nih.gov/9558908 Damasio et al. 1998]
+|NR in abstract
+|style="background-color:#91cf61"|Phase 2
+|-
+|}
+<div class="toccolours" style="background-color:#b3e2cd">
+====Chemotherapy====
+*[[Cladribine (Leustatin)]] 0.15 mg/kg IV over 2 hours once per day on days 1 to 5
 '''5-day course'''
+</div></div>
-===Regimen #3, Zenhäusern, et al. 2009 (SAKK 32/98)===
+===References===
-Level of Evidence:
+# Piro LD, Carrera CJ, Carson DA, Beutler E. Lasting remissions in hairy-cell leukemia induced by a single infusion of 2-chlorodeoxyadenosine. N Engl J Med. 1990 Apr 19;322(16):1117-21. [https://doi.org/10.1056/NEJM199004193221605 link to original article] '''contains dosing details in abstract''' [https://pubmed.ncbi.nlm.nih.gov/1969613/ PubMed]
-<span
+# Tallman MS, Hakimian D, Rademaker AW, Zanzig C, Wollins E, Rose E, Peterson LC. Relapse of hairy cell leukemia after 2-chlorodeoxyadenosine: long-term follow-up of the Northwestern University experience. Blood. 1996 Sep 15;88(6):1954-9. [https://doi.org/10.1182/blood.V88.6.1954.bloodjournal8861954 link to original article] '''contains dosing details in manuscript''' [https://pubmed.ncbi.nlm.nih.gov/8822913/ PubMed]
-style="background:#00CD00;
+## '''Update:''' Chadha P, Rademaker AW, Mendiratta P, Kim B, Evanchuk DM, Hakimian D, Peterson LC, Tallman MS. Treatment of hairy cell leukemia with 2-chlorodeoxyadenosine (2-CdA): long-term follow-up of the Northwestern University experience. Blood. 2005 Jul 1;106(1):241-6. Epub 2005 Mar 10. [https://doi.org/10.1182/blood-2005-01-0173 link to original article] '''contains dosing details in abstract''' [https://pubmed.ncbi.nlm.nih.gov/15761021/ PubMed] content property of [https://hemonc.org HemOnc.org]
-padding:3px 6px 3px 6px;
+# Damasio EE, Resegotti L, Masoudi B, Bruni R, Cerri R, Isaza A, Clavio M, Risso M, Rossi E, Spriano M, Truini M; Italian Group for the Hairy Cell Leukemia. Five day intermittent vs seven day continuous 2-chlorodeoxyadenosine infusion for the treatment of hairy cell leukemia: a study by Italian Group for the Hairy Cell Leukemia. Recenti Prog Med. 1998 Feb;89(2):68-73. [https://pubmed.ncbi.nlm.nih.gov/9558908/ PubMed]
-border-color:black;
+# Saven A, Burian C, Koziol JA, Piro LD. Long-term follow-up of patients with hairy cell leukemia after cladribine treatment. Blood. 1998 Sep 15;92(6):1918-26. [https://doi.org/10.1182/blood.V92.6.1918 link to original article] [https://pubmed.ncbi.nlm.nih.gov/9731048/ PubMed]
-border-width:2px;
+# Robak T, Błasińska-Morawiec M, Błoński J, Hellmann A, Hałaburda K, Konopka L, Kotlarek-Haus S, Potoczek S, Hansz J, Dmoszyńska A, Urasiński I, Zdziarska B, Dwilewicz-Trojaczek J, Hołowiecki J, Skotnicki AB. 2-chlorodeoxyadenosine (cladribine) in the treatment of hairy cell leukemia and hairy cell leukemia variant: 7-year experience in Poland. Eur J Haematol. 1999 Jan;62(1):49-56. [https://doi.org/10.1111/j.1600-0609.1999.tb01114.x link to original article] [https://pubmed.ncbi.nlm.nih.gov/9918312/ PubMed]
-border-style:solid;">Phase III</span>
+# Robak T, Jamroziak K, Gora-Tybor J, Blonski JZ, Kasznicki M, Dwilewicz-Trojaczek J, Wiater E, Zdunczyk A, Dybowicz J, Dmoszynska A, Wojtaszko M, Zdziarska B, Calbecka M, Kostyra A, Hellmann A, Lewandowski K, Stella-Holowiecka B, Sulek K, Gawronski K, Skotnicki AB, Nowak W, Zawilska K, Molendowicz-Portala L, Kloczko J, Sokolowski J, Warzocha K, Seferynska I, Ceglarek B, Konopka L; PALG. Cladribine in a weekly versus daily schedule for untreated active hairy cell leukemia: final report from the Polish Adult Leukemia Group (PALG) of a prospective, randomized, multicenter trial. Blood. 2007 May 1;109(9):3672-5. Epub 2007 Jan 5. [https://doi.org/10.1182/blood-2006-08-042929 link to original article] '''contains dosing details in manuscript''' [https://pubmed.ncbi.nlm.nih.gov/17209059/ PubMed]
+# '''SAKK 32/98:''' Zenhäusern R, Schmitz SF, Solenthaler M, Heim D, Meyer-Monard S, Hess U, Leoncini L, Bargetzi M, Rufener B, Tobler A. Randomized trial of daily versus weekly administration of 2-chlorodeoxyadenosine in patients with hairy cell leukemia: a multicenter phase III trial (SAKK 32/98). Leuk Lymphoma. 2009 Sep;50(9):1501-11. [https://doi.org/10.1080/10428190903131755 link to original article] '''contains dosing details in manuscript''' [https://pubmed.ncbi.nlm.nih.gov/19672771/ PubMed] [https://clinicaltrials.gov/study/NCT00003746 NCT00003746]
-*[[Cladribine (Leustatin)]] 0.14 mg/kg/day IV once per day on days 1, 8, 15, 22, 29
+==Cladribine & Rituximab {{#subobject:2beb9d|Regimen=1}}==
+<div class="toccolours" style="background-color:#eeeeee">
-'''5-week course'''
+===Regimen {{#subobject:668e5c|Variant=1}}===
+{| class="wikitable sortable" style="width: 60%; text-align:center;"
+!style="width: 33%"|Study
+!style="width: 33%"|Dates of enrollment
+!style="width: 33%"|[[Levels_of_Evidence#Evidence|Evidence]]
+|-
+|[https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4081440/ Ravandi et al. 2011]
+|2004-2009
+|style="background-color:#91cf61"|Phase 2
+|-
+|}
