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先天性纯红再障是以骨髓红细胞系统增生极度低下,外周血网织红细胞低或缺如,而白细胞和血小板正常或接近正常为本病的主要特征,我院共收治12例,现报告如下: 1 一般资料 12例中,男8例,女4例,最小1例3个月,最大3岁。出生6个月开始出现贫血者11例,12例中均有明显面黄和精神萎靡等贫血症状,无出血倾向,无肝及淋巴结肿大,3例有轻度脾肿大,12例中伴先天性心脏病1例,肾发育不全2例。 实验室检查:血象:血红蛋白<30g/L3例,～60g/L8例,～90g/L1例,网织红细胞0者9例,<0.5％者4例,白细胞总数和血小板均在正常范围。骨髓象:骨髓增生活跃者11例,增生减低者1例,有核红细胞缺乏7例,有核红细胞所占比例,除1例7％,1例7.8％,余10例均在5％以下。
Congenital pure azoospermia is bone marrow erythrocyte system hyperplasia is extremely low, peripheral blood reticulocyte is low or absent, while the white blood cells and platelets normal or nearly normal for the main features of this disease were treated in our hospital were 12 cases, are as follows : 1 General Information 12 cases, 8 males and 4 females, a minimum of 3 months, maximum 3 years old. Eleven anemia occurred in 6 months after birth, anemia was obvious in both facial and apathetic manifestations in 12 cases, no bleeding tendency, no liver and lymph nodes, 3 cases with mild splenomegaly, and 12 cases accompanied by 1 case of congenital heart disease, 2 cases of renal hypoplasia. Laboratory tests: blood: hemoglobin <30g / L3 cases, ~ 60g / L 8 cases, ~ 90g / L1 cases, reticulocyte 0 in 9 cases, <0.5% in 4 cases, the total number of white blood cells and platelets are in the normal range. Bone marrow: active bone marrow hyperplasia in 11 cases, 1 cases of hyperplasia, a lack of nucleated erythrocyte in 7 cases, the proportion of nucleated red blood cells, except for 1 case of 7%, 1 case of 7.8%, the remaining 10 cases were below 5%.