PULMONARY-EMBOLISM DEVELOPING IN PATIENTS WITH SICKLE-CELL DISEASE ONHYPERTRANSFUSION AND IV DEFEROXAMINE CHELATION-THERAPY

Pulmonary disease, including thromboembolic problems, accounts for a l arge portion of the morbidity of sickle cell disease. Chronic transfus ion therapy is now a part of long-term treatment of sickle cell patien ts with stroke and chest syndrome. The resultant iron overload must be treated with chelation therapy using deferoxamine. Poor compliance wi th subcutaneous chelation therapy has necessitated intravenous deferox amine treatment. We describe two patients with sickle cell disease on such a regimen, who became hypoxic as a result of pulmonary thromboemb olism, secondary to venous thrombophlebitis. The thrombophlebitis and subsequent pulmonary embolism probably reflect the hypercoagulable sta te seen in sickle cell and are not due to the deferoxamine therapy.