La Tunisie medicale · 2007
[Hemoglobin O Arab: about 20 cases].
Аннотация на английскомСтатьяНазвание и аннотация: Перевод пока не подготовлен
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Hafsia Raouf, Gouider Emna, Ben Moussa Sinda, Ben Salah Naouel, Elborji Wijdene, Hafsia Aicha
Аннотация
BACKGROUND: Hemoglobin O Arab is a rare abnormal hemoglobin.
AIM: We report the Clinical and biological features of this disease
METHODS: 20 patients.:16 were compound hétérozygous Hb O Arab/Béta thalassemia and 4 homozygous Hemoglobin O Arab. Patients are 7 men and 13 women.
RESULTS: 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.
CONCLUSION: Haemoglobin O Arab is an abnormal hemoglobin well tolerated except for heterozygous category which requires iterative transfusions. Spelenectomy is indicated in case of hypersplenious. The evolution is generally good with a long survival.
AIM: We report the Clinical and biological features of this disease
METHODS: 20 patients.:16 were compound hétérozygous Hb O Arab/Béta thalassemia and 4 homozygous Hemoglobin O Arab. Patients are 7 men and 13 women.
RESULTS: 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.
CONCLUSION: Haemoglobin O Arab is an abnormal hemoglobin well tolerated except for heterozygous category which requires iterative transfusions. Spelenectomy is indicated in case of hypersplenious. The evolution is generally good with a long survival.