+''Note: 28 days after administration of cladribine, bone marrow examination was repeated, and then rituximab was started.''
+<div class="toccolours" style="background-color:#b3e2cd">
+====Chemotherapy, first portion (course 1)====
+*[[Cladribine (Leustatin)]] 5.6 mg/m<sup>2</sup> IV over 2 hours once per day on days 1 to 5
+====Supportive therapy, first portion (course 1)====
+''All were administered at the discretion of the treating physician.''
+*[[Levofloxacin (Levaquin)]]
+*[[Valacyclovir (Valtrex)]]
+*[[Fluconazole (Diflucan)]]
+*[[Filgrastim (Neupogen)]]
+====Targeted therapy, second portion (cycles 2 to 9)====
+*[[Rituximab (Rituxan)]] 375 mg/m<sup>2</sup> IV once on day 1
+'''28-day course, then 7-day cycle for 8 cycles'''
+</div></div>
 ===References===
-# Tallman MS, Hakimian D, Rademaker AW, Zanzig C, Wollins E, Rose E, Peterson LC. Relapse of hairy cell leukemia after 2-chlorodeoxyadenosine: long-term follow-up of the Northwestern University experience. Blood. 1996 Sep 15;88(6):1954-9. [http://bloodjournal.hematologylibrary.org/content/88/6/1954.long link to original article] '''contains verified protocol''' [http://www.ncbi.nlm.nih.gov/pubmed/8822913 PubMed]
+# Ravandi F, O'Brien S, Jorgensen J, Pierce S, Faderl S, Ferrajoli A, Koller C, Challagundla P, York S, Brandt M, Luthra R, Burger J, Thomas D, Keating M, Kantarjian H. Phase 2 study of cladribine followed by rituximab in patients with hairy cell leukemia. Blood. 2011 Oct 6;118(14):3818-23. Epub 2011 Aug 5. [https://doi.org/10.1182/blood-2011-04-351502 link to original article] '''contains dosing details in manuscript''' [https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4081440/ link to PMC article] [https://pubmed.ncbi.nlm.nih.gov/21821712/ PubMed]
-# '''Update:''' Chadha P, Rademaker AW, Mendiratta P, Kim B, Evanchuk DM, Hakimian D, Peterson LC, Tallman MS. Treatment of hairy cell leukemia with 2-chlorodeoxyadenosine (2-CdA): long-term follow-up of the Northwestern University experience. Blood. 2005 Jul 1;106(1):241-6. Epub 2005 Mar 10. [http://bloodjournal.hematologylibrary.org/content/106/1/241.long link to original article] '''contains protocol''' [http://www.ncbi.nlm.nih.gov/pubmed/15761021 PubMed] content property of [http://hemonc.org HemOnc.org]
+## '''Update:''' Chihara D, Kantarjian H, O'Brien S, Jorgensen J, Pierce S, Faderl S, Ferrajoli A, Poku R, Jain P, Thompson P, Brandt M, Luthra R, Burger J, Keating M, Ravandi F. Long-term durable remission by cladribine followed by rituximab in patients with hairy cell leukaemia: update of a phase II trial. Br J Haematol. 2016 Sep;174(5):760-6. Epub 2016 Jun 15. [https://doi.org/10.1111/bjh.14129 link to original article] [https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5396841/ link to PMC article] [https://pubmed.ncbi.nlm.nih.gov/27301277/ PubMed]
-# Zenhäusern R, Schmitz SF, Solenthaler M, Heim D, Meyer-Monard S, Hess U, Leoncini L, Bargetzi M, Rufener B, Tobler A. Randomized trial of daily versus weekly administration of 2-chlorodeoxyadenosine in patients with hairy cell leukemia: a multicenter phase III trial (SAKK 32/98). Leuk Lymphoma. 2009 Sep;50(9):1501-11. doi: 10.1080/10428190903131755. [http://informahealthcare.com/doi/abs/10.1080/10428190903131755 link to original article] '''contains protocol''' [http://www.ncbi.nlm.nih.gov/pubmed/19672771 PubMed]
+==Interferon alfa-2a monotherapy {{#subobject:cmrxb3|Regimen=1}}==
+<div class="toccolours" style="background-color:#eeeeee">
-==Cladribine & Rituximab==
+===Regimen {{#subobject:mww551|Variant=1}}===
-===Regimen, Ravandi et al. 2011===
+{| class="wikitable sortable" style="width: 100%; text-align:center;"
-Level of Evidence:
+!style="width: 20%"|Study
-<span
+!style="width: 20%"|Dates of enrollment
-style="background:#EEEE00;
+!style="width: 20%"|[[Levels_of_Evidence#Evidence|Evidence]]
-padding:3px 6px 3px 6px;
+!style="width: 20%"|Comparator
-border-color:black;
+!style="width: 20%"|[[Levels_of_Evidence#Comparative_efficacy|Comparative Efficacy]]
-border-width:2px;
+|-
-border-style:solid;">Phase II</span>
+|[https://doi.org/10.1200/jco.1995.13.4.974 Grever et al. 1995 (SWOG 8691)]
+|1986-1989
-*[[Cladribine (Leustatin)]] 5.6 mg/m2 IV 2 hours daily for 5 days
+|style="background-color:#1a9851"|Phase 3 (C)
+|[[#Pentostatin_monotherapy|Pentostatin]]
-'''28 days after administration of cladribine, bone marrow examination was repeated, and then rituximab was started'''
+| style="background-color:#d73027" |Inferior RFS (co-primary endpoint)
+|-
-*[[Rituximab (Rituxan)]] 375 mg/m2 IV weekly for 8 weeks
+|}
+<div class="toccolours" style="background-color:#b3e2cd">
-Supportive medications:
+====Immunotherapy====
-''All were administered at the discretion of the treating physician''
+*[[Interferon alfa-2a (Roferon-A)]] 3,000,000 units SC once per day on days 1, 3, 5 (3 times per week)
-*Levofloxacin (Levaquin)
+'''7-day cycles'''
-*Valcyclovir (Valcyte)
+</div></div>
-*Fluconazole (Diflucan)
-*Allopurinol (Aloprim)
-*Filgrastim (Neupogen)
 ===References===
