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[一例因严重贫血发现的老年阵发性冷性血红蛋白尿(PCH)病例]

[A case of paroxysmal cold hemoglobinuria (PCH) in the elderly found by severe anemia].

作者信息

Saito M, Endo O, Okazaki H, Inoue S, Takamura Y, Watanabe S

机构信息

3rd Department of Internal Medicine, Yokohama City University.

出版信息

Rinsho Byori. 1991 Jun;39(6):671-4.

PMID:1880946
Abstract

69 year-old female. Two weeks before the admission, the patient had suffered from upper respiratory tract infection. Severe anemia was detected (Hb was 6.0 mg/dl) and she received a transfusion. Four days after the transfusion, she became icteric and was transferred to our hospital on Feb. 3, 1990 for evaluation of anemia. Laboratory data on admission are as follows; Hb 6.3 g/dl, reticulocytes 128%, total bilirubin 4.6 mg/dl, indirect bilirubin 3.4 mg/dl, urobilinogen in urine positive, WR negative, direct anti-globulin test positive, indirect of the test negative, and Donath-Landsteiner test positive (both direct and indirect). Her red blood cells were classified as P2-positive. From these findings, non-syphilitic PCH was diagnosed. She required bed rest and therapy to protect her from cold exposure. The hemoglobin recovered to 8.3 g/dl. The D-L test was positive for 9 months. This is a rare case of PCH because the patient fell ill in old age and PCH was found because of her severe anemia in spite of her unawareness of hemoglobinuria.

摘要

69岁女性。入院前两周,患者患上呼吸道感染。检测发现严重贫血(血红蛋白为6.0mg/dl),她接受了输血治疗。输血后四天,她出现黄疸,并于1990年2月3日被转至我院评估贫血情况。入院时实验室检查数据如下:血红蛋白6.3g/dl,网织红细胞128%,总胆红素4.6mg/dl,间接胆红素3.4mg/dl,尿胆原阳性,梅毒血清试验阴性,直接抗球蛋白试验阳性,间接抗球蛋白试验阴性,唐纳-兰斯泰纳试验阳性(直接和间接均阳性)。她的红细胞分类为P2阳性。根据这些发现,诊断为非梅毒型阵发性寒冷性血红蛋白尿症。她需要卧床休息并接受治疗以避免受寒。血红蛋白恢复至8.3g/dl。唐纳-兰斯泰纳试验在9个月内一直呈阳性。这是一例罕见的阵发性寒冷性血红蛋白尿症病例,因为患者发病时年龄较大,且尽管她未意识到血红蛋白尿,但因严重贫血而发现了阵发性寒冷性血红蛋白尿症。

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