Difference between revisions of "Hypereosinophilic syndrome"
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Warner-admin (talk | contribs) m (Text replacement - "'''contains dosing details in abstract'''" to "'''dosing details in abstract have been reviewed by our editors'''") |
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− | + | <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"> | |
− | + | [[#top|Back to Top]] | |
− | + | </div> | |
− | + | {{#lst:Editorial board transclusions|mpn}} | |
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<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> | <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> | ||
|} | |} | ||
+ | ''For placebo or observational studies in this condition, please visit [[Hypereosinophilic syndrome - null regimens|this page]].'' | ||
{{TOC limit|limit=3}} | {{TOC limit|limit=3}} | ||
=Guidelines= | =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.''' | ||
==NCCN== | ==NCCN== | ||
− | *[https://www.nccn.org/ | + | *[https://www.nccn.org/guidelines/guidelines-detail?category=1&id=1505 NCCN Guidelines - Myeloid/Lymphoid Neoplasms with Eosinophilia and Tyrosine Kinase Fusion Genes] |
=All lines of therapy= | =All lines of therapy= | ||
==Alemtuzumab monotherapy {{#subobject:9207db|Regimen=1}}== | ==Alemtuzumab monotherapy {{#subobject:9207db|Regimen=1}}== | ||
− | + | <div class="toccolours" style="background-color:#eeeeee"> | |
− | |||
===Regimen {{#subobject:45d126|Variant=1}}=== | ===Regimen {{#subobject:45d126|Variant=1}}=== | ||
{| class="wikitable" style="width: 40%; text-align:center;" | {| class="wikitable" style="width: 40%; text-align:center;" | ||
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!style="width: 25%"|[[Levels_of_Evidence#Evidence|Evidence]] | !style="width: 25%"|[[Levels_of_Evidence#Evidence|Evidence]] | ||
|- | |- | ||
− | |[ | + | |[https://doi.org/10.1016/j.jaci.2009.01.069 Wagner et al. 2009] |
|style="background-color:#ffffbe"|Case report | |style="background-color:#ffffbe"|Case report | ||
|- | |- | ||
|} | |} | ||
''Note: treatment is to be given bi-weekly, exact days not specified; below is an example. Duration also not specified.'' | ''Note: treatment is to be given bi-weekly, exact days not specified; below is an example. Duration also not specified.'' | ||
+ | <div class="toccolours" style="background-color:#b3e2cd"> | ||
====Targeted therapy==== | ====Targeted therapy==== | ||
*[[Alemtuzumab (Campath)]] 30 mg IV once per day on days 1 & 4 | *[[Alemtuzumab (Campath)]] 30 mg IV once per day on days 1 & 4 | ||
− | |||
'''7-day cycles''' | '''7-day cycles''' | ||
− | + | </div></div> | |
===References=== | ===References=== | ||
− | # '''Case report:''' Wagner LA, Speckart S, Cutter B, Gleich GJ. Treatment of FIP1L1/PDGFRA-negative hypereosinophilic syndrome with alemtuzumab, an anti-CD52 antibody. J Allergy Clin Immunol. 2009 Jun;123(6):1407-8. Epub 2009 Apr 1. [ | + | # '''Case report:''' Wagner LA, Speckart S, Cutter B, Gleich GJ. Treatment of FIP1L1/PDGFRA-negative hypereosinophilic syndrome with alemtuzumab, an anti-CD52 antibody. J Allergy Clin Immunol. 2009 Jun;123(6):1407-8. Epub 2009 Apr 1. [https://doi.org/10.1016/j.jaci.2009.01.069 link to original article] '''dosing details in abstract have been reviewed by our editors''' [https://pubmed.ncbi.nlm.nih.gov/19342084/ PubMed] |
− | # '''Retrospective:''' Strati P, Cortes J, Faderl S, Kantarjian H, Verstovsek S. Long-term follow-up of patients with hypereosinophilic syndrome treated with Alemtuzumab, an anti-CD52 antibody. Clin Lymphoma Myeloma Leuk. 2013 Jun;13(3):287-91. Epub 2012 Nov 1. [ | + | # '''Retrospective:''' Strati P, Cortes J, Faderl S, Kantarjian H, Verstovsek S. Long-term follow-up of patients with hypereosinophilic syndrome treated with Alemtuzumab, an anti-CD52 antibody. Clin Lymphoma Myeloma Leuk. 2013 Jun;13(3):287-91. Epub 2012 Nov 1. [https://doi.org/10.1016/j.clml.2012.09.018 link to original article] [https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4445419/ link to PMC article] [https://pubmed.ncbi.nlm.nih.gov/23123105/ PubMed] |
− | |||
==Benralizumab monotherapy {{#subobject:39b68c|Regimen=1}}== | ==Benralizumab monotherapy {{#subobject:39b68c|Regimen=1}}== | ||
− | + | <div class="toccolours" style="background-color:#eeeeee"> | |
===Regimen {{#subobject:2gh77d|Variant=1}}=== | ===Regimen {{#subobject:2gh77d|Variant=1}}=== | ||
{| class="wikitable sortable" style="width: 100%; text-align:center;" | {| class="wikitable sortable" style="width: 100%; text-align:center;" | ||
!style="width: 20%"|Study | !style="width: 20%"|Study | ||
− | !style="width: 20%"| | + | !style="width: 20%"|Dates of enrollment |
!style="width: 20%"|[[Levels_of_Evidence#Evidence|Evidence]] | !style="width: 20%"|[[Levels_of_Evidence#Evidence|Evidence]] | ||
!style="width: 20%"|Comparator | !style="width: 20%"|Comparator | ||
Line 51: | Line 49: | ||
|[https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6557265/ Kuang et al. 2019 (NIAID 14-I-0081)] | |[https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6557265/ Kuang et al. 2019 (NIAID 14-I-0081)] | ||
|2014-2017 | |2014-2017 | ||
− | | style="background-color:#91cf61" |Randomized Phase 2, | + | | style="background-color:#91cf61" |Randomized Phase 2, fewer than 20 pts (E-esc) |
− | |[[# | + | |[[#Placebo|Placebo]] |
| style="background-color:#91cf60" |Seems to have superior primary endpoint | | style="background-color:#91cf60" |Seems to have superior primary endpoint | ||
|- | |- | ||
|} | |} | ||
+ | <div class="toccolours" style="background-color:#b3e2cd"> | ||
====Immunosuppressive therapy==== | ====Immunosuppressive therapy==== | ||
*[[Benralizumab (Fasenra)]] 30 mg SC once on day 1 | *[[Benralizumab (Fasenra)]] 30 mg SC once on day 1 | ||
− | |||
'''28-day cycle for 6 or more cycles''' | '''28-day cycle for 6 or more cycles''' | ||
+ | </div></div> | ||
===References=== | ===References=== | ||
