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CHELATION THERAPY IN MAJOR β THALASSEMIA

Mirbehbehani, N. (2009) CHELATION THERAPY IN MAJOR β THALASSEMIA. HAEMATOLOGICA-THE HEMATOLOGY JOURNAL, 94. pp. 519-520.

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Abstract

Background. Progressive iron overload is the life-limiting complication of transfusion therapy.Effective and convenient iron chelation remains one of the main targets of clinical management of thalassemia major. The combined treatment with deferoxamine (DFX) and Deferiprone (DFO) could have an increased chelation efficacy. Aims: Deferiprone may play a role in shuttling iron within membranes and within intracellular pools.We compare DFX alone with combined therapy with DFX and DFO in b thalassemia patients with iron overload. Design and Methods. We studied 28 patients with b thalassemia major in two group (mean age±SD, 20.02±5.3; range 10-34 years) attending the day care unit for regular transfusional support. They received packed red cells every 3-4 weeks to maintain pretransfusion hemoglobin major concentration above 9 g/dl. They had been receiving DFX at a daily dose of 40 mg/kg/d by subcutaneous infusion for 8-10 h on 5-6 nights each week for the past several years. (DFX) alone (40-50 mg/kg/d S.C. over 8-12 h, five times weekly) was compared with combined DFX 40 mg/kg/d (3times a week) and DFO 75 mg/kg/d daily over 6 months period. Serum ferritin level and side effects were monitored over a 6 month period. Results. Serum ferritin fell from98/3434±83/7539 μg/L (mean±SD) to 26/2706±66/4848 μg/L(p<0.001) and to75/2308±33/4338 (p<0.001) after three and six month in the combined group and raised from83/3613±00/5668 μg/L to 93/3940±81/6210 μg/L (P153/0≤) and to99/3205±07/5742 (p/0≤902) after three and six month in the DFX group. The most common adverse events were gastrointestinal symptoms (8.25%) and joint pain (8.25%) and transaminasemia (21%) in combined therapy. Conclusions. The availability of effective oral chelators would be a major clinical advance.Acording our study combined therapy with DFX and DFO is a practical and effective procedure to decrease severe iron overload in patients with thalassemia major.

Item Type: Article
Additional Information: 14th Annual Meeting of the European-Hematology-Association, Berlin, GERMANY, JUN 04-07, 2009
Subjects: مقالات نمایه شده محققین دانشگاه در سایت ,Web of Science ,Scopus
موارد کلی
Divisions: معاونت تحقیقات و فناوری
Depositing User: GOUMS
Date Deposited: 27 Apr 2015 06:12
Last Modified: 23 Jun 2017 17:11
URI: http://eprints.goums.ac.ir/id/eprint/4061

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