-# Ravandi F, O'Brien S, Jorgensen J, Pierce S, Faderl S, Ferrajoli A, Koller C, Challagundla P, York S, Brandt M, Luthra R, Burger J, Thomas D, Keating M, Kantarjian H. Phase 2 study of cladribine followed by rituximab in patients with hairy cell leukemia. Blood. 2011 Oct 6;118(14):3818-23. [http://bloodjournal.hematologylibrary.org/content/118/14/3818.long link to original article] '''contains verified protocol''' [http://www.ncbi.nlm.nih.gov/pubmed/21821712 PubMed]
+# '''SWOG 8691:''' Grever M, Kopecky K, Foucar MK, Head D, Bennett JM, Hutchison RE, Corbett WE, Cassileth PA, Habermann T, Golomb H, Rai K, Eisenhauer E, Appelbaum F, Cheson BD. Randomized comparison of pentostatin versus interferon alfa-2a in previously untreated patients with hairy cell leukemia: an intergroup study. J Clin Oncol. 1995 Apr;13(4):974-82. [https://doi.org/10.1200/jco.1995.13.4.974 link to original article] '''contains dosing details in abstract''' [https://pubmed.ncbi.nlm.nih.gov/7707126/ PubMed]
+## '''Update:''' Flinn IW, Kopecky KJ, Foucar MK, Head D, Bennett JM, Hutchison R, Corbett W, Cassileth P, Habermann T, Golomb H, Rai K, Eisenhauer E, Appelbaum F, Cheson B, Grever MR. Long-term follow-up of remission duration, mortality, and second malignancies in hairy cell leukemia patients treated with pentostatin. Blood. 2000 Nov 1;96(9):2981-6. [https://doi.org/10.1182/blood.V96.9.2981 link to original article] [https://pubmed.ncbi.nlm.nih.gov/11049974/ PubMed]
-==Pentostatin (Nipent)==
-Level of Evidence:
-<span
-style="background:#EEEE00;
-padding:3px 6px 3px 6px;
-border-color:black;
-border-width:2px;
-border-style:solid;">Phase II</span>
-*[[Pentostatin (Nipent)]] 5 mg/m2 IV over 2 minutes once per day on days 1 & 2
+==Pentostatin monotherapy {{#subobject:c911b3|Regimen=1}}==
+<div class="toccolours" style="background-color:#eeeeee">
+===Regimen variant #1 {{#subobject:85a551|Variant=1}}===
+{| class="wikitable sortable" style="width: 60%; text-align:center;"
+!style="width: 33%"|Study
+!style="width: 33%"|Dates of enrollment
+!style="width: 33%"|[[Levels_of_Evidence#Evidence|Evidence]]
+|-
+|[https://doi.org/10.1056/NEJM198704023161401 Spiers et al. 1987]
+|1984-1987
+|style="background-color:#91cf61"|Non-randomized
+|-
+|}
+<div class="toccolours" style="background-color:#b3e2cd">
+====Chemotherapy====
+*[[Pentostatin (Nipent)]] 5 mg/m<sup>2</sup> IV over 2 minutes once per day on days 1 & 2
 '''14-day cycles, continued to the point of maximal response or treatment failure'''
+</div></div><br>
+<div class="toccolours" style="background-color:#eeeeee">
+===Regimen variant #2 {{#subobject:3c386e|Variant=1}}===
+{| class="wikitable sortable" style="width: 100%; text-align:center;"
+!style="width: 20%"|Study
+!style="width: 20%"|Dates of enrollment
+!style="width: 20%"|[[Levels_of_Evidence#Evidence|Evidence]]
+!style="width: 20%"|Comparator
+!style="width: 20%"|[[Levels_of_Evidence#Comparative_efficacy|Comparative Efficacy]]
+|-
+|[https://doi.org/10.1200/jco.1995.13.4.974 Grever et al. 1995 (SWOG 8691)]
+|1986-1989
+|style="background-color:#1a9851"|Phase 3 (E-RT-switch-ooc)
+|[[#Interferon_alfa-2a_monotherapy|Interferon alfa-2a]]
+|style="background-color:#1a9850"|Superior RFS (co-primary endpoint)
+|-
+|}
+''Note: patients who had complete response before 6 months were given 2 additional cycles of pentostatin, and then treatment was stopped. Patients with objective partial response after 6 months of treatment received an additional 6 months of additional therapy, until they had a complete response or progression of disease.''
+<div class="toccolours" style="background-color:#b3e2cd">
+====Chemotherapy====
+*[[Pentostatin (Nipent)]] 4 mg/m<sup>2</sup> IV rapid injection once on day 1
+====Supportive therapy====
+*1.5 liters of IV fluid hydration with every dose
+'''14-day cycles (see note)'''
+</div>
+<div class="toccolours" style="background-color:#fff2ae">
+====Dose and schedule modifications====
+*Patients with performance status of 3 received an initial dose of 2 mg/m<sup>2</sup> IV rapid injection once on day 1, which would be increased in later cycles to 4 mg/m<sup>2</sup> IV rapid injection once on day 1 if patients had no adverse reactions.
+*Doses of pentostatin were held for patients who had an increase in the serum creatinine concentration greater than 20% above their baseline. Pentostatin was not given until renal function returned to baseline or if 24-hour creatinine clearance was greater than 50 mL/min/1.73m<sup>2</sup>.
+</div></div>
-Supportive medications:
+===References===
-*Allopurinol (Aloprim) 300 mg PO once per day in "the initial courses of treatment" for prophylaxis
+# Spiers AS, Moore D, Cassileth PA, Harrington DP, Cummings FJ, Neiman RS, Bennett JM, O'Connell MJ; ECOG. Remissions in hairy-cell leukemia with pentostatin (2'-deoxycoformycin). N Engl J Med. 1987 Apr 2;316(14):825-30. [https://doi.org/10.1056/NEJM198704023161401 link to original article] '''contains dosing details in manuscript''' [https://pubmed.ncbi.nlm.nih.gov/2434850/ PubMed]
