National clinical recommendations for the diagnosis and treatment of mastocytosis

Author:

Melikyan A. L.1ORCID,Subortseva I. N.1ORCID,Shuvaev V. A.2ORCID,Morozova E. V.3ORCID,Lukina K. A.1ORCID,Artemieva S. I.4ORCID,Livov A. N.5ORCID,Baykov V. V.3ORCID,Vinogradova O. Yu.6ORCID,Galstyan G. M.1ORCID,Gilyazitdinova E. A.1ORCID,Dvirnyk V. N.1ORCID,Zhukova O. V.4ORCID,Kovrigina A. M.1ORCID,Kohno A. V.1ORCID,Kuzmina L. A.1ORCID,Lomaia E. G.7ORCID,Lukina E. A.1ORCID,Martynkevich I. S.2ORCID,Mitina T. A.6ORCID,Potekaev N. N.8ORCID,Sudarikov A. B.1ORCID,Shatokhina E. A.9ORCID,Frigo N. V.4ORCID,Savchenko V. G.1ORCID

Affiliation:

1. National Research Center for Hematology

2. Russian Research Institute of Hematology and Transfusiology

3. Raisa Gorbacheva Memorial Research Institute for Pediatric Oncology, Hematology and Transplantation, Pavlov First Saint-Petersburg State Medical University

4. Moscow Scientific and Practical Center of Dermatovenereology and Сosmetology

5. Moscow Scientific and Practical Center of Dermatovenereology and Сosmetology;

6. Botkin Hospital Moscow Hematology Center

7. Almazov National Medical Research Centre

8. Moscow Scientific and Practical Center of Dermatovenereology and Сosmetology; Pirogov Russian National Research Medical University

9. National Research Center for Hematology; Central State Medical Academy of the Russian President Administration

Abstract

Introduction. Recommendations cover the current state of diagnosis and treatment of mastocytosis.Aim — а consolidation of the Russian experts’ opinion on treatment for adult mastocytosis.Main findings. The recommendations have been developed taking into account foreign literature, national experience and world clinical evidence on therapy for systemic and cutaneous mastocytoses, mast cell leukaemia and other mastocytosis forms. The significance of bone marrow and peripheral blood molecular genetic testing for the presence of KITD816V gene variants is demonstrated. The treatment regimens described are based on midostaurin, imatinib, cladribine, hydroxycarbamide, interferon alfa and haematopoietic stem cell transplantation. Prognosis in different forms of mastocytosis is provided.

Publisher

National Medical Research Center of Hematology of the Ministry of Health of the Russian Federation

Subject

Hematology

Cited by 4 articles. 订阅此论文施引文献 订阅此论文施引文献,注册后可以免费订阅5篇论文的施引文献,订阅后可以查看论文全部施引文献

1. Difficulties of primary diagnosis in patients with suspected systemic mastocytosis;Oncohematology;2024-09-02

2. Targeted therapy for advanced forms of systemic mastocytosis in real clinical practice;Oncohematology;2023-12-07

3. A rare case of cutaneous mastocytosis in pediatric practice;Rossiyskiy Vestnik Perinatologii i Pediatrii (Russian Bulletin of Perinatology and Pediatrics);2023-11-16

4. Clinical Cases: Diagnostic Errors of Mastocytosis Cutaneous Forms in Children;Pediatric pharmacology;2022-07-12

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