Difference between revisions of "Systemic mastocytosis"
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− | ===Regimen #1 | + | ===Regimen #1 {{#subobject:1e79f9|Variant=1}}=== |
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+ | {| border="1" style="text-align:center;" !align="left" | ||
+ | |'''Study''' | ||
+ | |[[Levels_of_Evidence#Evidence|'''Evidence''']] | ||
+ | |- | ||
+ | |[http://www.sciencedirect.com/science/article/pii/S0145212608005675 Vega-Ruiz et al. 2009] | ||
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border-style:solid;">Phase II</span> | border-style:solid;">Phase II</span> | ||
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+ | |} | ||
− | *[[Imatinib (Gleevec)]] 400 mg PO once per day | + | *[[Imatinib (Gleevec)]] 400 mg PO once per day, increased by 200 mg every month to a maximum of 800 mg PO once per day |
Supportive medications: | Supportive medications: | ||
− | *[ | + | *[[:Category:Hematopoietic_growth_factors|Hematopoietic growth factors]] at investigator discretion |
+ | |||
+ | '''Continued until disease progression or unacceptable toxicity.''' | ||
− | + | ===Regimen #2 {{#subobject:abb8c6|Variant=1}}=== | |
− | === | + | {| border="1" style="text-align:center;" !align="left" |
− | <span | + | |'''Study''' |
+ | |[[Levels_of_Evidence#Evidence|'''Evidence''']] | ||
+ | |- | ||
+ | |[http://onlinelibrary.wiley.com/doi/10.1002/cncr.21996/full Droogendijk et al. 2006] | ||
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border-style:solid;">Phase II, <20 patients reported</span> | border-style:solid;">Phase II, <20 patients reported</span> | ||
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+ | |} | ||
*[[Imatinib (Gleevec)]] 400 mg PO once per day | *[[Imatinib (Gleevec)]] 400 mg PO once per day | ||
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===References=== | ===References=== | ||
+ | # Pardanani A, Elliott M, Reeder T, Li CY, Baxter EJ, Cross NC, Tefferi A. Imatinib for systemic mast-cell disease. Lancet. 2003 Aug 16;362(9383):535-6. [http://www.thelancet.com/journals/lancet/article/PIIS0140-6736(03)14115-3/fulltext link to original article] [http://www.ncbi.nlm.nih.gov/pubmed/12932387 PubMed] | ||
# Droogendijk HJ, Kluin-Nelemans HJ, van Doormaal JJ, Oranje AP, van de Loosdrecht AA, van Daele PL. Imatinib mesylate in the treatment of systemic mastocytosis: a phase II trial. Cancer. 2006 Jul 15;107(2):345-51. [http://onlinelibrary.wiley.com/doi/10.1002/cncr.21996/full link to original article] '''contains verified protocol''' [http://www.ncbi.nlm.nih.gov/pubmed/16779792 PubMed] | # Droogendijk HJ, Kluin-Nelemans HJ, van Doormaal JJ, Oranje AP, van de Loosdrecht AA, van Daele PL. Imatinib mesylate in the treatment of systemic mastocytosis: a phase II trial. Cancer. 2006 Jul 15;107(2):345-51. [http://onlinelibrary.wiley.com/doi/10.1002/cncr.21996/full link to original article] '''contains verified protocol''' [http://www.ncbi.nlm.nih.gov/pubmed/16779792 PubMed] | ||
# '''Retrospective:''' Pagano L, Valentini CG, Caira M, Rondoni M, Van Lint MT, Candoni A, Allione B, Cattaneo C, Marbello L, Caramatti C, Pogliani EM, Iannitto E, Giona F, Ferrara F, Invernizzi R, Fanci R, Lunghi M, Fianchi L, Sanpaolo G, Stefani PM, Pulsoni A, Martinelli G, Leone G, Musto P. Advanced mast cell disease: an Italian Hematological Multicenter experience. Int J Hematol. 