Aspirin responsive platelet thrombophilia in essential thrombocythemia and polycythemia vera

Figure 7 Acrocyanotic ischemic progression in a case of polycythemia vera 6 mo after presentation of erythromelalgia in the fingers of the right hand caused by endthrombotic occlusions of the superficial arterial arcade of the digital arteries. A: A skin biopsy taken from the blue ischemic spot of the handpalm (arrow) showed an occluded arteriole consistent with thrombo-angiitis obliterans; B: The erythromelalgic acrocyanotic congested fingers persited after treatment of polycythemia vera with phlebotomy for 1 mo (4 wk), but treatment with aspirin during a subsequent second month (4 wk) induced complete relief of the painfull blue swelling, which was associated with a significant increase of circulating platelets counts from below to above 400 × 10⁹/L. Four weeks of phlebomotomy treatment (horizontal axis) had no effect on acrocyanotic erythromelalgia of the fingers, and subsequent treatment with aspirin one dose daily for several weeks was associated with the disappearance of painfull acrocyantic erythromelalic sign and symptoms and a significant rise in circulating platelet counts of about 200 × 10⁹/L. Originated from Michiels et al[83].