− | # '''NIAID 14-I-0081:''' Kuang FL, Legrand F, Makiya M, Ware J, Wetzler L, Brown T, Magee T, Piligian B, Yoon P, Ellis JH, Sun X, Panch SR, Powers A, Alao H, Kumar S, Quezado M, Yan L, Lee N, Kolbeck R, Newbold P, Goldman M, Fay MP, Khoury P, Maric I, Klion AD. Benralizumab for PDGFRA-negative hypereosinophilic syndrome. N Engl J Med. 2019 Apr 4;380(14):1336-1346. [https://doi.org/10.1056/NEJMoa1812185 link to original article] ''' | + | # '''NIAID 14-I-0081:''' Kuang FL, Legrand F, Makiya M, Ware J, Wetzler L, Brown T, Magee T, Piligian B, Yoon P, Ellis JH, Sun X, Panch SR, Powers A, Alao H, Kumar S, Quezado M, Yan L, Lee N, Kolbeck R, Newbold P, Goldman M, Fay MP, Khoury P, Maric I, Klion AD. Benralizumab for PDGFRA-negative hypereosinophilic syndrome. N Engl J Med. 2019 Apr 4;380(14):1336-1346. [https://doi.org/10.1056/NEJMoa1812185 link to original article] '''dosing details in manuscript have been reviewed by our editors''' [https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6557265/ link to PMC article] [https://pubmed.ncbi.nlm.nih.gov/30943337/ PubMed] [https://clinicaltrials.gov/study/NCT00001406 NCT00001406] |
− | |||
==Cladribine & Cytarabine {{#subobject:b80308|Regimen=1}}== | ==Cladribine & Cytarabine {{#subobject:b80308|Regimen=1}}== | ||
− | + | <div class="toccolours" style="background-color:#eeeeee"> | |
===Regimen {{#subobject:d51fc2|Variant=1}}=== | ===Regimen {{#subobject:d51fc2|Variant=1}}=== | ||
− | {| class="wikitable" style="width: | + | {| class="wikitable sortable" style="width: 60%; text-align:center;" |
− | !style="width: | + | !style="width: 33%"|Study |
− | !style="width: | + | !style="width: 33%"|Dates of enrollment |
+ | !style="width: 33%"|[[Levels_of_Evidence#Evidence|Evidence]] | ||
|- | |- | ||
− | |[https:// | + | |[https://doi.org/10.1002/cncr.21186 Jabbour et al. 2005] |
− | |style="background-color:#ffffbe"|Phase 2, | + | |1999-04 to 2001-04 |
+ | |style="background-color:#ffffbe"|Phase 2, fewer than 20 pts | ||
|- | |- | ||
|} | |} | ||
− | ''Note | + | ''Note: the timing of drug administration is in hours in the manuscript, starting at t = 0 hours.'' |
+ | <div class="toccolours" style="background-color:#b3e2cd"> | ||
====Chemotherapy==== | ====Chemotherapy==== | ||
*[[Cladribine (Leustatin)]] 12 mg/m<sup>2</sup>/day IV continuous infusion over 120 hours, started on day 2 (total dose: 60 mg/m<sup>2</sup>) | *[[Cladribine (Leustatin)]] 12 mg/m<sup>2</sup>/day IV continuous infusion over 120 hours, started on day 2 (total dose: 60 mg/m<sup>2</sup>) | ||
*[[Cytarabine (Ara-C)]] 1000 mg/m<sup>2</sup> IV over 2 hours once per day on days 1, 3, 4, 5, 6 | *[[Cytarabine (Ara-C)]] 1000 mg/m<sup>2</sup> IV over 2 hours once per day on days 1, 3, 4, 5, 6 | ||
− | |||
'''21-day cycle, repeated once if PR/CR; see text for definition of PR''' | '''21-day cycle, repeated once if PR/CR; see text for definition of PR''' | ||
− | + | </div></div> | |
===References=== | ===References=== | ||
− | # Jabbour E, Verstovsek S, Giles F, Gandhi V, Cortes J, O'Brien S, Plunkett W, Garcia-Manero G, Jackson CE, Kantarjian H, Andreeff M. 2-Chlorodeoxyadenosine and cytarabine combination therapy for idiopathic hypereosinophilic syndrome. Cancer. 2005 Aug 1;104(3):541-6. [https:// | + | # Jabbour E, Verstovsek S, Giles F, Gandhi V, Cortes J, O'Brien S, Plunkett W, Garcia-Manero G, Jackson CE, Kantarjian H, Andreeff M. 2-Chlorodeoxyadenosine and cytarabine combination therapy for idiopathic hypereosinophilic syndrome. Cancer. 2005 Aug 1;104(3):541-6. [https://doi.org/10.1002/cncr.21186 link to original article] '''dosing details in manuscript have been reviewed by our editors''' [https://pubmed.ncbi.nlm.nih.gov/15977212/ PubMed] |
− | |||
==Hydroxyurea monotherapy {{#subobject:e819ce|Regimen=1}}== | ==Hydroxyurea monotherapy {{#subobject:e819ce|Regimen=1}}== | ||
− | + | <div class="toccolours" style="background-color:#eeeeee"> | |
− | |||
===Regimen {{#subobject:4e5567|Variant=1}}=== | ===Regimen {{#subobject:4e5567|Variant=1}}=== | ||
− | {| class="wikitable" style="width: | + | {| class="wikitable sortable" style="width: 60%; text-align:center;" |
− | !style="width: | + | !style="width: 33%"|Study |
− | !style="width: | + | !style="width: 33%"|Dates of enrollment |
+ | !style="width: 33%"|[[Levels_of_Evidence#Evidence|Evidence]] | ||
|- | |- | ||
− | |[ | + | |[https://doi.org/10.7326/0003-4819-89-2-167 Parrillo et al. 1978] |
− | |style="background-color:#ffffbe"|Non-randomized, | + | |Not reported in abstract |
+ | |style="background-color:#ffffbe"|Non-randomized, fewer than 20 pts | ||
|- | |- | ||
|} | |} | ||
− | '' | + | ''Note: treatment details are not available in the abstract.'' |
+ | <div class="toccolours" style="background-color:#b3e2cd"> | ||
====Chemotherapy==== | ====Chemotherapy==== | ||
*[[Hydroxyurea (Hydrea)]] | *[[Hydroxyurea (Hydrea)]] | ||
− | + | </div></div> | |
===References=== | ===References=== | ||
− | # Parrillo JE, Fauci AS, Wolff SM. Therapy of the hypereosinophilic syndrome. Ann Intern Med. 1978 Aug;89(2):167-72. [ | + | # Parrillo JE, Fauci AS, Wolff SM. Therapy of the hypereosinophilic syndrome. Ann Intern Med. 1978 Aug;89(2):167-72. [https://doi.org/10.7326/0003-4819-89-2-167 link to original article] [https://pubmed.ncbi.nlm.nih.gov/677578/ PubMed] |
− | |||
==Imatinib monotherapy {{#subobject:77c7b0|Regimen=1}}== | ==Imatinib monotherapy {{#subobject:77c7b0|Regimen=1}}== | ||
− | + | <div class="toccolours" style="background-color:#eeeeee"> | |
− | |||
===Regimen {{#subobject:0be8cc|Variant=1}}=== | ===Regimen {{#subobject:0be8cc|Variant=1}}=== | ||
{| class="wikitable" style="color:white; background-color:#404040" | {| class="wikitable" style="color:white; background-color:#404040" | ||
Line 111: | Line 110: | ||
|- | |- | ||
|} | |} | ||
− | {| class="wikitable" style="width: | + | {| class="wikitable sortable" style="width: 60%; text-align:center;" |
− | !style="width: | + | !style="width: 33%"|Study |
