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鉴于阵发性睡眠性血红蛋白尿(PNH)的临床表现、血液学改变、病情轻重的多样性,以及实验室检查在诊断中的作用,考虑如下。 甲、诊断条件 一、临床表现符合PNH。 二、实验室检查:酸化血清(Ham)试验 糖水试验 蛇毒因子溶血试验 尿潜血(或尿含铁血黄素) 符合下述任何一种,即可诊断: 1.二项以上阳性; 2.一项阳性,但须(1)二次以上阳性,或—次阳性,但即时重复,操作正规,有阴性对照,结果可靠。(2)有溶血的其他直接或间接证据,或有肯定的血红蛋白尿发作。(3)能除外其他溶血,特别是HS、AIHA、G6PD缺乏,冷性血红蛋白尿等。
Given the clinical manifestations of paroxysmal nocturnal hemoglobinuria (PNH), hematological changes, the severity of the disease, and the role of laboratory tests in the diagnosis, consider the following. A, the diagnosis of a clinical manifestations consistent with PNH. Second, the laboratory test: acidified serum test (Syrup test) snake venom factor hemolysis test Urine occult blood (or urinary hemosiderin) in line with any of the following, you can diagnose: 1. Two or more positive; 2. One Positive, but to be (1) more than two positive, or - sub-positive, but immediate, regular, negative control, the results are reliable. (2) other direct or indirect evidence of hemolysis, or definite hemoglobinuria. (3) except for other hemolysis, especially HS, AIHA, G6PD deficiency, cold hemoglobinuria and so on.