Recurrent thromboembolic disease following splenectomy for pyruvate kinase deficiency

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...

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Bibliographic Details
Published in:American journal of hematology 2001-07, Vol.67 (3), p.197-199
Main Authors: Chou, Roger, DeLoughery, Thomas G.
Format: Article
Language:English
Subjects:
Adult
Anemia, Hemolytic, Congenital Nonspherocytic - complications
Anemia, Hemolytic, Congenital Nonspherocytic - surgery
Anemias. Hemoglobinopathies
Anticoagulants - therapeutic use
Biological and medical sciences
Blood Transfusion
Combined Modality Therapy
Diseases of red blood cells
Dyspnea - etiology
Dyspnea - therapy
Female
Femoral Vein
Genotype
Hematologic and hematopoietic diseases
hemolytic anemia
Humans
Iliac Vein
Medical sciences
Oxygen Inhalation Therapy
pulmonary embolism
Pulmonary Embolism - etiology
pyruvate kinase
Pyruvate Kinase - deficiency
Recurrence
splenectomy
Splenectomy - adverse effects
Thrombophilia - drug therapy
Thrombophilia - etiology
thrombosis
Venous Thrombosis - etiology
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Summary: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.
ISSN:0361-8609
1096-8652
DOI:10.1002/ajh.1107