+## '''Update:''' Cassileth PA, Cheuvart B, Spiers AS, Harrington DP, Cummings FJ, Neiman RS, Bennett JM, O'Connell MJ. Pentostatin induces durable remissions in hairy cell leukemia. J Clin Oncol. 1991 Feb;9(2):243-6. [https://doi.org/10.1200/jco.1991.9.2.243 link to original article] '''contains dosing details in abstract''' [https://pubmed.ncbi.nlm.nih.gov/1988572/ PubMed]
+# '''SWOG 8691:''' Grever M, Kopecky K, Foucar MK, Head D, Bennett JM, Hutchison RE, Corbett WE, Cassileth PA, Habermann T, Golomb H, Rai K, Eisenhauer E, Appelbaum F, Cheson BD. Randomized comparison of pentostatin versus interferon alfa-2a in previously untreated patients with hairy cell leukemia: an intergroup study. J Clin Oncol. 1995 Apr;13(4):974-82. [https://doi.org/10.1200/jco.1995.13.4.974 link to original article] '''contains dosing details in manuscript''' [https://pubmed.ncbi.nlm.nih.gov/7707126/ PubMed]
+## '''Update:''' Flinn IW, Kopecky KJ, Foucar MK, Head D, Bennett JM, Hutchison R, Corbett W, Cassileth P, Habermann T, Golomb H, Rai K, Eisenhauer E, Appelbaum F, Cheson B, Grever MR. Long-term follow-up of remission duration, mortality, and second malignancies in hairy cell leukemia patients treated with pentostatin. Blood. 2000 Nov 1;96(9):2981-6. [https://doi.org/10.1182/blood.V96.9.2981 link to original article] '''contains dosing details in manuscript''' [https://pubmed.ncbi.nlm.nih.gov/11049974/ PubMed]
+=Relapsed or refractory=
+==Bendamustine & Rituximab (BR) {{#subobject:3904a|Regimen=1}}==
+BR: '''<u>B</u>'''endamustine, '''<u>R</u>'''ituximab
+<div class="toccolours" style="background-color:#eeeeee">
+===Regimen {{#subobject:98f0da|Variant=1}}===
+{| class="wikitable sortable" style="width: 60%; text-align:center;"
+!style="width: 33%"|Study
+!style="width: 33%"|Dates of enrollment
+!style="width: 33%"|[[Levels_of_Evidence#Evidence|Evidence]]
+|-
+|[https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3861900/ Burotto et al. 2013]
+|NR
+|style="background-color:#ffffbe"|Pilot, fewer than 20 patients
+|-
+|}
+''Note: this was a dose-escalation study and the bendamustine dose was selected for further study based on absence of DLT.''
+<div class="toccolours" style="background-color:#b3e2cd">
+====Chemotherapy====
+*[[Bendamustine]] 90 mg/m<sup>2</sup> IV once per day on days 1 & 2
+====Targeted therapy====
+*[[Rituximab (Rituxan)]] 375 mg/m<sup>2</sup> IV once per day on days 1 & 15
+'''28-day cycle for 6 cycles'''
+</div></div>
 ===References===
-# Cassileth PA, Cheuvart B, Spiers AS, Harrington DP, Cummings FJ, Neiman RS, Bennett JM, O'Connell MJ. Pentostatin induces durable remissions in hairy cell leukemia. J Clin Oncol. 1991 Feb;9(2):243-6. [http://jco.ascopubs.org/content/9/2/243.long link to original article] '''contains protocol''' [http://www.ncbi.nlm.nih.gov/pubmed/1988572 PubMed]
+# Burotto M, Stetler-Stevenson M, Arons E, Zhou H, Wilson W, Kreitman RJ. Bendamustine and rituximab in relapsed and refractory hairy cell leukemia. Clin Cancer Res. 2013 Nov 15;19(22):6313-21. Epub 2013 Oct 28. [https://doi.org/10.1158/1078-0432.CCR-13-1848 link to original article] [https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3861900/ link to PMC article] '''contains dosing details in manuscript''' [https://pubmed.ncbi.nlm.nih.gov/24097860/ PubMed]
-=Relapsed=
+==Cladribine & Rituximab {{#subobject:5f1038|Regimen=1}}==
-==Rituximab==
+<div class="toccolours" style="background-color:#eeeeee">
-===Regimen, Nieva et al. Blood 2003===
+===Regimen {{#subobject:821f7d|Variant=1}}===
-Level of Evidence:
+{| class="wikitable sortable" style="width: 60%; text-align:center;"
-<span
+!style="width: 33%"|Study
-style="background:#EEEE00;
+!style="width: 33%"|Dates of enrollment
-padding:3px 6px 3px 6px;
+!style="width: 33%"|[[Levels_of_Evidence#Evidence|Evidence]]
-border-color:black;
+|-
-border-width:2px;
+|[https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4081440/ Ravandi et al. 2011]
-border-style:solid;">Phase II</span>
+|2004-2009
+|style="background-color:#ffffbe"|Phase 2, fewer than 20 pts in this cohort
+|-
+|}
+''Note: 28 days after administration of cladribine, bone marrow examination was repeated, and then rituximab was started.''
+<div class="toccolours" style="background-color:#b3e2cd">
+====Chemotherapy, first portion (course 1)====
+*[[Cladribine (Leustatin)]] 5.6 mg/m<sup>2</sup> IV over 2 hours once per day on days 1 to 5
+====Supportive therapy, first portion (course 1)====
+''All were administered at the discretion of the treating physician.''
+*[[Levofloxacin (Levaquin)]]
+*[[Valacyclovir (Valtrex)]]
+*[[Fluconazole (Diflucan)]]
+*[[Filgrastim (Neupogen)]]
+====Targeted therapy, second portion (cycles 2 to 9)====
+*[[Rituximab (Rituxan)]] 375 mg/m<sup>2</sup> IV once on day 1
+'''28-day course, then 7-day cycle for 8 cycles'''
+</div></div>
+===References===
