Difference between revisions of "Langerhans cell histiocytosis"
Jump to navigation
Jump to search
Warner-admin (talk | contribs) m (Text replacement - "Cytarabine (Cytosar)" to "Cytarabine (Ara-C)") |
m |
||
| Line 3: | Line 3: | ||
|- | |- | ||
|style="background-color:#F0F0F0"|[[File:Ronaldgo.jpg|frameless|upright=0.3|center]] | |style="background-color:#F0F0F0"|[[File:Ronaldgo.jpg|frameless|upright=0.3|center]] | ||
| − | |<big>[[User:Ronaldgo|Ronald S. Go, MD]]<br>Rochester, MN</big> | + | |<big>[[User:Ronaldgo|Ronald S. Go, MD]]<br>Mayo Clinic<br>Rochester, MN</big> |
|- | |- | ||
|} | |} | ||
Revision as of 04:07, 31 January 2019
| Section editor | |
|---|---|
 |
Ronald S. Go, MD Mayo Clinic Rochester, MN |
11 regimens on this page
11 variants on this page
|
Note: the majority of the regimens on this page are intended for multi-system Langerhans cell histiocytosis (MS-LCH); many patients with other forms of LCH, such as pulmonary (PLCH) do not necessarily need antineoplastic treatment.
Guidelines
Euro-Histio-Net
- 2013: Management of adult patients with Langerhans cell histiocytosis: Recommendations from an expert panel on behalf of Euro-Histio-Net
- 2013: Langerhans cell histiocytosis (LCH): Guidelines for diagnosis, clinical work-up, and treatment for patients till the age of 18 years
Untreated
Etoposide & Prednisone
| back to top |
Regimen
| Study | Evidence | Comparator | Efficacy |
|---|---|---|---|
| Gadner et al. 2001 (LCH-I) | Phase III (E) | Vinblastine & Prednisone | Seems not superior |
To be completed
Chemotherapy
References
- Gadner H, Grois N, Arico M, Broadbent V, Ceci A, Jakobson A, Komp D, Michaelis J, Nicholson S, Pötschger U, Pritchard J, Ladisch S; Histiocyte Society. A randomized trial of treatment for multisystem Langerhans' cell histiocytosis. J Pediatr. 2001 May;138(5):728-34. Erratum in: J Pediatr 2001 Jul;139(1):170. link to original article PubMed
Etoposide, Vinblastine, Prednisone
| back to top |
Regimen
| Study | Evidence | Comparator | Efficacy |
|---|---|---|---|
| Gadner et al. 2007 (LCH-II) | Phase III (E) | Vinblastine & Prednisone | Seems not superior |
Total duration of therapy is 24 weeks.
Chemotherapy, initial
- Etoposide (Vepesid) 150 mg/m2 IV over 60 minutes once per week
- Vinblastine (Velban) 6 mg/m2 IV bolus once per week
- Prednisone (Sterapred) 40 mg/m2/day split three times per day for 4 weeks, then tapering off over 2 weeks
6-week course, followed by:
Chemotherapy, continuation
- Etoposide (Vepesid) 150 mg/m2 IV over 60 minutes once on day 1
- Vinblastine (Velban) 6 mg/m2 IV bolus once on day 1
- Prednisone (Sterapred) 40 mg/m2/day split three times per day on days 1 to 5
21-day cycle for 6 cycles
References
- Gadner H, Grois N, Pötschger U, Minkov M, Aricò M, Braier J, Broadbent V, Donadieu J, Henter JI, McCarter R, Ladisch S; Histiocyte Society. Improved outcome in multisystem Langerhans cell histiocytosis is associated with therapy intensification. Blood. 2008 Mar 1;111(5):2556-62. Epub 2007 Dec 18. link to original article contains verified protocol PubMed
Methotrexate, Vinblastine, Prednisone
| back to top |
Regimen
| Study | Evidence | Comparator | Efficacy |
|---|---|---|---|
| Gadner et al. 2013 (LCH-III) | Phase III (E) | Vinblastine & Prednisone | Seems not superior |
To be completed
Chemotherapy
References
- Gadner H, Minkov M, Grois N, Pötschger U, Thiem E, Aricò M, Astigarraga I, Braier J, Donadieu J, Henter JI, Janka-Schaub G, McClain KL, Weitzman S, Windebank K, Ladisch S; Histiocyte Society. Therapy prolongation improves outcome in multisystem Langerhans cell histiocytosis. Blood. 2013 Jun 20;121(25):5006-14. Epub 2013 Apr 15. link to original article PubMed
Vinblastine & Prednisone
| back to top |
Regimen
| Study | Evidence | Comparator | Efficacy |
|---|---|---|---|
| Gadner et al. 2001 (LCH-I) | Phase III (E) | Etoposide & Prednisone | Seems not superior |
| Gadner et al. 2007 (LCH-II) | Phase III (C) | Etoposide, Vinblastine, Prednisone | Seems not superior |
| Gadner et al. 2013 (LCH-III) | Phase III (C) | Methotrexate, Vinblastine, Prednisone | Seems not superior |
Total duration of therapy is 24 weeks, in LCH-II.
