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Prepublished online as a Blood First Edition Paper on August 21, 2003; DOI 10.1182/blood-2003-05-1699. This Article Full Text (PDF) All Versions of this Article: 2003-05-1699v1 102/13/4270    most recent Alert me when this article is cited Alert me if a correction is posted Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Download to citation manager Rights and Permissions Citing Articles Citing Articles via HighWire Citing Articles via CrossRef Citing Articles via Google Scholar Google Scholar Articles by Kluin-Nelemans, H. C Articles by Fijnheer, R. Search for Related Content PubMed PubMed Citation Articles by Kluin-Nelemans, H. C Articles by Fijnheer, R. Social Bookmarking                   What's this? Submitted May 28, 2003 Accepted July 10, 2003 Cladribine therapy for systemic mastocytosis Hanneke C Kluin-Nelemans*, J Marja Oldhoff, Jasper J van Doormaal, Jan W van 't Wout, Gregor Verhoef, Wim B J Gerrits, O Aart van Dobbenburgh, Suzanne G Pasmans, and Rob Fijnheer Department of Hematology, University Hospital Groningen, Groningen, The Netherlands Department of Dermatology, University Medical Center Utrecht, Utrecht, The Netherlands Department of Allergology, University Hospital Groningen, Groningen, The Netherlands Department of Internal Medicine, Bronovo Hospital, The Hague, The Netherlands Department of Hematology, University Hosptial Leuven, Leuven, Belgium Department of Hematology, Leyenburg Hospital, The Hague, The Netherlands Department of Internal Medicine, Gelre Hospital, Zutphen, The Netherlands Department of Hematology, University Medical Center Utrecht, Utrecht, The Netherlands * Corresponding author; email: j.c.kluin.nelemans{at}int.azg.nl. Patients with systemic mastocytosis (SM) can suffer from disabling symptoms related to mast cell mediator release or mast cell infiltration requiring mast cell eradication. In the present absence of any curative therapy, a recent case report describing the efficacy of cladribine showed promising results. In a pilot study, the efficacy of cladribine (0.10 till 0.13 mg/kg in a 2 hours infusion, day 1-5; repeated at 4-8 weeks until 6 cycles) was studied. Ten patients with SM suffering of severe symptoms were treated. Four were classified as indolent or smoldering mastocytosis, three as aggressive systemic mastocytosis, and three as SM with an accompanying hematological malignancy. Nine received 6 courses, one patient stopped because of toxicodermia. All responded concerning signs, symptoms and mast cell parameters (serum tryptase and urinary histamine metabolite excretion), though none achieved a complete remission. Prolonged follow-up is required as response is ongoing in most cases. One patient relapsed within 11 months and showed a second response. Side effects were mainly related to bone marrow suppression. Single agent cladribine is an effective and relatively safe treatment for severe systemic mastocytosis. The optimal dose and schedule need to be explored. CiteULike    Connotea    Del.icio.us    Digg    Reddit    Technorati    What's this? This article has been cited by other articles: D. D. Metcalfe Mast cells and mastocytosis Blood, August 15, 2008; 112(4): 946 - 956. [Abstract] [Full Text] [PDF] C. Sucker, G. Mansmann, S. Steiner, N. Gattermann, A. Schmitt-Graeff, R. Loncar, R. E. Scharf, and M. Stockschlader Fatal Bleeding Due to a Heparin-Like Anticoagulant in a 37-Year-Old Woman Suffering From Systemic Mastocytosis Clinical and Applied Thrombosis/Hemostasis, July 1, 2008; 14(3): 360 - 364. [Abstract] [PDF] S. Verstovsek, A. Tefferi, J. Cortes, S. O'Brien, G. Garcia-Manero, A. Pardanani, C. Akin, S. Faderl, T. Manshouri, D. Thomas, et al. 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