Smoldering systemic mastocytosis. Successful therapy with cladribine

Schleyer, Verena and Meyer, S. and Landthaler, M. and Szeimies, R. M. (2004) Smoldering systemic mastocytosis. Successful therapy with cladribine. HAUTARZT, 55 (7). pp. 658-662. ISSN 0017-8470,

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Abstract

Mastocytoses are a heterogenous group of diseases characterized by proliferation and accumulation of mast cells in the skin and other organs. They are subdivided into cutaneous mastocytoses; systemic forms, which may appear with or without skin lesions; mast cell sarcomas and extracutaneous, localized, benign mastocytomas. Systemic mastocytoses apart from the skin mainly involve bone marrow, gastrointestinal tract, bones, lymph nodes, spleen and liver. Whereas indolent forms of systemic mastocytosis are mainly treated with antihistamines, glucocorticosteroids and PUVA therapy, the more aggressive forms, including mast cell leukemia, often require cytostatic chemotherapy. A 53-year old patient with beginning "smoldering systemic mastocytosis" failed to respond to high-dose systemic glucocorticosteroids and interferon-alpha. Treatment with clacribine led to an impressive improvement of skin lesions,a significant decrease in tryptase serum levels and stabilization of bone marrow infiltrates.

Item Type: Article
Uncontrolled Keywords: MAST-CELL DISEASE; URTICARIA PIGMENTOSA; CLASSIFICATION; PUVA; mastocytosis; urticaria pigmentosa; cladribine; glucocorticosteroids; interferon-alpha
Subjects: 600 Technology > 610 Medical sciences Medicine
Divisions: Medicine > Lehrstuhl für Dermatologie und Venerologie
Depositing User: Dr. Gernot Deinzer
Date Deposited: 07 Jul 2021 15:40
Last Modified: 07 Jul 2021 15:40
URI: https://pred.uni-regensburg.de/id/eprint/37489

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