[Thalassemia intermedia: 36 cases]

Tunis Med. 2010 Feb;88(2):102-4.
[Article in French]

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, 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).

Conclusion: Thalassemia intermedia is well tolerated. Transfusions and splenectomy were indicated in case of hypersplenious.

Publication types

  • English Abstract

MeSH terms

  • Adolescent
  • Adult
  • Aged
  • Aged, 80 and over
  • Child
  • Craniofacial Abnormalities / etiology
  • Female
  • Humans
  • Male
  • Middle Aged
  • Retrospective Studies
  • Splenomegaly / etiology
  • Thalassemia / complications*
  • Thalassemia / therapy*
  • Young Adult