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Recurrent thromboembolic disease following splenectomy for pyruvate kinase deficiency
Author(s) -
Chou Roger,
DeLoughery Thomas G.
Publication year - 2001
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.1107
Subject(s) - splenectomy , medicine , pyruvate kinase deficiency , hemolytic anemia , pyruvate kinase , complication , chronic thromboembolic pulmonary hypertension , anemia , disease , pulmonary hypertension , spleen , gastroenterology , surgery , glycolysis , metabolism
We report a case of recurrent thromboembolic disease and chronic pulmonary hypertension in an adult patient with pyruvate kinase deficiency who underwent splenectomy as a child. Thromboembolism has been reported as a complication following splenectomy for various hereditary chronic hemolytic anemias. To our knowledge, this association has not been described in patients specifically with pyruvate kinase deficiency. Our patient presented at age 37 with recurrent pulmonary emboli, 36 years after splenectomy for severe hemolytic anemia. Work‐up for other hypercoagulable states was negative. The mechanism for hypercoagulability in this condition is unclear but may involve a quantitative or qualitative change in disrupted thrombogenic red blood cell membranes that would normally be removed by the spleen. Clinicians should have a high index of suspicion for thrombotic events in these patients, as early diagnosis and treatment can reduce morbidity and mortality, and chronic anticoagulation may help prevent the sequelae of repeated thromboembolic events. Am. J. Hematol. 67:197–199, 2001. © 2001 Wiley‐Liss, Inc.