+# Ravandi F, O'Brien S, Jorgensen J, Pierce S, Faderl S, Ferrajoli A, Koller C, Challagundla P, York S, Brandt M, Luthra R, Burger J, Thomas D, Keating M, Kantarjian H. Phase 2 study of cladribine followed by rituximab in patients with hairy cell leukemia. Blood. 2011 Oct 6;118(14):3818-23. Epub 2011 Aug 5. [https://doi.org/10.1182/blood-2011-04-351502 link to original article] '''contains dosing details in manuscript''' [https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4081440/ link to PMC article] [https://pubmed.ncbi.nlm.nih.gov/21821712/ PubMed]
+## '''Update:''' Chihara D, Kantarjian H, O'Brien S, Jorgensen J, Pierce S, Faderl S, Ferrajoli A, Poku R, Jain P, Thompson P, Brandt M, Luthra R, Burger J, Keating M, Ravandi F. Long-term durable remission by cladribine followed by rituximab in patients with hairy cell leukaemia: update of a phase II trial. Br J Haematol. 2016 Sep;174(5):760-6. Epub 2016 Jun 15. [https://doi.org/10.1111/bjh.14129 link to original article] [https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5396841/ link to PMC article] [https://pubmed.ncbi.nlm.nih.gov/27301277/ PubMed]
+==Interferon alfa-2a monotherapy {{#subobject:jgu3f0|Regimen=1}}==
+<div class="toccolours" style="background-color:#ee6b6e">
+===Regimen {{#subobject:3d3ig9|Variant=1}}===
+{| class="wikitable" style="width: 60%; text-align:center;"
+!style="width: 33%"|Study
+!style="width: 33%"|Dates of enrollment
+!style="width: 33%"|[[Levels_of_Evidence#Evidence|Evidence]]
+|-
+|[https://doi.org/10.1182/blood.V68.2.493.493 Quesada et al. 1986 (N2752)]
+|1984-NR
+| style="background-color:#91cf61" |Non-randomized (RT)
+|-
+|}
+''Note: This is of historic interest. It is not completely clear whether the cited study is the same as N2752, which is cited by the FDA in the package insert.''
+<div class="toccolours" style="background-color:#b3e2cd">
+====Immunotherapy====
+*[[Interferon alfa-2a (Roferon-A)]]
+</div></div>
+===References===
+#'''N2752:''' Quesada JR, Hersh EM, Manning J, Reuben J, Keating M, Schnipper E, Itri L, Gutterman JU. Treatment of hairy cell leukemia with recombinant alpha-interferon. Blood. 1986 Aug;68(2):493-7. [https://doi.org/10.1182/blood.V68.2.493.493 link to original article] [https://pubmed.ncbi.nlm.nih.gov/3730612/ PubMed]
+==Interferon alfa-2b monotherapy {{#subobject:ca73f0|Regimen=1}}==
+<div class="toccolours" style="background-color:#ee6b6e">
+===Regimen {{#subobject:3d3149|Variant=1}}===
+{| class="wikitable sortable" style="width: 100%; text-align:center;"
+!style="width: 17%"|Study
+!style="width: 15%"|Dates of enrollment
+!style="width: 17%"|[[Levels_of_Evidence#Evidence|Evidence]]
+!style="width: 17%"|Comparator
+!style="width: 17%"|[[Levels_of_Evidence#Comparative_efficacy|Comparative Efficacy]]
+!style="width: 17%"|[[Levels_of_Evidence#Toxicity|Comparative Toxicity]]
+|-
+|[https://doi.org/10.1056/NEJM198401053100104 Quesada et al. 1984]
+|NR
+|style="background-color:#ffffbe"|Pilot, fewer than 20 pts
+| style="background-color:#d3d3d3" |
+| style="background-color:#d3d3d3" |
+| style="background-color:#d3d3d3" |
+|-
+|[https://doi.org/10.1200/JCO.1986.4.6.900 Golomb et al. 1986]
+|1983-1984
+| style="background-color:#91cf61" |Non-randomized
+| style="background-color:#d3d3d3" |
+| style="background-color:#d3d3d3" |
+| style="background-color:#d3d3d3" |
+|-
+|[https://doi.org/10.1093/jnci/80.5.369 Golomb et al. 1988]
+|1985-1987
+| style="background-color:#1a9851" |Randomized (E-RT-de-esc)
+|[[#Interferon_alfa-2b_monotherapy|Inteferon alfa-2b]] x 18 mo
+|
+| style="background-color:#91cf60" |Seems to have lower incidence of fatigue
+|-
+|}
+''Note: This is of historic interest. Most patients had previously undergone splenectomy.''
+<div class="toccolours" style="background-color:#b3e2cd">
+====Immunotherapy====
+*[[Interferon alfa-2b (Intron-A)]] 2,000,000 units SC three times per week
+'''12-month course'''
+</div></div>
+===References===
+# Quesada JR, Reuben J, Manning JT, Hersh EM, Gutterman JU. Alpha interferon for induction of remission in hairy-cell leukemia. N Engl J Med. 1984 Jan 5;310(1):15-8. [https://doi.org/10.1056/NEJM198401053100104 link to original article] [https://pubmed.ncbi.nlm.nih.gov/6689734/ PubMed]
+# Golomb HM, Jacobs A, Fefer A, Ozer H, Thompson J, Portlock C, Ratain M, Golde D, Vardiman J, Burke JS, Brady J, Bonnem E, Spiegel R. Alpha-2 interferon therapy of hairy-cell leukemia: a multicenter study of 64 patients. J Clin Oncol. 1986 Jun;4(6):900-5. [https://doi.org/10.1200/JCO.1986.4.6.900 link to original article] [https://pubmed.ncbi.nlm.nih.gov/3519880/ PubMed]
+# Golomb HM, Ratain MJ, Fefer A, Thompson J, Golde DW, Ozer H, Portlock C, Silber R, Rappeport J, Bonnem E, Spiegel R, Tensen L, Burke JS, Vardiman JW. Randomized study of the duration of treatment with interferon alfa-2B in patients with hairy cell leukemia. J Natl Cancer Inst. 1988 May 4;80(5):369-73. [https://doi.org/10.1093/jnci/80.5.369 link to original article] [https://pubmed.ncbi.nlm.nih.gov/3282078/ PubMed]
-*[[Rituximab (Rituxan)]] 375 mg/m2 IV weekly for 4 consecutive weeks
+==Moxetumomab pasudotox monotherapy {{#subobject:06ef3f|Regimen=1}}==
