Erythromelalgia and thrombocythemia: A causal relation

Primary thrombocythemia: diagnosis, clinical manifestations and management

“…Histopathological examination of erythromelalgic areas demonstrates a thrombotic occlusion and inflammation of the arterial microvasculature of the extremities, without evidence of pre-existing vasculature [36]. This characteristic microvascular thrombotic complication of thrombocythemia in its primary form or in association with polycythemia vera may already arise at platelet counts above 400 x 109/1 and may therefore provide an early clue to the diagnosis of thrombocythemia [34,36,37]. In contrast to the complete ineffectiveness of coumadin, treatment with low-dose acetylsalicylic acid (ASA) provides lasting relief of the erythromelalgic distress quickly [34,36,37].…”

“…Simple laboratory investigations including bone marrow smear and biopsy most likely yield clues as to whether an elevated platelet count is primary and persistent. Markers suggestive of PT are an increase of clustered mature megakaryocytes with multilobulated nuclei and the presence of reticulin fibers in bone marrow biopsy material in the absence of any other underlying disorder [11,18,34]. In some cases, a clear distinction between the different MPD cannot be made, and these patients are diagnosed as having an "overlap MPD" until further evolution of the clinical and laboratory features leads to a more clearly delineated form.…”

“…Pregnancy in PT is frequently complicated by recurrent spontaneous abortions and fetal growth retardation, probably due to multiple placental infarctions that lead to chronic placental insufficiency [51. 59 prerequisite for the development of erythromelalgic distress. The disabling and ASA-responsive burning distress of erythromelalgia is usually preceded by acroparesthesias (e.g., tingling, "pins and needles" sensations, and numbness in the toes or fingers) [34,36,37]. It left untreated, erythromelalgia usually proceeds to painful acrocyanosis and peripheral gangrene with preservation of normal peripheral arterial pulses [34,36,37].…”

“…These functional ischemic symptoms are attributed to in vivo platelet activation in the arterioles, with or without thrombosis per se, and may resolve dramatically after intake of the anti-aggregating agent aspirin or reduction of the platelet count to normal [34,39,46,55]. Of these symptoms, erythromelalgia [17,34], featured by painful, burning, red, congested toes or fingers, deserves special mention. Histopathological examination of erythromelalgic areas demonstrates a thrombotic occlusion and inflammation of the arterial microvasculature of the extremities, without evidence of pre-existing vasculature [36].…”

Erythromelalgia: A Clue to the Diagnosis of Polycythemia vera

“…Such a variance dictates important therapeutic consequences. Erythromelalgia is causally related to thrombocythemia [14] which can be idiopathic or related to myelo proliferative disorders 115]. The clinical pic ture is based on arteriolar inflammation and thrombosis mediated by dysfunctional plate lets.…”