[Thalassemia intermedia: 36 cases].
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).


CONCLUSIONS
Thalassemia intermedia is well tolerated. Transfusions and splenectomy were indicated in case of hypersplenious.
Hafsia
Raouf
R
Service d'Hématologie Biologique, Hôpital Aziza Othmana, Tunis.
Ben Salah
Naouel
N
Hafhouf
Emna
E
Belakhal
Fatma
F
Gouider
Emna
E
El Borji
Wijdene
W
Meddeb
Balkis
B
D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D002648:Child; D019465:Craniofacial Abnormalities / Q000209:etiology; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D012189:Retrospective Studies; D013163:Splenomegaly / Q000209:etiology; D013789:Thalassemia / Q000150:complications* / Q000628:therapy*; D055815:Young Adult