-**Initial infusions were given at 50 mg per hour for the first 30 minutes, and if no toxicity was observed, the infusion rate was escalated at 30-minute intervals to a peak infusion rate of 300 mg per hour.
+<div class="toccolours" style="background-color:#eeeeee">
+===Regimen {{#subobject:3d2dea|Variant=1}}===
+{| class="wikitable" style="color:white; background-color:#404040"
+|<small>'''FDA-recommended dose'''</small>
+|-
+|}
+{| class="wikitable" style="width: 60%; text-align:center;"
+!style="width: 33%"|Study
+!style="width: 33%"|Dates of enrollment
+!style="width: 33%"|[[Levels_of_Evidence#Evidence|Evidence]]
+|-
+|[https://www.ncbi.nlm.nih.gov/pmc/articles/pmc6087717/ Kreitman et al. 2018 (Study 1053)]
+|2013-2016
+| style="background-color:#91cf61" |Phase 2 (RT)
+|-
+|}
+<div class="toccolours" style="background-color:#b3e2cd">
+====Targeted therapy====
+*[[Moxetumomab pasudotox (Lumoxiti)]] 0.04 mg/kg IV over 30 minutes once per day on days 1, 3, 5
+'''28-day cycle for up to 6 cycles'''
+</div></div>
+===References===
+# '''Study 1053:''' Kreitman RJ, Dearden C, Zinzani PL, Delgado J, Karlin L, Robak T, Gladstone DE, le Coutre P, Dietrich S, Gotic M, Larratt L, Offner F, Schiller G, Swords R, Bacon L, Bocchia M, Bouabdallah K, Breems DA, Cortelezzi A, Dinner S, Doubek M, Gjertsen BT, Gobbi M, Hellmann A, Lepretre S, Maloisel F, Ravandi F, Rousselot P, Rummel M, Siddiqi T, Tadmor T, Troussard X, Yi CA, Saglio G, Roboz GJ, Balic K, Standifer N, He P, Marshall S, Wilson W, Pastan I, Yao NS, Giles F. Moxetumomab pasudotox in relapsed/refractory hairy cell leukemia. Leukemia. 2018 Aug;32(8):1768-1777. Epub 2018 Jul 20. [https://doi.org/10.1038/s41375-018-0210-1 link to original article] [https://www.ncbi.nlm.nih.gov/pmc/articles/pmc6087717/ link to PMC article] '''contains dosing details in abstract''' [https://pubmed.ncbi.nlm.nih.gov/30030507/ PubMed] [https://clinicaltrials.gov/study/NCT01829711 NCT01829711]
+## '''Update:''' Kreitman RJ, Dearden C, Zinzani PL, Delgado J, Robak T, le Coutre PD, Gjertsen BT, Troussard X, Roboz GJ, Karlin L, Gladstone DE, Kuptsova-Clarkson N, Liu S, Patel P, Rotolo F, Mitry E, Pastan I, Giles F; Study 1053 investigators. Moxetumomab pasudotox in heavily pre-treated patients with relapsed/refractory hairy cell leukemia (HCL): long-term follow-up from the pivotal trial. J Hematol Oncol. 2021 Feb 24;14(1):35. [https://doi.org/10.1186/s13045-020-01004-y link to original article] [https://www.ncbi.nlm.nih.gov/pmc/articles/pmc7905554/ link to PMC article] [https://pubmed.ncbi.nlm.nih.gov/33627164/ PubMed]
+==Rituximab monotherapy {{#subobject:9b852|Regimen=1}}==
+<div class="toccolours" style="background-color:#eeeeee">
+===Regimen {{#subobject:929d4a|Variant=1}}===
+{| class="wikitable sortable" style="width: 60%; text-align:center;"
+!style="width: 33%"|Study
+!style="width: 33%"|Dates of enrollment
+!style="width: 33%"|[[Levels_of_Evidence#Evidence|Evidence]]
+|-
+|[https://doi.org/10.1182/blood-2003-01-0014 Nieva et al. 2003]
+|2000-2002
+|style="background-color:#91cf61"|Phase 2
+|-
+|}
+<div class="toccolours" style="background-color:#b3e2cd">
+====Targeted therapy====
+*[[Rituximab (Rituxan)]] 375 mg/m<sup>2</sup> IV once per day on days 1, 8, 15, 22
+====Supportive therapy====
+*[[Allopurinol (Zyloprim)]] 300 mg PO once per day on days 1 to 7
+'''4-week course'''
+</div></div>
+===References===
+# Nieva J, Bethel K, Saven A. Phase 2 study of rituximab in the treatment of cladribine-failed patients with hairy cell leukemia. Blood. 2003 Aug 1;102(3):810-3. Epub 2003 Mar 27. [https://doi.org/10.1182/blood-2003-01-0014 link to original article] '''contains dosing details in manuscript''' [https://pubmed.ncbi.nlm.nih.gov/12663446/ PubMed]
-Supportive medications:
+==Vemurafenib monotherapy {{#subobject:c957e9|Regimen=1}}==
-*Allopurinol (Aloprim) 300 mg PO once per day for the first 7 days of treatment
+<div class="toccolours" style="background-color:#eeeeee">
+===Regimen {{#subobject:5b6425|Variant=1}}===
+{| class="wikitable sortable" style="width: 80%; text-align:center;"
+!style="width: 25%"|Study
+!style="width: 25%"|Dates of enrollment
+!style="width: 25%"|[[Levels_of_Evidence#Evidence|Evidence]]
+!style="width: 25%"|[[Levels_of_Evidence#Efficacy|Efficacy]]
+|-
+|[https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4811324/ Tiacci et al. 2015 (MSKCC 12-200)]
+|2013-2015
+|style="background-color:#91cf61"|Phase 2
+| style="background-color:#f7fcfd" |ORR: 96-100%
+|-
+|[https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4811324/ Tiacci et al. 2015 (HCL-PG01)]
+|2013-2015
+|style="background-color:#91cf61"|Phase 2
+| style="background-color:#f7fcfd" |ORR: 96-100%
+|-
+|}
+''Note: a pooled analysis of two phase 2 trials is reported in Tiacci et al. 2015; they have slightly different durations - please see original text for details.''
+<div class="toccolours" style="background-color:#b3e2cd">
+====Targeted therapy====
+*[[Vemurafenib (Zelboraf)]] 960 mg PO twice per day on days 1 to 28