− | !style="width: | + | !style="width: 33%"|Dates of enrollment |
+ | !style="width: 33%"|[[Levels_of_Evidence#Evidence|Evidence]] | ||
|- | |- | ||
|[https://doi.org/10.1016/S0140-6736(02)08505-7 Gleich et al. 2002] | |[https://doi.org/10.1016/S0140-6736(02)08505-7 Gleich et al. 2002] | ||
− | |style="background-color:#ffffbe"| | + | |Not reported |
+ | |style="background-color:#ffffbe"|Case series | ||
|- | |- | ||
− | |[https://doi.org/10.1056/NEJMoa020150 Apperley et al. 2002] | + | |[https://doi.org/10.1056/NEJMoa020150 Apperley et al. 2002 (STIB2225)] |
− | |style="background-color:#ffffbe"|Phase 2, | + | |Not reported |
+ | |style="background-color:#ffffbe"|Phase 2, fewer than 20 pts | ||
|- | |- | ||
− | |[ | + | |[https://doi.org/10.1182/blood-2002-10-3103 Pardanani et al. 2002] |
− | |style="background-color:#ffffbe"|Phase 2, | + | |Not reported |
+ | |style="background-color:#ffffbe"|Phase 2, fewer than 20 pts | ||
|- | |- | ||
− | |[ | + | |[https://doi.org/10.1182/blood-2003-01-0081 Cortes et al. 2003] |
− | |style="background-color:#ffffbe"|Phase 2, | + | |2001-2002 |
+ | |style="background-color:#ffffbe"|Phase 2, fewer than 20 pts | ||
|- | |- | ||
|[https://doi.org/10.1056/NEJMoa025217 Cools et al. 2003] | |[https://doi.org/10.1056/NEJMoa025217 Cools et al. 2003] | ||
− | |style="background-color:#ffffbe"|Non-randomized, | + | |2001-2002 |
+ | |style="background-color:#ffffbe"|Non-randomized, fewer than 20 pts | ||
|- | |- | ||
− | |[ | + | |[https://doi.org/10.3324/haematol.11420 Baccarani et al. 2007 (HES0203)] |
− | |style="background-color:# | + | |2001-2007 |
+ | |style="background-color:#91cf61"|Phase 2 | ||
|- | |- | ||
− | |[ | + | |[https://doi.org/10.1182/blood-2003-08-2798 Klion et al. 2003] |
− | |style="background-color:# | + | |2003 |
+ | |style="background-color:#ffffbe"|Non-randomized, fewer than 20 pts | ||
|- | |- | ||
− | |[https:// | + | |[https://doi.org/10.1111/j.1365-2141.2008.07033.x Helbig et al. 2008] |
− | |style="background-color:#ffffbe"|Non-randomized, | + | |Not reported |
+ | |style="background-color:#ffffbe"|Non-randomized, fewer than 20 pts | ||
|- | |- | ||
− | |[https:// | + | |[https://doi.org/10.1111/j.1365-2141.2008.07294.x Metzgeroth et al. 2008] |
+ | |Not reported | ||
|style="background-color:#91cf61"|Phase 2 | |style="background-color:#91cf61"|Phase 2 | ||
|- | |- | ||
|} | |} | ||
− | ''Dosing is as described in the Metzgeroth et al. 2008 paper.'' | + | ''Note: Dosing is as described in the Metzgeroth et al. 2008 paper.'' |
+ | <div class="toccolours" style="background-color:#b3e2cd"> | ||
====Targeted therapy==== | ====Targeted therapy==== | ||
*[[Imatinib (Gleevec)]] by the following mutation-specific criteria: | *[[Imatinib (Gleevec)]] by the following mutation-specific criteria: | ||
− | **FIP1L1-PDGFRA positive | + | **FIP1L1-PDGFRA fusion positive: 100 mg PO once per day |
**PDGFRB fusion positive or without known molecular aberration: 400 mg PO once per day | **PDGFRB fusion positive or without known molecular aberration: 400 mg PO once per day | ||
− | |||
'''28-day cycle for 13 or more cycles''' | '''28-day cycle for 13 or more cycles''' | ||
+ | </div> | ||
+ | <div class="toccolours" style="background-color:#cbd5e7"> | ||
====Subsequent treatment==== | ====Subsequent treatment==== | ||
− | *Helbig et al. 2008: Patients proceeded to | + | *Helbig et al. 2008: Patients proceeded to [[#Imatinib_monotherapy|imatinib]] maintenance after 3 to 6 months of therapy which consisted of weekly dosing; dose not specified in the manuscript |
+ | </div></div> | ||
===References=== | ===References=== | ||
− | # Gleich GJ, Leiferman KM, Pardanani A, Tefferi A, Butterfield JH. Treatment of hypereosinophilic syndrome with imatinib mesilate. Lancet. 2002 May 4;359(9317):1577-8. [https://doi.org/10.1016/S0140-6736(02)08505-7 link to original article] [https://pubmed.ncbi.nlm.nih.gov/12047970 PubMed] | + | # Gleich GJ, Leiferman KM, Pardanani A, Tefferi A, Butterfield JH. Treatment of hypereosinophilic syndrome with imatinib mesilate. Lancet. 2002 May 4;359(9317):1577-8. [https://doi.org/10.1016/S0140-6736(02)08505-7 link to original article] [https://pubmed.ncbi.nlm.nih.gov/12047970/ PubMed] |
− | # Apperley JF, Gardembas M, Melo JV, Russell-Jones R, Bain BJ, Baxter EJ, Chase A, Chessells JM, Colombat M, Dearden CE, Dimitrijevic S, Mahon FX, Marin D, Nikolova Z, Olavarria E, Silberman S, Schultheis B, Cross NC, Goldman JM. Response to imatinib mesylate in patients with chronic myeloproliferative diseases with rearrangements of the platelet-derived growth factor receptor beta. N Engl J Med. 2002 Aug 15;347(7):481-7. [https://doi.org/10.1056/NEJMoa020150 link to original article] ''' | + | # '''STIB2225:''' Apperley JF, Gardembas M, Melo JV, Russell-Jones R, Bain BJ, Baxter EJ, Chase A, Chessells JM, Colombat M, Dearden CE, Dimitrijevic S, Mahon FX, Marin D, Nikolova Z, Olavarria E, Silberman S, Schultheis B, Cross NC, Goldman JM. Response to imatinib mesylate in patients with chronic myeloproliferative diseases with rearrangements of the platelet-derived growth factor receptor beta. N Engl J Med. 2002 Aug 15;347(7):481-7. [https://doi.org/10.1056/NEJMoa020150 link to original article] '''dosing details in abstract have been reviewed by our editors''' [https://pubmed.ncbi.nlm.nih.gov/12181402/ PubMed] |
− | ## '''Pooled update:''' Cheah CY, Burbury K, Apperley JF, Huguet F, Pitini V, Gardembas M, Ross DM, Forrest D, Genet P, Rousselot P, Patton N, Smith G, Dunbar CE, Ito S, Aguiar RC, Odenike O, Gimelfarb A, Cross NC, Seymour JF. Patients with myeloid malignancies bearing PDGFRB fusion genes achieve durable long-term remissions with imatinib. Blood. 2014 Jun 5;123(23):3574-7. Epub 2014 Mar 31. [ | + | ## '''Pooled update:''' Cheah CY, Burbury K, Apperley JF, Huguet F, Pitini V, Gardembas M, Ross DM, Forrest D, Genet P, Rousselot P, Patton N, Smith G, Dunbar CE, Ito S, Aguiar RC, Odenike O, Gimelfarb A, Cross NC, Seymour JF. Patients with myeloid malignancies bearing PDGFRB fusion genes achieve durable long-term remissions with imatinib. Blood. 2014 Jun 5;123(23):3574-7. Epub 2014 Mar 31. [https://doi.org/10.1182/blood-2014-02-555607 link to original article] [https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4047496/ link to PMC article] [https://pubmed.ncbi.nlm.nih.gov/24687085/ PubMed] |