2008 Dec;88(5):483-8. Epub 2008 Nov 26. [http://link.springer.com/article/10.1007%2Fs12185-008-0166-4 link to original article] [http://www.ncbi.nlm.nih.gov/pubmed/19034614 PubMed] | # '''Retrospective:''' Pagano L, Valentini CG, Caira M, Rondoni M, Van Lint MT, Candoni A, Allione B, Cattaneo C, Marbello L, Caramatti C, Pogliani EM, Iannitto E, Giona F, Ferrara F, Invernizzi R, Fanci R, Lunghi M, Fianchi L, Sanpaolo G, Stefani PM, Pulsoni A, Martinelli G, Leone G, Musto P. Advanced mast cell disease: an Italian Hematological Multicenter experience. Int J Hematol. 2008 Dec;88(5):483-8. Epub 2008 Nov 26. [http://link.springer.com/article/10.1007%2Fs12185-008-0166-4 link to original article] [http://www.ncbi.nlm.nih.gov/pubmed/19034614 PubMed] | ||
# Vega-Ruiz A, Cortes JE, Sever M, Manshouri T, Quintás-Cardama A, Luthra R, Kantarjian HM, Verstovsek S. Phase II study of imatinib mesylate as therapy for patients with systemic mastocytosis. Leuk Res. 2009 Nov;33(11):1481-4. Epub 2009 Feb 3. [http://www.sciencedirect.com/science/article/pii/S0145212608005675 link to original article] '''contains verified protocol''' [http://www.ncbi.nlm.nih.gov/pubmed/19193436 PubMed] | # Vega-Ruiz A, Cortes JE, Sever M, Manshouri T, Quintás-Cardama A, Luthra R, Kantarjian HM, Verstovsek S. Phase II study of imatinib mesylate as therapy for patients with systemic mastocytosis. Leuk Res. 2009 Nov;33(11):1481-4. Epub 2009 Feb 3. [http://www.sciencedirect.com/science/article/pii/S0145212608005675 link to original article] '''contains verified protocol''' [http://www.ncbi.nlm.nih.gov/pubmed/19193436 PubMed] | ||
# '''Retrospective:''' Lim KH, Pardanani A, Butterfield JH, Li CY, Tefferi A. Cytoreductive therapy in 108 adults with systemic mastocytosis: Outcome analysis and response prediction during treatment with interferon-alpha, hydroxyurea, imatinib mesylate or 2-chlorodeoxyadenosine. Am J Hematol. 2009 Dec;84(12):790-4. [http://onlinelibrary.wiley.com/doi/10.1002/ajh.21561/abstract link to original article] [http://www.ncbi.nlm.nih.gov/pubmed/19890907 PubMed] | # '''Retrospective:''' Lim KH, Pardanani A, Butterfield JH, Li CY, Tefferi A. Cytoreductive therapy in 108 adults with systemic mastocytosis: Outcome analysis and response prediction during treatment with interferon-alpha, hydroxyurea, imatinib mesylate or 2-chlorodeoxyadenosine. Am J Hematol. 2009 Dec;84(12):790-4. [http://onlinelibrary.wiley.com/doi/10.1002/ajh.21561/abstract link to original article] [http://www.ncbi.nlm.nih.gov/pubmed/19890907 PubMed] |
Revision as of 15:00, 6 September 2015
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7 regimens on this page
13 variants on this page
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Aggressive systemic mastocytosis (ASM)
Cladribine (Leustatin)
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Regimen #1, Kluin-Nelemans et al. 2003
Pilot, <20 patients reported
- Cladribine (Leustatin) 0.13 mk/kg IV over 2 hours once per day on days 1 to 5
Supportive medications:
- Clemastine (Tavist) 1 to 2 mg IV prior to Cladribine (Leustatin)
- Dexamethasone (Decadron) 5 mg IV prior to Cladribine (Leustatin)
- Fluconazole (Diflucan) (dose not specified)
- Co-trimaxazole (dose not specified)
- Valacyclovir (Valtrex) (dose not specified) "in some patients"
4 to 6-week cycles x 6 cycles
Regimen #2, Lim et al. 2009
Retrospective
This is a retrospective series.
- Cladribine (Leustatin) 5 mg/m2 or 0.13 to 0.17 mg/kg IV over 2 hours once per day on days 1 to 5
Median number of treatment cycles was 3 (range 1 to 9)
Regimen #3, Hermine et al. 2010
Non-randomized
This is the largest series but is published in abstract only.
