阿尔及利亚镰状细胞β地中海贫血与镰状细胞贫血的比较。

Sickle cell beta-thalassaemia compared with sickle cell anaemia in Algeria.

作者信息

Belhani M, Morle L, Godet J, Bachir D, Henni T, Zerhouni F, Bensenouci A, Colonna P

出版信息

Scand J Haematol. 1984 Apr;32(4):346-50. doi: 10.1111/j.1600-0609.1984.tb00687.x.

DOI:10.1111/j.1600-0609.1984.tb00687.x

PMID:6719040

Abstract

Clinical, haematological and biochemical features in 42 subjects with S-beta thalassaemia (31 subjects with S-beta thalassaemia and 11 subjects with S-beta+ thalassaemia); and in 42 with homozygous sickle cell disease were compared. Persistent splenomegaly was more common and painful crises less common in the S-beta thalassaemia group. Total Hb was higher and reticulocyte count lower in S-beta+ thalassaemia than in S-beta thalassaemia or SS disease. Microcytosis was marked in the S-beta thalassaemia group while the MCV was normal in sickle cell anaemia. Hb F was significantly higher in the S-beta thalassaemia group, without any influence on the severity of the disease. Many features suggest that sickle cell thalassaemia is more severe in Algeria than in Negro subjects and similar to the disease in Italian patients.

摘要

对42例S-β地中海贫血患者（31例S-β地中海贫血患者和11例S-β⁺地中海贫血患者）以及42例纯合子镰状细胞病患者的临床、血液学和生化特征进行了比较。持续性脾肿大在S-β地中海贫血组中更为常见，而疼痛性危象则较少见。S-β⁺地中海贫血患者的总血红蛋白较高，网织红细胞计数较低，低于S-β地中海贫血或镰状细胞病患者。S-β地中海贫血组的小红细胞症明显，而镰状细胞贫血患者的平均红细胞体积正常。S-β地中海贫血组的胎儿血红蛋白明显更高，但对疾病严重程度没有任何影响。许多特征表明，镰状细胞性地中海贫血在阿尔及利亚比在黑人受试者中更为严重，并且与意大利患者的疾病相似。