− | # Pardanani A, Reeder T, Porrata LF, Li CY, Tazelaar HD, Baxter EJ, Witzig TE, Cross NC, Tefferi A. Imatinib therapy for hypereosinophilic syndrome and other eosinophilic disorders. Blood. 2003 May 1;101(9):3391-7. Epub 2002 Dec 27. [ | + | # Pardanani A, Reeder T, Porrata LF, Li CY, Tazelaar HD, Baxter EJ, Witzig TE, Cross NC, Tefferi A. Imatinib therapy for hypereosinophilic syndrome and other eosinophilic disorders. Blood. 2003 May 1;101(9):3391-7. Epub 2002 Dec 27. [https://doi.org/10.1182/blood-2002-10-3103 link to original article] '''dosing details in abstract have been reviewed by our editors''' [https://pubmed.ncbi.nlm.nih.gov/12506022/ PubMed] |
− | # Cortes J, Ault P, Koller C, Thomas D, Ferrajoli A, Wierda W, Rios MB, Letvak L, Kaled ES, Kantarjian H. Efficacy of imatinib mesylate in the treatment of idiopathic hypereosinophilic syndrome. Blood. 2003 Jun 15;101(12):4714-6. Epub 2003 Feb 20. [ | + | # Cortes J, Ault P, Koller C, Thomas D, Ferrajoli A, Wierda W, Rios MB, Letvak L, Kaled ES, Kantarjian H. Efficacy of imatinib mesylate in the treatment of idiopathic hypereosinophilic syndrome. Blood. 2003 Jun 15;101(12):4714-6. Epub 2003 Feb 20. [https://doi.org/10.1182/blood-2003-01-0081 link to original article] '''dosing details in manuscript have been reviewed by our editors''' [https://pubmed.ncbi.nlm.nih.gov/12595304/ PubMed] |
− | # Cools J, DeAngelo DJ, Gotlib J, Stover EH, Legare RD, Cortes J, Kutok J, Clark J, Galinsky I, Griffin JD, Cross NC, Tefferi A, Malone J, Alam R, Schrier SL, Schmid J, Rose M, Vandenberghe P, Verhoef G, Boogaerts M, Wlodarska I, Kantarjian H, Marynen P, Coutre SE, Stone R, Gilliland DG. A tyrosine kinase created by fusion of the PDGFRA and FIP1L1 genes as a therapeutic target of imatinib in idiopathic hypereosinophilic syndrome. N Engl J Med. 2003 Mar 27;348(13):1201-14. [https://doi.org/10.1056/NEJMoa025217 link to original article] ''' | + | # Cools J, DeAngelo DJ, Gotlib J, Stover EH, Legare RD, Cortes J, Kutok J, Clark J, Galinsky I, Griffin JD, Cross NC, Tefferi A, Malone J, Alam R, Schrier SL, Schmid J, Rose M, Vandenberghe P, Verhoef G, Boogaerts M, Wlodarska I, Kantarjian H, Marynen P, Coutre SE, Stone R, Gilliland DG. A tyrosine kinase created by fusion of the PDGFRA and FIP1L1 genes as a therapeutic target of imatinib in idiopathic hypereosinophilic syndrome. N Engl J Med. 2003 Mar 27;348(13):1201-14. [https://doi.org/10.1056/NEJMoa025217 link to original article] '''dosing details in abstract have been reviewed by our editors''' [https://pubmed.ncbi.nlm.nih.gov/12660384/ PubMed] |
− | # Klion AD, Robyn J, Akin C, Noel P, Brown M, Law M, Metcalfe DD, Dunbar C, Nutman TB. Molecular remission and reversal of myelofibrosis in response to imatinib mesylate treatment in patients with the myeloproliferative variant of hypereosinophilic syndrome. Blood. 2004 Jan 15;103(2):473-8. Epub 2003 Sep 22. [ | + | # Klion AD, Robyn J, Akin C, Noel P, Brown M, Law M, Metcalfe DD, Dunbar C, Nutman TB. Molecular remission and reversal of myelofibrosis in response to imatinib mesylate treatment in patients with the myeloproliferative variant of hypereosinophilic syndrome. Blood. 2004 Jan 15;103(2):473-8. Epub 2003 Sep 22. [https://doi.org/10.1182/blood-2003-08-2798 link to original article] '''dosing details in abstract have been reviewed by our editors''' [https://pubmed.ncbi.nlm.nih.gov/14504092/ PubMed] |
− | # Baccarani M, Cilloni D, Rondoni M, Ottaviani E, Messa F, Merante S, Tiribelli M, Buccisano F, Testoni N, Gottardi E, de Vivo A, Giugliano E, Iacobucci I, Paolini S, Soverini S, Rosti G, Rancati F, Astolfi C, Pane F, Saglio G, Martinelli G. The efficacy of imatinib mesylate in patients with FIP1L1-PDGFRalpha-positive hypereosinophilic syndrome: results of a multicenter prospective study. Haematologica. 2007 Sep;92(9):1173-9. Epub 2007 Aug 1. [ | + | #'''HES0203:''' Baccarani M, Cilloni D, Rondoni M, Ottaviani E, Messa F, Merante S, Tiribelli M, Buccisano F, Testoni N, Gottardi E, de Vivo A, Giugliano E, Iacobucci I, Paolini S, Soverini S, Rosti G, Rancati F, Astolfi C, Pane F, Saglio G, Martinelli G. The efficacy of imatinib mesylate in patients with FIP1L1-PDGFRalpha-positive hypereosinophilic syndrome: results of a multicenter prospective study. Haematologica. 2007 Sep;92(9):1173-9. Epub 2007 Aug 1. [https://doi.org/10.3324/haematol.11420 link to original article] '''dosing details in abstract have been reviewed by our editors''' [https://pubmed.ncbi.nlm.nih.gov/17666373/ PubMed] [https://clinicaltrials.gov/study/NCT00276926 NCT00276926] |
− | # Helbig G, Stella-Hołowiecka B, Majewski M, Całbecka M, Gajkowska J, Klimkiewicz R, Moskwa A, Grzegorczyk J, Lewandowska M, Hołowiecki J. A single weekly dose of imatinib is sufficient to induce and maintain remission of chronic eosinophilic leukaemia in FIP1L1-PDGFRA-expressing patients. Br J Haematol. 2008 Apr;141(2):200-4. Epub 2008 Feb 26. [https:// | + | # Helbig G, Stella-Hołowiecka B, Majewski M, Całbecka M, Gajkowska J, Klimkiewicz R, Moskwa A, Grzegorczyk J, Lewandowska M, Hołowiecki J. A single weekly dose of imatinib is sufficient to induce and maintain remission of chronic eosinophilic leukaemia in FIP1L1-PDGFRA-expressing patients. Br J Haematol. 2008 Apr;141(2):200-4. Epub 2008 Feb 26. [https://doi.org/10.1111/j.1365-2141.2008.07033.x link to original article] '''dosing details in abstract have been reviewed by our editors''' [https://pubmed.ncbi.nlm.nih.gov/18307562/ PubMed] |
