Michiels J J, ten Kate F J
Department of Hematology, University Hospital, Erasmus University Rotterdam, The Netherlands.
Am J Hematol. 1992 Feb;39(2):131-6. doi: 10.1002/ajh.2830390211.
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 erythromelalgia 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.
红斑性肢痛症是由血小板介导的肢端炎症和原发性血小板增多症或真性红细胞增多症相关的小动脉血栓形成引起的。低剂量阿司匹林能迅速且持久地缓解灼痛是血小板增多性红斑性肢痛症诊断的前提条件。在此，我们扩展了对与原发性骨髓纤维化、费城染色体阳性微小巨核细胞性骨髓纤维化和II型骨髓增生异常综合征相关的血小板增多症中红斑性肢痛症发生情况的观察。得出的结论是，如果血小板计数足够高，红斑性肢痛症可能发生在所有慢性骨髓增殖性疾病变体以及骨髓增生异常综合征的血小板增多症中。