Chemotherapy, initial
- Vinblastine (Velban) 6 mg/m2 IV bolus once per week
- Prednisone (Sterapred) 40 mg/m2/day split three times per day for 4 weeks, then tapering off over 2 weeks
6-week course, followed by:
Chemotherapy, continuation
- Vinblastine (Velban) 6 mg/m2 IV bolus once on day 1
- Prednisone (Sterapred) 40 mg/m2/day split three times per day on days 1 to 5
21-day cycle for 6 cycles
References
- Gadner H, Grois N, Arico M, Broadbent V, Ceci A, Jakobson A, Komp D, Michaelis J, Nicholson S, Pötschger U, Pritchard J, Ladisch S; Histiocyte Society. A randomized trial of treatment for multisystem Langerhans' cell histiocytosis. J Pediatr. 2001 May;138(5):728-34. Erratum in: J Pediatr 2001 Jul;139(1):170. link to original article PubMed
- Gadner H, Grois N, Pötschger U, Minkov M, Aricò M, Braier J, Broadbent V, Donadieu J, Henter JI, McCarter R, Ladisch S; Histiocyte Society. Improved outcome in multisystem Langerhans cell histiocytosis is associated with therapy intensification. Blood. 2008 Mar 1;111(5):2556-62. Epub 2007 Dec 18. link to original article contains verified protocol PubMed
- Gadner H, Minkov M, Grois N, Pötschger U, Thiem E, Aricò M, Astigarraga I, Braier J, Donadieu J, Henter JI, Janka-Schaub G, McClain KL, Weitzman S, Windebank K, Ladisch S; Histiocyte Society. Therapy prolongation improves outcome in multisystem Langerhans cell histiocytosis. Blood. 2013 Jun 20;121(25):5006-14. Epub 2013 Apr 15. link to original article PubMed
Relapsed or refractory
Cladribine monotherapy
| back to top |
Regimen
| Study | Evidence |
|---|---|
| Weitzman et al. 2009 (LCH-S-98) | Phase II |
To be completed
Chemotherapy
References
- LCH-S-98: Weitzman S, Braier J, Donadieu J, Egeler RM, Grois N, Ladisch S, Pötschger U, Webb D, Whitlock J, Arceci RJ. 2'-Chlorodeoxyadenosine (2-CdA) as salvage therapy for Langerhans cell histiocytosis (LCH): results of the LCH-S-98 protocol of the Histiocyte Society. Pediatr Blood Cancer. 2009 Dec 15;53(7):1271-6. link to original article PubMed
Cladribine & Cytarabine
| back to top |
Regimen
| Study | Evidence |
|---|---|
| Donadieu et al. 2015 | Phase II |
Chemotherapy
- Cladribine (Leustatin) 9 mg/m2 IV over 2 hours once per day on days 2 to 6, should not be administered simultaneously with cytarabine
- Children weighing less than 10 kg received a dose of 0.3 mg/kg/day
- Cytarabine (Ara-C) 500 mg/m2 IV over 2 hours twice per day on days 1 to 5, should not be administered simultaneously with cladribine
35-day cycle for 2 or more cycles
Subsequent treatment
- Patients with a "good response": Cladribine consolidation
References
- Donadieu J, Bernard F, van Noesel M, Barkaoui M, Bardet O, Mura R, Arico M, Piguet C, Gandemer V, Armari Alla C, Clausen N, Jeziorski E, Lambilliote A, Weitzman S, Henter JI, Van Den Bos C; Salvage Group of the Histiocyte Society. Cladribine and cytarabine in refractory multisystem Langerhans cell histiocytosis: results of an international phase 2 study. Blood. 2015 Sep 17;126(12):1415-23. Epub 2015 Jul 20. link to original article contains verified protocol link to PMC article PubMed
Vemurafenib monotherapy
| back to top |
Regimen
| Study | Evidence |
|---|---|
| Hyman et al. 2015 (VE-BASKET) | Phase II, <20 pts in this arm |
This was part of a basket trial, all patients had BRAF p.V600E mutations. A minority (7/18, 39%) of participants were untreated.
