NATIONAL CLINICAL RECOMMENDATIONS FOR THE DIAGNOSIS AND TREATMENT OF MASTOCYTOSIS

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

Russian journal of hematology and transfusiology（2021）

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Abstract

Introduction. Recommendations cover the current state of diagnosis and treatment of mastocytosis. Aim - a 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.

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Key words

mastocytosis, systemic mastocytosis, cutaneous mastocytosis, mast cell leukaemia, KITD816V, prognosis, midostaurin, imatinib, cladribine, hydroxy-carbamide, interferon alpha, haematopoietic stem cell transplantation

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