+'''28-day cycle for up to 4 cycles (HCL-PG01) or 6 cycles (MSKCC 12-200)'''
+</div></div>
+===References===
+# '''MSKCC 12-200:''' Tiacci E, Park JH, De Carolis L, Chung SS, Broccoli A, Scott S, Zaja F, Devlin S, Pulsoni A, Chung YR, Cimminiello M, Kim E, Rossi D, Stone RM, Motta G, Saven A, Varettoni M, Altman JK, Anastasia A, Grever MR, Ambrosetti A, Rai KR, Fraticelli V, Lacouture ME, Carella AM, Levine RL, Leoni P, Rambaldi A, Falzetti F, Ascani S, Capponi M, Martelli MP, Park CY, Pileri SA, Rosen N, Foà R, Berger MF, Zinzani PL, Abdel-Wahab O, Falini B, Tallman MS. Targeting mutant BRAF in relapsed or refractory hairy-cell leukemia. N Engl J Med. 2015 Oct 29;373(18):1733-47. Epub 2015 Sep 9. [https://doi.org/10.1056/NEJMoa1506583 link to original article] '''contains dosing details in manuscript''' [https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4811324/ link to PMC article] [https://pubmed.ncbi.nlm.nih.gov/26352686/ PubMed] [https://clinicaltrials.gov/study/NCT01711632 NCT01711632]
+# '''HCL-PG01:''' Tiacci E, Park JH, De Carolis L, Chung SS, Broccoli A, Scott S, Zaja F, Devlin S, Pulsoni A, Chung YR, Cimminiello M, Kim E, Rossi D, Stone RM, Motta G, Saven A, Varettoni M, Altman JK, Anastasia A, Grever MR, Ambrosetti A, Rai KR, Fraticelli V, Lacouture ME, Carella AM, Levine RL, Leoni P, Rambaldi A, Falzetti F, Ascani S, Capponi M, Martelli MP, Park CY, Pileri SA, Rosen N, Foà R, Berger MF, Zinzani PL, Abdel-Wahab O, Falini B, Tallman MS. Targeting mutant BRAF in relapsed or refractory hairy-cell leukemia. N Engl J Med. 2015 Oct 29;373(18):1733-47. Epub 2015 Sep 9. [https://doi.org/10.1056/NEJMoa1506583 link to original article] '''contains dosing details in manuscript''' [https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4811324/ link to PMC article] [https://pubmed.ncbi.nlm.nih.gov/26352686/ PubMed] EudraCT 2011-005487-13
+=Hairy cell leukemia variant (HCLv), all lines of therapy=
+''Note: this is a distinct disease process from hairy cell leukemia, but will be included on this page for now given the similarities. We may create a dedicated page in the future.''
+==Cladribine & Rituximab {{#subobject:9989c5|Regimen=1}}==
+<div class="toccolours" style="background-color:#eeeeee">
+===Regimen {{#subobject:e39d58|Variant=1}}===
+{| class="wikitable sortable" style="width: 60%; text-align:center;"
+!style="width: 33%"|Study
+!style="width: 33%"|Dates of enrollment
+!style="width: 33%"|[[Levels_of_Evidence#Evidence|Evidence]]
+|-
+|[https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4081440/ Ravandi et al. 2011]
+|2004-2009
+|style="background-color:#ffffbe"|Phase 2, fewer than 20 pts in this cohort
+|-
+|}
+''Note: 28 days after administration of cladribine, bone marrow examination was repeated, and then rituximab was started.''
+<div class="toccolours" style="background-color:#b3e2cd">
+====Chemotherapy, first portion (course 1)====
+*[[Cladribine (Leustatin)]] 5.6 mg/m<sup>2</sup> IV over 2 hours once per day on days 1 to 5
+====Supportive therapy, first portion (course 1)====
+''All were administered at the discretion of the treating physician.''
+*[[Levofloxacin (Levaquin)]]
+*[[Valacyclovir (Valtrex)]]
+*[[Fluconazole (Diflucan)]]
+*[[Filgrastim (Neupogen)]]
+====Targeted therapy, second portion (cycles 2 to 9)====
+*[[Rituximab (Rituxan)]] 375 mg/m<sup>2</sup> IV once on day 1
+'''28-day course, then 7-day cycle for 8 cycles'''
+</div></div>
 ===References===
-# Nieva J, Bethel K, Saven A. Phase 2 study of rituximab in the treatment of cladribine-failed patients with hairy cell leukemia. Blood. 2003 Aug 1;102(3):810-3. [http://bloodjournal.hematologylibrary.org/content/102/3/810.long link to original article] '''contains verified protocol''' [http://www.ncbi.nlm.nih.gov/pubmed/12663446 PubMed]
+# Ravandi F, O'Brien S, Jorgensen J, Pierce S, Faderl S, Ferrajoli A, Koller C, Challagundla P, York S, Brandt M, Luthra R, Burger J, Thomas D, Keating M, Kantarjian H. Phase 2 study of cladribine followed by rituximab in patients with hairy cell leukemia. Blood. 2011 Oct 6;118(14):3818-23. Epub 2011 Aug 5. [https://doi.org/10.1182/blood-2011-04-351502 link to original article] '''contains dosing details in manuscript''' [https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4081440/ link to PMC article] [https://pubmed.ncbi.nlm.nih.gov/21821712/ PubMed]
+## '''Update:''' Chihara D, Kantarjian H, O'Brien S, Jorgensen J, Pierce S, Faderl S, Ferrajoli A, Poku R, Jain P, Thompson P, Brandt M, Luthra R, Burger J, Keating M, Ravandi F. Long-term durable remission by cladribine followed by rituximab in patients with hairy cell leukaemia: update of a phase II trial. Br J Haematol. 2016 Sep;174(5):760-6. Epub 2016 Jun 15. [https://doi.org/10.1111/bjh.14129 link to original article] [https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5396841/ link to PMC article] [https://pubmed.ncbi.nlm.nih.gov/27301277/ PubMed]
+[[Category:Hairy cell leukemia regimens]]
+[[Category:Disease-specific pages]]
+[[Category:Indolent lymphomas]]