− | # Metzgeroth G, Walz C, Erben P, Popp H, Schmitt-Graeff A, Haferlach C, Fabarius A, Schnittger S, Grimwade D, Cross NC, Hehlmann R, Hochhaus A, Reiter A. Safety and efficacy of imatinib in chronic eosinophilic leukaemia and hypereosinophilic syndrome: a phase-II study. Br J Haematol. 2008 Dec;143(5):707-15. Epub 2008 Oct 17. [https:// | + | # Metzgeroth G, Walz C, Erben P, Popp H, Schmitt-Graeff A, Haferlach C, Fabarius A, Schnittger S, Grimwade D, Cross NC, Hehlmann R, Hochhaus A, Reiter A. Safety and efficacy of imatinib in chronic eosinophilic leukaemia and hypereosinophilic syndrome: a phase-II study. Br J Haematol. 2008 Dec;143(5):707-15. Epub 2008 Oct 17. [https://doi.org/10.1111/j.1365-2141.2008.07294.x link to original article] '''dosing details in manuscript have been reviewed by our editors''' [https://pubmed.ncbi.nlm.nih.gov/18950453/ PubMed] |
==Mepolizumab monotherapy {{#subobject:39b7a4|Regimen=1}}== | ==Mepolizumab monotherapy {{#subobject:39b7a4|Regimen=1}}== | ||
− | + | <div class="toccolours" style="background-color:#eeeeee"> | |
===Regimen {{#subobject:2ef22d|Variant=1}}=== | ===Regimen {{#subobject:2ef22d|Variant=1}}=== | ||
{| class="wikitable sortable" style="width: 100%; text-align:center;" | {| class="wikitable sortable" style="width: 100%; text-align:center;" | ||
!style="width: 20%"|Study | !style="width: 20%"|Study | ||
− | !style="width: 20%"| | + | !style="width: 20%"|Dates of enrollment |
!style="width: 20%"|[[Levels_of_Evidence#Evidence|Evidence]] | !style="width: 20%"|[[Levels_of_Evidence#Evidence|Evidence]] | ||
!style="width: 20%"|Comparator | !style="width: 20%"|Comparator | ||
Line 179: | Line 191: | ||
|2004-2006 | |2004-2006 | ||
| style="background-color:#1a9851" |Phase 3 (E-esc) | | style="background-color:#1a9851" |Phase 3 (E-esc) | ||
− | |[[# | + | |[[#Placebo|Placebo]] |
| style="background-color:#1a9850" |Superior primary endpoint | | style="background-color:#1a9850" |Superior primary endpoint | ||
|- | |- | ||
|} | |} | ||
+ | <div class="toccolours" style="background-color:#b3e2cd"> | ||
====Immunosuppressive therapy==== | ====Immunosuppressive therapy==== | ||
*[[Mepolizumab (Nucala)]] 750 mg IV once on day 1 | *[[Mepolizumab (Nucala)]] 750 mg IV once on day 1 | ||
− | |||
'''21-day cycle for 8 cycles''' | '''21-day cycle for 8 cycles''' | ||
+ | </div></div> | ||
===References=== | ===References=== | ||
− | # '''GSK 100185:''' Rothenberg ME, Klion AD, Roufosse FE, Kahn JE, Weller PF, Simon HU, Schwartz LB, Rosenwasser LJ, Ring J, Griffin EF, Haig AE, Frewer PI, Parkin JM, Gleich GJ; Mepolizumab HES Study Group. Treatment of patients with the hypereosinophilic syndrome with mepolizumab. N Engl J Med. 2008 Mar 20;358(12):1215-28. Epub 2008 Mar 16. Erratum in: N Engl J Med. 2008 Jun 5;358(23): 2530. [https://doi.org/10.1056/NEJMoa070812 link to original article] ''' | + | # '''GSK 100185:''' Rothenberg ME, Klion AD, Roufosse FE, Kahn JE, Weller PF, Simon HU, Schwartz LB, Rosenwasser LJ, Ring J, Griffin EF, Haig AE, Frewer PI, Parkin JM, Gleich GJ; Mepolizumab HES Study Group. Treatment of patients with the hypereosinophilic syndrome with mepolizumab. N Engl J Med. 2008 Mar 20;358(12):1215-28. Epub 2008 Mar 16. Erratum in: N Engl J Med. 2008 Jun 5;358(23): 2530. [https://doi.org/10.1056/NEJMoa070812 link to original article] '''dosing details in manuscript have been reviewed by our editors''' [https://pubmed.ncbi.nlm.nih.gov/18344568/ PubMed] [https://clinicaltrials.gov/study/NCT00086658 NCT00086658] |
− | ## '''Update:''' Roufosse FE, Kahn JE, Gleich GJ, Schwartz LB, Singh AD, Rosenwasser LJ, Denburg JA, Ring J, Rothenberg ME, Sheikh J, Haig AE, Mallett SA, Templeton DN, Ortega HG, Klion AD. Long-term safety of mepolizumab for the treatment of hypereosinophilic syndromes. J Allergy Clin Immunol. 2013 Feb;131(2):461-7.e1-5. Epub 2012 Oct 4. [ | + | ## '''Update:''' Roufosse FE, Kahn JE, Gleich GJ, Schwartz LB, Singh AD, Rosenwasser LJ, Denburg JA, Ring J, Rothenberg ME, Sheikh J, Haig AE, Mallett SA, Templeton DN, Ortega HG, Klion AD. Long-term safety of mepolizumab for the treatment of hypereosinophilic syndromes. J Allergy Clin Immunol. 2013 Feb;131(2):461-7.e1-5. Epub 2012 Oct 4. [https://doi.org/10.1016/j.jaci.2012.07.055 link to original article] [https://www.ncbi.nlm.nih.gov/pmc/articles/pmc3558744/ link to PMC article] [https://pubmed.ncbi.nlm.nih.gov/23040887/ PubMed] |
− | |||
==Nilotinib monotherapy {{#subobject:007581|Regimen=1}}== | ==Nilotinib monotherapy {{#subobject:007581|Regimen=1}}== | ||
− | + | <div class="toccolours" style="background-color:#eeeeee"> | |
===Regimen {{#subobject:267a4e|Variant=1}}=== | ===Regimen {{#subobject:267a4e|Variant=1}}=== | ||
− | {| class="wikitable" style="width: | + | {| class="wikitable" style="width: 60%; text-align:center;" |
− | !style="width: | + | !style="width: 33%"|Study |
− | !style="width: | + | !style="width: 33%"|Dates of enrollment |
+ | !style="width: 33%"|[[Levels_of_Evidence#Evidence|Evidence]] | ||
|- | |- | ||
|[https://doi.org/10.1056/NEJMoa055104 Kantarjian et al. 2006 (A2101)] | |[https://doi.org/10.1056/NEJMoa055104 Kantarjian et al. 2006 (A2101)] | ||
− | |style="background-color:#ffffbe"|Phase 1/2, | + | |2004-2005 |
+ | |style="background-color:#ffffbe"|Phase 1/2, fewer than 20 pts of this subtype | ||
|- | |- | ||
|} | |} | ||
+ | <div class="toccolours" style="background-color:#b3e2cd"> | ||
====Targeted therapy==== | ====Targeted therapy==== | ||
− | *[[Nilotinib (Tasigna)]] 400 mg PO twice per day | + | *[[Nilotinib (Tasigna)]] 400 mg PO twice per day on days 1 to 28 |
− | + | '''28-day cycles''' | |
+ | </div></div> | ||
===References=== | ===References=== | ||
