Thalassemia intermedia : About 36 cases

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Published Feb 17, 2010
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Vol. 88 No. 2 (2010): Issue Feb 2010

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Raouf Hafsia
Naouel Ben Salah
Emna Hafhouf
Fatma Belakhal
Emna Gouider
Wijden El Borgi
Balkis Meddeb

Abstract

Background : Thalassemia intermedia empasses a mild clinical and biological spectrum
The aim is to report the clinical and biological features and treatment of this disease.
Methods: It is a retrospective study about 36 thalassemia intermedia patients (17 males, 19 females). Epidemiological, haematological aspects and treatment were reported
Results: The diagnosis was carried out at a relatively old age 15 years (1-72 ).The thalassemia intermedia was characterized by mild facial deformities, splenomegaly and moderate anemia ( Hb= 9.1 g/dl). The mean serum ferritin was 518 ng/ml (25-1800).Three phenotypes are caracterised: heterozygosis beta thalassemia, ß° thalassemia and ß + thalassemia. Clinical complications were hypersplenism, extra medullary hematopoiesis, leg ulcers, thrombosis and pulmonary hypertension. Treatment was based on occasionally transfusion and splenectomy on event of hypersplenious (47%). Evolution of this disease was generally good with a long lifespan at 31 years (6-83).
Conclusion: Thalassemia intermedia is well tolerated. Transfusions and splenectomy were indicated in case of hypersplenious.

Keywords:

Thalassemia intermedia, Diagnosis, Complications, Treatment

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