[Hemoglobin O Arab: about 20 cases]
Tunisie Medicale [La]. 2007; 85 (8): 637-640
en Francés
| IMEMR
| ID: emr-108801
ABSTRACT
Hemoglobin O Arab is a rare abnormal hemoglobin. We report the Clinical and biological features of this disease 20 patients 16 were compound heterozygous Hb O Arab/Beta thalassemia and 4 homozygous Hemoglobin O Arab. Patients are 7 men and 13 women. Most of them are originated from the North West of Tunisia with a age average of 39. 7 years. Diagnosis was carried out at a relatively old age [26. 9 years old]. The homozygous form was not very symptomatic. The compound heterozygous form was more severe and characterized by a mild form of thalassemia with a moderate microcytic hypochromic anaemia [Hb =8. 8 g/dl]. It was often complicated of thrombopenia due to hypersplenism in 40% of the cases. Treatment was based on occasionally transfusion and splenectomy on event of hypersplenism. Evolution of this disease was generally good with a long lifespan of patients. Haemoglobin O Arab is an abnormal hemoglobin well tolerated except for heterozygous category which requires iterative transfusions. Spelenectomy is indicated in case of hypersplenism. The evolution is generally good with a long survival
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Índice:
IMEMR (Mediterraneo Oriental)
Asunto principal:
Esplenectomía
/
Hemoglobinopatías
/
Hiperesplenismo
Límite:
Femenino
/
Humanos
/
Masculino
Idioma:
Francés
Revista:
Tunisie Med.
Año:
2007
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