- Cladribine (Leustatin) 0.15 mg/kg IV over 2 hours or SC once per day on days 1 to 5
4 to 12-week cycles x 1 to 6 cycles
References
- Kluin-Nelemans HC, Oldhoff JM, Van Doormaal JJ, Van 't Wout JW, Verhoef G, Gerrits WB, van Dobbenburgh OA, Pasmans SG, Fijnheer R. Cladribine therapy for systemic mastocytosis. Blood. 2003 Dec 15;102(13):4270-6. Epub 2003 Aug 21. link to original article contains verified protocol PubMed
- Retrospective: Lim KH, Pardanani A, Butterfield JH, Li CY, Tefferi A. Cytoreductive therapy in 108 adults with systemic mastocytosis: Outcome analysis and response prediction during treatment with interferon-alpha, hydroxyurea, imatinib mesylate or 2-chlorodeoxyadenosine. Am J Hematol. 2009 Dec;84(12):790-4. link to original article PubMed
- Abstract: Hermine, Olivier, Hirsh, Isabelle, Damaj, Gandhi, Granpeix, Catherine, Barete, Stephane, Suarez, Felipe, Chansderis, Olivia, Ghez, David, Delarue, Richard, Lanternier, Fanny, Deau, Benedicte, Coignard, Helene, Fraitag, Sylvie, Canioni, Danielle, Casassus, Philippe, Dubreuil, Patrice, Lortholary, Olivier. Long Term Efficacy and Safety of Cladribine In Adult Systemic mastocytosis: a French Multicenter Study of 44 Patients. ASH Annual Meeting Abstracts 2010 116: 1982 link to original abstract
Dasatinib (Sprycel)
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Regimen, Verstovsek et al. 2008
Phase II
Only 9 of 67 patients studied had ASM; 18 has ISM and 6 had SM-AHNMD.
- Dasatinib (Sprycel) 70 mg PO BID or 140 mg PO once per day
Supportive medications:
- Hematopoietic growth factors at investigator discretion
28-day cycles until disease progression or intolerable toxicity
References
- Verstovsek S, Tefferi A, Cortes J, O'Brien S, Garcia-Manero G, Pardanani A, Akin C, Faderl S, Manshouri T, Thomas D, Kantarjian H. Phase II study of dasatinib in Philadelphia chromosome-negative acute and chronic myeloid diseases, including systemic mastocytosis. Clin Cancer Res. 2008 Jun 15;14(12):3906-15. link to original article contains verified protocol PubMed
Imatinib (Gleevec)
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Regimen #1
Study | Evidence |
Vega-Ruiz et al. 2009 | Phase II |
- Imatinib (Gleevec) 400 mg PO once per day, increased by 200 mg every month to a maximum of 800 mg PO once per day
Supportive medications:
- Hematopoietic growth factors at investigator discretion
Continued until disease progression or unacceptable toxicity.
Regimen #2
Study | Evidence |
Droogendijk et al. 2006 | Phase II, <20 patients reported |
- Imatinib (Gleevec) 400 mg PO once per day
Supportive medications:
- Prednisone (Sterapred) 15 mg PO BID for first 2 weeks of treatment
Up to 6 month course
References
- Pardanani A, Elliott M, Reeder T, Li CY, Baxter EJ, Cross NC, Tefferi A. Imatinib for systemic mast-cell disease. Lancet. 2003 Aug 16;362(9383):535-6. link to original article PubMed
- Droogendijk HJ, Kluin-Nelemans HJ, van Doormaal JJ, Oranje AP, van de Loosdrecht AA, van Daele PL. Imatinib mesylate in the treatment of systemic mastocytosis: a phase II trial. Cancer. 2006 Jul 15;107(2):345-51. link to original article contains verified protocol PubMed
- Retrospective: Pagano L, Valentini CG, Caira M, Rondoni M, Van Lint MT, Candoni A, Allione B, Cattaneo C, Marbello L, Caramatti C, Pogliani EM, Iannitto E, Giona F, Ferrara F, Invernizzi R, Fanci R, Lunghi M, Fianchi L, Sanpaolo G, Stefani PM, Pulsoni A, Martinelli G, Leone G, Musto P. Advanced mast cell disease: an Italian Hematological Multicenter experience. Int J Hematol. 2008 Dec;88(5):483-8. Epub 2008 Nov 26. link to original article PubMed
- Vega-Ruiz A, Cortes JE, Sever M, Manshouri T, Quintás-Cardama A, Luthra R, Kantarjian HM, Verstovsek S. Phase II study of imatinib mesylate as therapy for patients with systemic mastocytosis. Leuk Res. 2009 Nov;33(11):1481-4. Epub 2009 Feb 3. link to original article contains verified protocol PubMed
- Retrospective: Lim KH, Pardanani A, Butterfield JH, Li CY, Tefferi A. Cytoreductive therapy in 108 adults with systemic mastocytosis: Outcome analysis and response prediction during treatment with interferon-alpha, hydroxyurea, imatinib mesylate or 2-chlorodeoxyadenosine. Am J Hematol. 2009 Dec;84(12):790-4. link to original article PubMed