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本文报道我院儿科1981～1988年间确诊地中海贫血76例,计β地贫54例,α地贫(HbH病)22例。发病以男多于女(52∶24),贵州籍69例(90.7%),除汉族外以布依族较多(16例)。临床表现以面色苍黄、虚弱、发热、发育营养不良和肝脾肿大多见。重度以上贫血56例(73.6%),网织红增高54/64(84.3%),靶形细胞阳性66例(86.8%),白细胞及血小板减少9例,红细胞渗透脆性降低46/52(88.4%),红细胞包涵体生成试验HbH病全部阳性,β地贫18例均阴性。多有阳性家族史。治疗除输血外,切脾6例,以HbH病疗效较好。并对诊治予以讨论。
This article reports 76 pediatric patients diagnosed with thalassemia from 1981 to 1988 in our hospital, including 54 cases of β-thalassemia and 22 cases of α-thalassemia (HbH). Incidence of more men than women (52:24), Guizhou 69 cases (90.7%), with the exception of Han ethnic Buyi more (16 cases). Clinical manifestations of pale yellow, weak, fever, malnutrition and hepatosplenomegaly are common. There were 56 (73.6%) cases of severe anemia, 54/64 (84.3%) reticulocytes, 66 (86.8%) cases of target cells, 9 cases of white blood cells and thrombocytopenia, 46/52 (88.4% ), Erythrocyte inclusion body formation test all HbH positive, 18 cases of β thalassemia were negative. More positive family history. In addition to blood transfusion, splenectomy in 6 cases, HbH disease is better. And discuss the diagnosis and treatment.