Aspirin-responsive painful red, blue, black toe, or finger syndrome in polycythemia vera associated with thrombocythemia View Full Text


Ontology type: schema:ScholarlyArticle     


Article Info

DATE

2003-02-12

AUTHORS

J. J. Michiels, Z. Berneman, W. Schroyens, H. van Urk

ABSTRACT

Five patients with red, purple blue, or black toes or fingers due to thrombocythemia associated with polycythemia vera (polycythemia and thrombocythemia vera) in four and essential thrombocythemia (thrombocythemia vera) in one are described. The microvascular erythromelalgic syndrome of thrombocythemia was overlooked and progressed to cold blue swollen and painful fingers or black toes in three patients with polycythemia and thrombocythemia vera due to arteriographically documented occlusions of digital or large peripheral arteries with no evidence of preexistent atherosclerotic vascular disease. Concomitant erythromelalgia of the hand palm could be confirmed by the histopathological findings of arteriolar thrombotic lesions in the reticular dermis in two patients with polycythemia and thrombocythemia vera. The increased hematocrit in the presented patients with polycythemia and thrombocythemia vera contributed to the progression of the microvascular syndrome of thrombocythemia to major occlusive ischemic events of the extremities. Standard therapy with oral anticoagulants and reduction of the hematocrit to normal by bloodletting did not affect the platelet-mediated microvascular erythromelalgic, ischemic symptoms in the patients with polycythemia vera because thrombocythemia vera persisted. Complete relief of pain and restoration of the ischemic acral circulation disturbances in patients with thrombocythemia vera or thrombocythemia associated with polycythemia vera in maintained remission by bloodletting could be obtained by long-term treatment with low-dose aspirin. More... »

PAGES

153-159

Identifiers

URI

http://scigraph.springernature.com/pub.10.1007/s00277-002-0593-x

DOI

http://dx.doi.org/10.1007/s00277-002-0593-x

DIMENSIONS

https://app.dimensions.ai/details/publication/pub.1075251726

PUBMED

https://www.ncbi.nlm.nih.gov/pubmed/12634947


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197 schema:name Hemostasis and Thrombosis Research, Department of Hematology, University Hospital Antwerp, The Netherlands
198 The Goodheart Institute, MPD Center Europe and International Science Center of Hemostasis, Thrombosis and Vascular Pathology, Erasmus Tower, Veenmos 13, 3069 AT, Rotterdam, The Netherlands
199 rdf:type schema:Organization
200 grid-institutes:grid.5645.2 schema:alternateName Department of Vascular Surgery, University Hospital Dijkzigt, Erasmus University Rotterdam, The Netherlands
201 schema:name Department of Vascular Surgery, University Hospital Dijkzigt, Erasmus University Rotterdam, The Netherlands
202 rdf:type schema:Organization
 




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