title: Pulmonary embolism developing in patients with sickle cell disease on hypertransfusion and IV deferoxamine chelation therapy.
abstract: Pulmonary disease, including thromboembolic problems, accounts for a large portion of the morbidity of sickle cell disease. Chronic transfusion therapy is now a part of long-term treatment of sickle cell patients with stroke and chest syndrome. The resultant iron overload must be treated with chelation therapy using deferoxamine. Poor compliance with subcutaneous chelation therapy has necessitated intravenous deferoxamine treatment. We describe two patients with sickle cell disease on such a regimen, who became hypoxic as a result of pulmonary thromboembolism, secondary to venous thrombophlebitis. The thrombophlebitis and subsequent pulmonary embolism probably reflect the hypercoagulable state seen in sickle cell and are not due to the deferoxamine therapy.
authors.affiliation: Division of Pediatric Hematology, 180 Fort Washington Avenue, HP 5-554, New York, NY 10032, USA.
authors.forename: S
authors.initials: S
authors.lastname: Sheth
authors.forename: C
authors.initials: C
authors.lastname: Ruzal-Shapiro
authors.forename: A
authors.initials: A
authors.lastname: Hurlet-Jensen
authors.forename: S
authors.initials: S
authors.lastname: Piomelli
authors.forename: W E
authors.initials: WE
authors.lastname: Berdon
mesh_terms: D000293:Adolescent; D000755:Anemia, Sickle Cell / Q000150:complications / Q000628:therapy*; D002405:Catheterization, Central Venous; D015913:Chelation Therapy / Q000009:adverse effects*; D003676:Deferoxamine / Q000009:adverse effects* / Q000627:therapeutic use; D005260:Female; D006801:Humans; D007501:Iron; D019190:Iron Overload / Q000188:drug therapy / Q000209:etiology; D008297:Male; D011655:Pulmonary Embolism / Q000209:etiology*; D065227:Transfusion Reaction
chemical_list: D007501:Iron; D003676:Deferoxamine