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女性,12岁。因反复四肢末梢紫绀5天,于1992年2月24日入院。患儿入院前5天室外遇冷后出现两下肢末端发绀、发凉,持续1～2小时,当日两上肢末端、面颊、鼻尖、耳轮也出现类似发绀现象,发绀在肘、膝关节以下,尤以指趾发绀明显,伴局部麻木,无疼痛,紫绀发作前皮肤无苍白。每日发绀发作2～3次,寒冷时明显,经保暖后好转。病初小便呈浓茶色。既往无心肺病史及类似发作史。体检:神志清,无气促、四肢末端发绀,局部皮肤发冷,心肺听诊正常,腹软,肝脾未及。握拳试验(-),冷水试验(+)。实验室检查:血Hb90g/L,WBC10×10~9/L N0.75、L0.25,血小板数105×10~9/L,ESR40mm/h,尿液分析pH5.5,隐血(+),冷凝集素试验1∶128.诊断:冷凝集素病。给予保暖,静滴丹参、Vit C等治疗,1周后紫绀发作缓解,出院后随访1年,病情无反复。 讨论冷凝集素病也称冷凝集素综合
Female, 12 years old. Due to repeated limbs cyanosis 5 days, in February 24, 1992 admission. 5 days before admission to children with cold outside the case after the end of the two lower extremities cyanosis, cold, lasted 1 to 2 hours the same day the two upper extremities, cheeks, nose, the helix also appeared similar to cyanosis, cyanosis in the elbow, below the knee, To finger to cyanosis was obvious, with local numbness, no pain, pale skin before the onset of cyanosis. Daily onset of cyanosis 2 to 3 times, obviously when cold, after warming up and improved. Early childhood urine was dark brown. Past history of heart and lung disease and similar episodes. Physical examination: conscious, no gas, end of the extremities cyanosis, local skin chills, cardiopulmonary auscultation normal, abdominal soft, liver and spleen not yet. Fist test (-), cold water test (+). Laboratory tests: blood Hb90g / L, WBC10 × 10 ~ 9 / L N0.75, L0.25, platelet count 105 × 10 ~ 9 / L, ESR40mm / h, urine analysis of pH5.5, occult blood (+), Cold agglutinin test 1:128.Diagnosis: cold agglutinin disease. Give warm, intravenous Salvia, Vit C and other treatment, cyanosis onset after 1 week to ease, follow-up after discharge for 1 year, the disease was repeated. Cold agglutinin disease is also called cold agglutinin synthesis