− | # '''A2101:''' Kantarjian H, Giles F, Wunderle L, Bhalla K, O'Brien S, Wassmann B, Tanaka C, Manley P, Rae P, Mietlowski W, Bochinski K, Hochhaus A, Griffin JD, Hoelzer D, Albitar M, Dugan M, Cortes J, Alland L, Ottmann OG. Nilotinib in imatinib-resistant CML and Philadelphia chromosome-positive ALL. N Engl J Med. 2006 Jun 15;354(24):2542-51. [https://doi.org/10.1056/NEJMoa055104 link to original article] [https://pubmed.ncbi.nlm.nih.gov/16775235 PubMed] NCT00109707 | + | # '''A2101:''' Kantarjian H, Giles F, Wunderle L, Bhalla K, O'Brien S, Wassmann B, Tanaka C, Manley P, Rae P, Mietlowski W, Bochinski K, Hochhaus A, Griffin JD, Hoelzer D, Albitar M, Dugan M, Cortes J, Alland L, Ottmann OG. Nilotinib in imatinib-resistant CML and Philadelphia chromosome-positive ALL. N Engl J Med. 2006 Jun 15;354(24):2542-51. [https://doi.org/10.1056/NEJMoa055104 link to original article] [https://pubmed.ncbi.nlm.nih.gov/16775235/ PubMed] [https://clinicaltrials.gov/study/NCT00109707 NCT00109707] |
− | ## '''Subgroup analysis:''' Hochhaus A, le Coutre PD, Kantarjian HM, Baccarani M, Erben P, Reiter A, McCulloch T, Fan X, Novick S, Giles FJ. Effect of the tyrosine kinase inhibitor nilotinib in patients with hypereosinophilic syndrome/chronic eosinophilic leukemia: analysis of the phase 2, open-label, single-arm A2101 study. J Cancer Res Clin Oncol. 2013 Dec;139(12):1985-93. Epub 2013 Sep 22. [ | + | ## '''Subgroup analysis:''' Hochhaus A, le Coutre PD, Kantarjian HM, Baccarani M, Erben P, Reiter A, McCulloch T, Fan X, Novick S, Giles FJ. Effect of the tyrosine kinase inhibitor nilotinib in patients with hypereosinophilic syndrome/chronic eosinophilic leukemia: analysis of the phase 2, open-label, single-arm A2101 study. J Cancer Res Clin Oncol. 2013 Dec;139(12):1985-93. Epub 2013 Sep 22. [https://doi.org/10.1007/s00432-013-1529-7 link to original article] [https://www.ncbi.nlm.nih.gov/pmc/articles/pmc5556980/ link to PMC article] [https://pubmed.ncbi.nlm.nih.gov/24057647/ PubMed] |
==Peginterferon alfa-2a monotherapy {{#subobject:010d91|Regimen=1}}== | ==Peginterferon alfa-2a monotherapy {{#subobject:010d91|Regimen=1}}== | ||
− | + | <div class="toccolours" style="background-color:#eeeeee"> | |
− | |||
===Regimen {{#subobject:72ed60|Variant=1}}=== | ===Regimen {{#subobject:72ed60|Variant=1}}=== | ||
{| class="wikitable" style="width: 40%; text-align:center;" | {| class="wikitable" style="width: 40%; text-align:center;" | ||
Line 217: | Line 232: | ||
!style="width: 25%"|[[Levels_of_Evidence#Evidence|Evidence]] | !style="width: 25%"|[[Levels_of_Evidence#Evidence|Evidence]] | ||
|- | |- | ||
− | |[ | + | |[https://doi.org/10.1016/j.leukres.2011.09.026 Butterfield et al. 2011] |
|style="background-color:#ffffbe"|Case series | |style="background-color:#ffffbe"|Case series | ||
|- | |- | ||
|} | |} | ||
− | + | ''Note: The authors describe using peginterferon alfa-2b initially but switched to peginterferon alfa-2a when the first was "no longer available. Dosing details are not available in the abstract." | |
− | ''The authors describe using peginterferon alfa-2b initially but switched to peginterferon alfa-2a when the first was "no longer available. Dosing details are not available in the abstract." | + | <div class="toccolours" style="background-color:#b3e2cd"> |
====Immunotherapy==== | ====Immunotherapy==== | ||
*[[Peginterferon alfa-2a (Pegasys)]] | *[[Peginterferon alfa-2a (Pegasys)]] | ||
− | + | </div></div> | |
===References=== | ===References=== | ||
− | # '''Case series:''' Butterfield JH, Weiler CR. Use of pegylated interferon in hypereosinophilic syndrome. Leuk Res. 2012 Feb;36(2):192-7. Epub 2011 Nov 26. [ | + | # '''Case series:''' Butterfield JH, Weiler CR. Use of pegylated interferon in hypereosinophilic syndrome. Leuk Res. 2012 Feb;36(2):192-7. Epub 2011 Nov 26. [https://doi.org/10.1016/j.leukres.2011.09.026 link to original article] [https://pubmed.ncbi.nlm.nih.gov/22118911/ PubMed] |
− | |||
==Ruxolitinib monotherapy {{#subobject:87790c|Regimen=1}}== | ==Ruxolitinib monotherapy {{#subobject:87790c|Regimen=1}}== | ||
− | + | <div class="toccolours" style="background-color:#eeeeee"> | |
− | |||
===Regimen {{#subobject:f86a11|Variant=1}}=== | ===Regimen {{#subobject:f86a11|Variant=1}}=== | ||
{| class="wikitable" style="width: 40%; text-align:center;" | {| class="wikitable" style="width: 40%; text-align:center;" | ||
Line 241: | Line 254: | ||
|- | |- | ||
|} | |} | ||
+ | <div class="toccolours" style="background-color:#b3e2cd"> | ||
====Targeted therapy==== | ====Targeted therapy==== | ||
*[[Ruxolitinib (Jakafi)]] 15 mg PO twice per day | *[[Ruxolitinib (Jakafi)]] 15 mg PO twice per day | ||
− | |||
'''Duration not specified''' | '''Duration not specified''' | ||
− | + | </div></div> | |
===References=== | ===References=== | ||
− | # '''Case report:''' Rumi E, Milosevic JD, Casetti I, Dambruoso I, Pietra D, Boveri E, Boni M, Bernasconi P, Passamonti F, Kralovics R, Cazzola M. Efficacy of ruxolitinib in chronic eosinophilic leukemia associated with a PCM1-JAK2 fusion gene. J Clin Oncol. 2013 Jun 10;31(17):e269-71. Epub 2013 Apr 29. [https://doi.org/10.1200/jco.2012.46.4370 link to original article] ''' | + | # '''Case report:''' Rumi E, Milosevic JD, Casetti I, Dambruoso I, Pietra D, Boveri E, Boni M, Bernasconi P, Passamonti F, Kralovics R, Cazzola M. Efficacy of ruxolitinib in chronic eosinophilic leukemia associated with a PCM1-JAK2 fusion gene. J Clin Oncol. 2013 Jun 10;31(17):e269-71. Epub 2013 Apr 29. [https://doi.org/10.1200/jco.2012.46.4370 link to original article] '''dosing details in abstract have been reviewed by our editors''' [https://pubmed.ncbi.nlm.nih.gov/23630205/ PubMed] |
− | |||
[[Category:Hypereosinophilic syndrome regimens]] | [[Category:Hypereosinophilic syndrome regimens]] | ||
[[Category:Disease-specific pages]] | [[Category:Disease-specific pages]] | ||
[[Category:Myeloproliferative neoplasms]] | [[Category:Myeloproliferative neoplasms]] | ||
− |
Latest revision as of 23:36, 15 July 2024
Section editor | |
---|---|
Sanjay R. Mohan, MD, MSCI Vanderbilt University Nashville, TN, USA |
9 regimens on this page
9 variants on this page
|
For placebo or observational studies in this condition, please visit 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.
