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血小板无力症(Thrombasthenia)系常染色体隐性遗传性疾病,是罕见的先天性出血性疾病之一,1918年Glanzmann 首先报道,至1969年国外共报道130例,国内上海广慈医院诊断8例。本院借助血小板聚集仪诊断一例,报道如下:患者苏某,2岁半。3月龄起皮肤出现瘀点、瘀斑,近一年来时有鼻衄,量较多。曾因皮肤外伤、舌咬破后出血难止,经输血多次才缓解。家族成员中无同样病史。体检:面色轻度苍白、四肢、躯干皮肤可见较密紫红色出血点,心肺无阳性体征,肝肋下2cm,脾未及,淋巴结无特殊肿
Thrombasthenia is an autosomal recessive disease, which is one of the rare congenital hemorrhagic diseases. First reported by Glanzmann in 1918, 130 cases were reported abroad in 1969 and 8 cases were diagnosed in Shanghai Guangci Hospital. Our hospital diagnosed with platelet aggregation instrument in one case, reported as follows: patients Sumou, 2 years and a half. 3-month-old skin petechia, ecchymosis, epistaxis nearly a year, the amount of more. Once due to skin trauma, bleeding after bleeding tongue difficult to stop, after repeated transfusion only ease. No similar family history. Physical examination: pale pale, limbs, trunk skin can be seen more dense purplish red bleeding, no positive signs of cardiopulmonary, liver ribs 2cm, spleen and lymph nodes without special swelling