title: Phlebotomy to reduce iron overload in patients cured of thalassemia by bone marrow transplantation. Italian Cooperative Group for Phlebotomy Treatment of Transplanted Thalassemia Patients.
abstract: In thalassemia after successful bone marrow transplantation (BMT), iron overload remains an important cause of morbidity. After BMT, patients have normal erythropoiesis capable of producing a hyperplastic response to phlebotomy so that this procedure can be contemplated as a method of mobilizing iron from overloaded tissues. A phlebotomy program (6 mL/kg blood withdrawal at 14-day intervals) was proposed to 48 patients with prolonged follow-up (range, 2 to 7 years) after BMT. Seven patients were not submitted to the program (five because of refusal and two because of reversible side effects). The remaining 41 patients (mean age, 16 +/- 2.9 years) were treated for a mean period of 35 +/- 18 months. All were evaluated before and after 3 +/- 0.6 years of follow-up. Values are expressed as mean +/- standard deviation (SD) or as median with a range (25 to 75 percentile). Serum ferritin decreased from 2,587 (2,129 to 4,817) to 417 (210 to 982) microg/L (P < .0001), total transferrin increased from 2.34 +/- 0.37 to 2.7 +/- 0.58 g/L (P = .0001), transferrin saturation decreased from 90% +/- 14% to 50% +/- 29% (P < .0001). Liver iron concentration evaluated on liver biopsy specimens decreased from 20.8 (15.5 to 28.1) to 4.2 (1.6 to 14.6) mg/g dry weight (P < .0001). Aspartate transaminase decreased from 2.7 +/- 2 to 1.1 +/- 0.6 (P < .0001) and alanine transaminase from 5.2 +/- 3.4 to 1.7 +/- 1.2 (P < .0001) times the upper level of normality. The Knodell score for liver histological activity decreased from 6.9 +/- 3 to 4.9 +/- 2.8 (P < .0001). These data indicate that phlebotomy is safe, efficient, and widely applicable to ex-thalassemics after BMT.
authors.affiliation: Divisione di Ematologia e Centro Trapianto Midollo Osseo di Muraglia, and Azienda Ospedale di Pesaro, Italy.
authors.forename: E
authors.initials: E
authors.lastname: Angelucci
authors.forename: P
authors.initials: P
authors.lastname: Muretto
authors.forename: G
authors.initials: G
authors.lastname: Lucarelli
authors.forename: M
authors.initials: M
authors.lastname: Ripalti
authors.forename: D
authors.initials: D
authors.lastname: Baronciani
authors.forename: B
authors.initials: B
authors.lastname: Erer
authors.forename: M
authors.initials: M
authors.lastname: Galimberti
authors.forename: C
authors.initials: C
authors.lastname: Giardini
authors.forename: D
authors.initials: D
authors.lastname: Gaziev
authors.forename: P
authors.initials: P
authors.lastname: Polchi
mesh_terms: D000293:Adolescent; D016026:Bone Marrow Transplantation; D015913:Chelation Therapy; D002648:Child; D003131:Combined Modality Therapy; D003676:Deferoxamine / Q000627:therapeutic use; D004920:Erythropoiesis; D005260:Female; D005293:Ferritins / Q000097:blood; D005500:Follow-Up Studies; D006801:Humans; D007501:Iron / Q000032:analysis; D019190:Iron Overload / Q000097:blood / Q000209:etiology / Q000628:therapy*; D008099:Liver / Q000737:chemistry; D008103:Liver Cirrhosis / Q000517:prevention & control; D008297:Male; D018962:Phlebotomy; D017262:Siderophores / Q000627:therapeutic use; D013789:Thalassemia / Q000628:therapy*; D065227:Transfusion Reaction; D016896:Treatment Outcome
chemical_list: D017262:Siderophores; D005293:Ferritins; D007501:Iron; D003676:Deferoxamine