Difference between revisions of "Hairy cell leukemia"

Latest revision as of 01:41, 26 June 2024

Guidelines

ESMO

Hairy Cell Leukemia Foundation

NCCN

Untreated

Cladribine monotherapy

Regimen variant #1, 0.09 mg/kg/day x 7 days

Chemotherapy

Regimen variant #2, 0.1 mg/kg/day x 7 days

Chemotherapy

Regimen variant #3, 0.12 mg/kg/day x 5 days

Chemotherapy

Regimen variant #4, 0.14 mg/kg/day x 5 days

Chemotherapy

Regimen variant #5, 0.15 mg/kg/day x 5 days

Chemotherapy

References

Cladribine & Rituximab

Regimen

Chemotherapy, first portion (course 1)

Supportive therapy, first portion (course 1)

Targeted therapy, second portion (cycles 2 to 9)

References

Interferon alfa-2a monotherapy

Regimen

Immunotherapy

References

Pentostatin monotherapy

Regimen variant #1

Chemotherapy

Regimen variant #2

Chemotherapy

Supportive therapy

Dose and schedule modifications

References

Relapsed or refractory

Bendamustine & Rituximab (BR)

Regimen

Chemotherapy

Targeted therapy

References

Cladribine & Rituximab

Regimen

Chemotherapy, first portion (course 1)

Supportive therapy, first portion (course 1)

Targeted therapy, second portion (cycles 2 to 9)

References

Interferon alfa-2a monotherapy

Regimen

Immunotherapy

References

Interferon alfa-2b monotherapy

Regimen

Immunotherapy

References

Moxetumomab pasudotox monotherapy

Regimen

Targeted therapy

References

Rituximab monotherapy

Regimen

Targeted therapy

Supportive therapy

References

Vemurafenib monotherapy

Regimen

Targeted therapy

References

Hairy cell leukemia variant (HCLv), all lines of therapy

Cladribine & Rituximab

Regimen

Chemotherapy, first portion (course 1)

Supportive therapy, first portion (course 1)

Targeted therapy, second portion (cycles 2 to 9)

References

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