NCCN
All lines of therapy
Alemtuzumab monotherapy
Regimen
Study | Evidence |
---|---|
Wagner et al. 2009 | Case report |
Note: treatment is to be given bi-weekly, exact days not specified; below is an example. Duration also not specified.
References
- Case report: Wagner LA, Speckart S, Cutter B, Gleich GJ. Treatment of FIP1L1/PDGFRA-negative hypereosinophilic syndrome with alemtuzumab, an anti-CD52 antibody. J Allergy Clin Immunol. 2009 Jun;123(6):1407-8. Epub 2009 Apr 1. link to original article dosing details in abstract have been reviewed by our editors PubMed
- Retrospective: Strati P, Cortes J, Faderl S, Kantarjian H, Verstovsek S. Long-term follow-up of patients with hypereosinophilic syndrome treated with Alemtuzumab, an anti-CD52 antibody. Clin Lymphoma Myeloma Leuk. 2013 Jun;13(3):287-91. Epub 2012 Nov 1. link to original article link to PMC article PubMed
Benralizumab monotherapy
Regimen
Study | Dates of enrollment | Evidence | Comparator | Comparative Efficacy |
---|---|---|---|---|
Kuang et al. 2019 (NIAID 14-I-0081) | 2014-2017 | Randomized Phase 2, fewer than 20 pts (E-esc) | Placebo | Seems to have superior primary endpoint |
Immunosuppressive therapy
- Benralizumab (Fasenra) 30 mg SC once on day 1
28-day cycle for 6 or more cycles
References
- NIAID 14-I-0081: Kuang FL, Legrand F, Makiya M, Ware J, Wetzler L, Brown T, Magee T, Piligian B, Yoon P, Ellis JH, Sun X, Panch SR, Powers A, Alao H, Kumar S, Quezado M, Yan L, Lee N, Kolbeck R, Newbold P, Goldman M, Fay MP, Khoury P, Maric I, Klion AD. Benralizumab for PDGFRA-negative hypereosinophilic syndrome. N Engl J Med. 2019 Apr 4;380(14):1336-1346. link to original article dosing details in manuscript have been reviewed by our editors link to PMC article PubMed NCT00001406
Cladribine & Cytarabine
Regimen
Study | Dates of enrollment | Evidence |
---|---|---|
Jabbour et al. 2005 | 1999-04 to 2001-04 | Phase 2, fewer than 20 pts |
Note: the timing of drug administration is in hours in the manuscript, starting at t = 0 hours.
Chemotherapy
- Cladribine (Leustatin) 12 mg/m2/day IV continuous infusion over 120 hours, started on day 2 (total dose: 60 mg/m2)
- Cytarabine (Ara-C) 1000 mg/m2 IV over 2 hours once per day on days 1, 3, 4, 5, 6
21-day cycle, repeated once if PR/CR; see text for definition of PR
References
- Jabbour E, Verstovsek S, Giles F, Gandhi V, Cortes J, O'Brien S, Plunkett W, Garcia-Manero G, Jackson CE, Kantarjian H, Andreeff M. 2-Chlorodeoxyadenosine and cytarabine combination therapy for idiopathic hypereosinophilic syndrome. Cancer. 2005 Aug 1;104(3):541-6. link to original article dosing details in manuscript have been reviewed by our editors PubMed
Hydroxyurea monotherapy
Regimen
Study | Dates of enrollment | Evidence |
---|---|---|
Parrillo et al. 1978 | Not reported in abstract | Non-randomized, fewer than 20 pts |
Note: treatment details are not available in the abstract.
Chemotherapy
References
- Parrillo JE, Fauci AS, Wolff SM. Therapy of the hypereosinophilic syndrome. Ann Intern Med. 1978 Aug;89(2):167-72. link to original article PubMed
Imatinib monotherapy
Regimen
FDA-recommended dose |
Study | Dates of enrollment | Evidence |
---|---|---|
Gleich et al. 2002 | Not reported | Case series |
Apperley et al. 2002 (STIB2225) | Not reported | Phase 2, fewer than 20 pts |
Pardanani et al. 2002 | Not reported | Phase 2, fewer than 20 pts |
Cortes et al. 2003 | 2001-2002 | Phase 2, fewer than 20 pts |
Cools et al. 2003 | 2001-2002 | Non-randomized, fewer than 20 pts |
Baccarani et al. 2007 (HES0203) | 2001-2007 | Phase 2 |
Klion et al. 2003 | 2003 | Non-randomized, fewer than 20 pts |
Helbig et al. 2008 | Not reported | Non-randomized, fewer than 20 pts |
Metzgeroth et al. 2008 | Not reported | Phase 2 |
Note: Dosing is as described in the Metzgeroth et al. 2008 paper.
