Systemic Mastocytosis: Response to the Efficacy of Cladribine(2-CdA) with Current Terminology and Approach
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Systemic Mastocytosis: Response to the Efficacy of Cladribine(2CdA) with Current Terminology and Approach Irfan Yavas¸ og˘lu1
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Atakan Turgutkaya1
Received: 30 July 2020 / Accepted: 6 August 2020 Ó Indian Society of Hematology and Blood Transfusion 2020
Keywords Mastocytosis WHO Myeloproliferative Dear Editor, The article entitled ‘‘The Efficacy of Cladribine (2-CdA) in Advanced Systemic Mastocytosis’’ written by Helbig et al. and published in your journal was quite interesting [1]. Herein, we wish to make some contributions to the article. World Health Organisation(WHO) 2016 is currently used in the evaluation of systemic mastocytosis(SM). By the terminology of WHO 2008; ‘‘Systemic Mastocytosis With An Associated Clonal Hematological Non-mast Cell Disease ( SM-AHMND)’’ has been replaced with the term ‘‘Systemic Mastocytosis With An Associated Hematological Neoplasm (SM-AHN)’’ in WHO 2016 classification of mastocytosis. Also the term ‘‘advanced SM’’ should be replaced with ‘‘aggressive SM’’ accordingly. By the novel update, advanced SM is a revised term for aggressive SM, SM-AHN and mast cell leukemia. The Overall Response Rate (ORR) was written as 67% in table 2; inconsistent with the text. Also in the table, the parameter ‘‘Loss of response (LOR)’’ can be added according to International Working Group—Myeloproliferative Neoplasms Research and Treatment (IWGMRT) & European Competence Network on Mastocytosis. The response duration must be at least C 12 week to evaluate, therefore evaluation of your cases might be insufficient due to short (1–2 cycle) & Atakan Turgutkaya [email protected] Irfan Yavas¸ og˘lu [email protected] 1
Division of Hematology, Adnan Menderes University Medical Faculty, Aytepe Location, 09010 Efeler, Aydın, Turkey
treatment duration. Also, obtaining the data about previous treatments such as interferon and imatinib might contribute to interpret the response to cladribine more accurately. In this context imatinib may be the first choice for eosinophilia with FIP1L1-PDGFRA mutated or KITD816V nonmutated cases. The therapeutic algorithm of aggressive SM and SM-AHN are different. The approach intended to underlying disease is more important for SM-AHN. Patients can experience transient responses to cladribine, but nearly all of them will eventually relapse. Prophylaxis to prevent Pneumocystis jirovecii pneumonia is suggested for at least three months after therapy is complete and until the CD4 count is [ 200/microL. Mayo clinical model and The Mutation-Adjusted Risk Score (MARS) are prognostic models which can be beneficial for the management [2–5]. As a conclusion individualized treatment algorithm should be applied for all SM cases.
Author contributions Concept—I.Y., A.T., Design—A.T., I.Y.; Supervision—I.Y..; Resources—I.Y., A.T.; Materials—I.Y., A.T.; Data Collection and/or Processing—A.T., I.Y.,; Analysis and/or Interpretation–I.Y., A.T.; Literature Search—A.T.; Writing Manuscript—A.T.; Critical Review—I.Y., A.T. Funding None. Compliance with ethical s
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