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Primary thrombocythaemia associated with systemic mastocytosis: a report of five cases
Author(s) -
Tourneau A. Le,
Gaulard P.,
D'Agay M. F.,
Vainchencker W.,
Cadiou M.,
Devidas A.,
Haioun C.,
Clauvel J. P.,
Audouin J.,
Diebold J.
Publication year - 1991
Publication title -
british journal of haematology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.907
H-Index - 186
eISSN - 1365-2141
pISSN - 0007-1048
DOI - 10.1111/j.1365-2141.1991.tb08011.x
Subject(s) - systemic mastocytosis , medicine , bone marrow , biopsy , cutaneous mastocytosis , pathology , urticaria pigmentosa , dermatology
Mastocytosis is an uncommon disease characterized by a proliferation of tissue mast cells involving various organs, particularly the bone marrow. Though rare, the association of mastocytosis and haematological malignancies is well established. However, the frequency of mastocytosis reported in patients with essential thrombocythaemia is relatively low. We report five cases of such an association. Our five patients were undergoing evaluation for thrombocy‐when the bone marrow biopsy revealed the presence of mastocytosis. The pathogenetic significance of this association is poorly understood. The different hypotheses are discussed.