Targeted therapy
- Imatinib (Gleevec) by the following mutation-specific criteria:
- FIP1L1-PDGFRA fusion positive: 100 mg PO once per day
- PDGFRB fusion positive or without known molecular aberration: 400 mg PO once per day
28-day cycle for 13 or more cycles
Subsequent treatment
- Helbig et al. 2008: Patients proceeded to imatinib maintenance after 3 to 6 months of therapy which consisted of weekly dosing; dose not specified in the manuscript
References
- Gleich GJ, Leiferman KM, Pardanani A, Tefferi A, Butterfield JH. Treatment of hypereosinophilic syndrome with imatinib mesilate. Lancet. 2002 May 4;359(9317):1577-8. link to original article PubMed
- STIB2225: Apperley JF, Gardembas M, Melo JV, Russell-Jones R, Bain BJ, Baxter EJ, Chase A, Chessells JM, Colombat M, Dearden CE, Dimitrijevic S, Mahon FX, Marin D, Nikolova Z, Olavarria E, Silberman S, Schultheis B, Cross NC, Goldman JM. Response to imatinib mesylate in patients with chronic myeloproliferative diseases with rearrangements of the platelet-derived growth factor receptor beta. N Engl J Med. 2002 Aug 15;347(7):481-7. link to original article dosing details in abstract have been reviewed by our editors PubMed
- Pooled update: Cheah CY, Burbury K, Apperley JF, Huguet F, Pitini V, Gardembas M, Ross DM, Forrest D, Genet P, Rousselot P, Patton N, Smith G, Dunbar CE, Ito S, Aguiar RC, Odenike O, Gimelfarb A, Cross NC, Seymour JF. Patients with myeloid malignancies bearing PDGFRB fusion genes achieve durable long-term remissions with imatinib. Blood. 2014 Jun 5;123(23):3574-7. Epub 2014 Mar 31. link to original article link to PMC article PubMed
- Pardanani A, Reeder T, Porrata LF, Li CY, Tazelaar HD, Baxter EJ, Witzig TE, Cross NC, Tefferi A. Imatinib therapy for hypereosinophilic syndrome and other eosinophilic disorders. Blood. 2003 May 1;101(9):3391-7. Epub 2002 Dec 27. link to original article dosing details in abstract have been reviewed by our editors PubMed
- Cortes J, Ault P, Koller C, Thomas D, Ferrajoli A, Wierda W, Rios MB, Letvak L, Kaled ES, Kantarjian H. Efficacy of imatinib mesylate in the treatment of idiopathic hypereosinophilic syndrome. Blood. 2003 Jun 15;101(12):4714-6. Epub 2003 Feb 20. link to original article dosing details in manuscript have been reviewed by our editors PubMed
- Cools J, DeAngelo DJ, Gotlib J, Stover EH, Legare RD, Cortes J, Kutok J, Clark J, Galinsky I, Griffin JD, Cross NC, Tefferi A, Malone J, Alam R, Schrier SL, Schmid J, Rose M, Vandenberghe P, Verhoef G, Boogaerts M, Wlodarska I, Kantarjian H, Marynen P, Coutre SE, Stone R, Gilliland DG. A tyrosine kinase created by fusion of the PDGFRA and FIP1L1 genes as a therapeutic target of imatinib in idiopathic hypereosinophilic syndrome. N Engl J Med. 2003 Mar 27;348(13):1201-14. link to original article dosing details in abstract have been reviewed by our editors PubMed
- Klion AD, Robyn J, Akin C, Noel P, Brown M, Law M, Metcalfe DD, Dunbar C, Nutman TB. Molecular remission and reversal of myelofibrosis in response to imatinib mesylate treatment in patients with the myeloproliferative variant of hypereosinophilic syndrome. Blood. 2004 Jan 15;103(2):473-8. Epub 2003 Sep 22. link to original article dosing details in abstract have been reviewed by our editors PubMed
- HES0203: Baccarani M, Cilloni D, Rondoni M, Ottaviani E, Messa F, Merante S, Tiribelli M, Buccisano F, Testoni N, Gottardi E, de Vivo A, Giugliano E, Iacobucci I, Paolini S, Soverini S, Rosti G, Rancati F, Astolfi C, Pane F, Saglio G, Martinelli G. The efficacy of imatinib mesylate in patients with FIP1L1-PDGFRalpha-positive hypereosinophilic syndrome: results of a multicenter prospective study. Haematologica. 2007 Sep;92(9):1173-9. Epub 2007 Aug 1. link to original article dosing details in abstract have been reviewed by our editors PubMed NCT00276926
- Helbig G, Stella-Hołowiecka B, Majewski M, Całbecka M, Gajkowska J, Klimkiewicz R, Moskwa A, Grzegorczyk J, Lewandowska M, Hołowiecki J. A single weekly dose of imatinib is sufficient to induce and maintain remission of chronic eosinophilic leukaemia in FIP1L1-PDGFRA-expressing patients. Br J Haematol. 2008 Apr;141(2):200-4. Epub 2008 Feb 26. link to original article dosing details in abstract have been reviewed by our editors PubMed
- Metzgeroth G, Walz C, Erben P, Popp H, Schmitt-Graeff A, Haferlach C, Fabarius A, Schnittger S, Grimwade D, Cross NC, Hehlmann R, Hochhaus A, Reiter A. Safety and efficacy of imatinib in chronic eosinophilic leukaemia and hypereosinophilic syndrome: a phase-II study. Br J Haematol. 2008 Dec;143(5):707-15. Epub 2008 Oct 17. link to original article dosing details in manuscript have been reviewed by our editors PubMed
Mepolizumab monotherapy
Regimen
Study | Dates of enrollment | Evidence | Comparator | Comparative Efficacy |
---|---|---|---|---|
Rothenberg et al. 2008 (GSK 100185) | 2004-2006 | Phase 3 (E-esc) | Placebo | Superior primary endpoint |
References
- GSK 100185: Rothenberg ME, Klion AD, Roufosse FE, Kahn JE, Weller PF, Simon HU, Schwartz LB, Rosenwasser LJ, Ring J, Griffin EF, Haig AE, Frewer PI, Parkin JM, Gleich GJ; Mepolizumab HES Study Group. Treatment of patients with the hypereosinophilic syndrome with mepolizumab. N Engl J Med. 2008 Mar 20;358(12):1215-28. Epub 2008 Mar 16. Erratum in: N Engl J Med. 2008 Jun 5;358(23): 2530. link to original article dosing details in manuscript have been reviewed by our editors PubMed NCT00086658
- Update: Roufosse FE, Kahn JE, Gleich GJ, Schwartz LB, Singh AD, Rosenwasser LJ, Denburg JA, Ring J, Rothenberg ME, Sheikh J, Haig AE, Mallett SA, Templeton DN, Ortega HG, Klion AD. Long-term safety of mepolizumab for the treatment of hypereosinophilic syndromes. J Allergy Clin Immunol. 2013 Feb;131(2):461-7.e1-5. Epub 2012 Oct 4. link to original article link to PMC article PubMed
Nilotinib monotherapy
Regimen
Study | Dates of enrollment | Evidence |
---|---|---|
Kantarjian et al. 2006 (A2101) | 2004-2005 | Phase 1/2, fewer than 20 pts of this subtype |
References
- A2101: Kantarjian H, Giles F, Wunderle L, Bhalla K, O'Brien S, Wassmann B, Tanaka C, Manley P, Rae P, Mietlowski W, Bochinski K, Hochhaus A, Griffin JD, Hoelzer D, Albitar M, Dugan M, Cortes J, Alland L, Ottmann OG. Nilotinib in imatinib-resistant CML and Philadelphia chromosome-positive ALL. N Engl J Med. 2006 Jun 15;354(24):2542-51. link to original article PubMed NCT00109707
- Subgroup analysis: Hochhaus A, le Coutre PD, Kantarjian HM, Baccarani M, Erben P, Reiter A, McCulloch T, Fan X, Novick S, Giles FJ. Effect of the tyrosine kinase inhibitor nilotinib in patients with hypereosinophilic syndrome/chronic eosinophilic leukemia: analysis of the phase 2, open-label, single-arm A2101 study. J Cancer Res Clin Oncol. 2013 Dec;139(12):1985-93. Epub 2013 Sep 22. link to original article link to PMC article PubMed
Peginterferon alfa-2a monotherapy
Regimen
Study | Evidence |
---|---|
Butterfield et al. 2011 | Case series |
Note: The authors describe using peginterferon alfa-2b initially but switched to peginterferon alfa-2a when the first was "no longer available. Dosing details are not available in the abstract."
Immunotherapy
References
- Case series: Butterfield JH, Weiler CR. Use of pegylated interferon in hypereosinophilic syndrome. Leuk Res. 2012 Feb;36(2):192-7. Epub 2011 Nov 26. link to original article PubMed
Ruxolitinib monotherapy
Regimen
Study | Evidence |
---|---|
Rumi et al. 2013 | Case report |
References
- Case report: Rumi E, Milosevic JD, Casetti I, Dambruoso I, Pietra D, Boveri E, Boni M, Bernasconi P, Passamonti F, Kralovics R, Cazzola M. Efficacy of ruxolitinib in chronic eosinophilic leukemia associated with a PCM1-JAK2 fusion gene. J Clin Oncol. 2013 Jun 10;31(17):e269-71. Epub 2013 Apr 29. link to original article dosing details in abstract have been reviewed by our editors PubMed