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再生障碍性贫血——阵发性睡眠性血红蛋白尿综合征(AA-PNH综合征),1952年由Letman首次报告,近年来我们遇到7例,报告如下。临床资料1.性别及年龄:男6例,女1例.发病年龄30～54岁,病程5个月～8年,平均2年1个月. 2.临床表现:患者多为贫血、发热(2例),贫血、出血(4例),以出血为主(1例)。3例巩膜有轻度黄染,无淋巴结肿大,肝肿大者3例,最大者肋下2.5cm;脾肿大者3例,最大侧位2.5cm。7例中,血红蛋白尿频发(每月1次以上)3例,偶发(数月发作1次)1例,8例尿色均呈酱油色,1例呈浓茶色。不发作者8例。5.实验室检查:6例入院时血红蛋白明显低于正常,60～90g/L;1例正常。白细胞2.2×10~9～3.6×10~9/L者8例,正常4例。血小板7例均低于正常,13×10~9/L～92×10~9/L。网织红细胞增高者4
Aplastic anemia - paroxysmal nocturnal hemoglobinuria syndrome (AA-PNH syndrome), first reported by Letman in 1952, in recent years we encountered 7 cases, the report is as follows. Clinical data 1. Sex and age: 6 males and 1 females. The age of onset of 30 to 54 years, duration of 5 months to 8 years, an average of 2 years and 1 month. 2. Clinical manifestations: Most patients with anemia, fever 2 cases), anemia and hemorrhage (4 cases), mainly bleeding (1 case). 3 cases of scleral mild yellow dye, no lymphadenopathy, hepatomegaly in 3 cases, the largest ribs 2.5cm; splenomegaly in 3 cases, the largest lateral position 2.5cm. Of the 7 patients, 3 were hemoglobinuria (more than once a month), 1 was sporadic (once a few months), 8 were urine-colored and 8 were dark-brown. No fatal in 8 cases. 5. Laboratory tests: 6 cases of hemoglobin admission was significantly lower than normal, 60 ~ 90g / L; 1 case of normal. Leukocytes 2.2 × 10 ~ 9 ~ 3.6 × 10 ~ 9 / L in 8 cases, normal in 4 cases. Seven cases of platelet were lower than normal, 13 × 10 ~ 9 / L ~ 92 × 10 ~ 9 / L. Reticulocyte increased 4