Chemotherapy
- Vemurafenib (Zelboraf) 960 mg PO twice per day
Duration of treatment not specified
References
- VE-BASKET: Hyman DM, Puzanov I, Subbiah V, Faris JE, Chau I, Blay JY, Wolf J, Raje NS, Diamond EL, Hollebecque A, Gervais R, Elez-Fernandez ME, Italiano A, Hofheinz RD, Hidalgo M, Chan E, Schuler M, Lasserre SF, Makrutzki M, Sirzen F, Veronese ML, Tabernero J, Baselga J. Vemurafenib in multiple nonmelanoma cancers with BRAF V600 mutations. N Engl J Med. 2015 Aug 20;373(8):726-736. link to original article contains verified protocol link to PMC article PubMed
Relapsed or refractory, consolidation
Cladribine monotherapy
| back to top |
Regimen
| Study | Evidence |
|---|---|
| Donadieu et al. 2015 | Phase II |
Preceding treatment
Chemotherapy
- Cladribine (Leustatin) 5 mg/m2 IV once per day on days 1 to 3
21-day cycle for 2 cycles
Subsequent treatment
References
- Donadieu J, Bernard F, van Noesel M, Barkaoui M, Bardet O, Mura R, Arico M, Piguet C, Gandemer V, Armari Alla C, Clausen N, Jeziorski E, Lambilliote A, Weitzman S, Henter JI, Van Den Bos C; Salvage Group of the Histiocyte Society. Cladribine and cytarabine in refractory multisystem Langerhans cell histiocytosis: results of an international phase 2 study. Blood. 2015 Sep 17;126(12):1415-23. Epub 2015 Jul 20. link to original article contains verified protocol link to PMC article PubMed
Relapsed or refractory, maintenance
Mercaptopurine, Methotrexate, Vinblastine, Prednisolone
| back to top |
Regimen
| Study | Evidence |
|---|---|
| Donadieu et al. 2015 | Phase II |
Preceding treatment
- Cladribine x 2
Chemotherapy
- Mercaptopurine (6-MP) 50 mg/m2 PO once per day
- Methotrexate (MTX) 20 mg/m2 PO once per week
- Vinblastine (Velban) as follows:
- Months 1 to 6: 6 mg/m2 IV once every 2 weeks
- Prednisolone (Millipred) as follows:
- Months 1 to 6: 40 mg/m2 PO once per day on days 1 to 5 of every 2nd week
18 months of maintenance
References
- Donadieu J, Bernard F, van Noesel M, Barkaoui M, Bardet O, Mura R, Arico M, Piguet C, Gandemer V, Armari Alla C, Clausen N, Jeziorski E, Lambilliote A, Weitzman S, Henter JI, Van Den Bos C; Salvage Group of the Histiocyte Society. Cladribine and cytarabine in refractory multisystem Langerhans cell histiocytosis: results of an international phase 2 study. Blood. 2015 Sep 17;126(12):1415-23. Epub 2015 Jul 20. link to original article contains verified protocol link to PMC article PubMed