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Erythromelalgia in thrombocythemia of various myeloproliferative disorders
Author(s) -
Michiels Jan J.,
ten Kate Fiebo J. W.
Publication year - 1992
Publication title -
american journal of hematology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 2.456
H-Index - 105
eISSN - 1096-8652
pISSN - 0361-8609
DOI - 10.1002/ajh.2830390211
Subject(s) - erythromelalgia , myelofibrosis , essential thrombocythemia , medicine , polycythemia vera , myeloproliferative disorders , thrombocytosis , thrombosis , aspirin , dermatology , anagrelide , platelet , gastroenterology , bone marrow
Erythromelalgia is caused by platelet‐mediated acral inflammation and arteriolar thrombosis in thrombocythemia in its primary form or associated with polycythemia vera. The prompt and lasting relief of burning pain by low‐dose aspirin is a prerequisite for the diagnosis of thrombocythemic erythromelalgia. Here we extend observations on the occurrence of erythtromelalgia in thrombocythemia associated with primary myelofibrosis, Philadelphia‐chromosome positive micromegakaryocytic myelofibrosis, and myelodysplastic syndrome type II. It is concluded that erythromelalgia may occur in thrombocythemia of all variants of chronic myeloproliferative disease as well as myelodysplastic syndrome if platelet counts are sufficiently high.