Thalassemia intermedia: 36 cases
Hafsia, R.; Ben Salah, N.; Hafhouf, E.; Belakhal, F.; Gouider, E.; El Borji, W.; Meddeb, B.
La Tunisie Medicale 88(2): 102-104
2010
ISSN/ISBN: 0041-4131 PMID: 20415168 Document Number: 642942
Thalassemia intermedia empasses a mild clinical and biological spectrum. The aim is to report the clinical and biological features and treatment of this disease. It is a retrospective study about 36 thalassemia intermedia patients (17 males, 19 females). Epidemiological, haematological aspects and treatment were reported 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, beta degrees thalassemia and beta + 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). Thalassemia intermedia is well tolerated. Transfusions and splenectomy were indicated in case of hypersplenious.