Hansen U, Wiese R, Knolle J
Medizinische Klinik, Diakonissenkrankenhaus Flensburg.
Dtsch Med Wochenschr. 1994 Sep 16;119(37):1231-4. doi: 10.1055/s-2008-1058826.
A 41-year-old man was admitted in circulatory shock of unknown aetiology (systolic pressure 50 mm Hg) and marked reddening of the upper part of the body as well as maculopapular rash over the whole body. After 1500 ml of colloidal solution had been infused the blood pressure rose to a level at which the patient's condition was no longer at risk. He reported having had a similar attack of flushing and circulatory collapse during the last few years, each time after drinking 3-41 of beer. Laboratory tests showed thromboplastin time 56%, partial thromboplastin time 130 s and thrombin time > 180 s. Three hours after admission the coagulation times had further deteriorated, but had become normal within 20 hours. After rest and after a provocation (hot bath) the serum concentrations were: heparin 0.21 U/ml and 0.85 U/ml; histamine 1.9 micrograms/ml and 2.3 micrograms/ml; serotonin 23 micrograms/ml and 38 micrograms/ml. Histological examination of an iliac crest bone marrow biopsy revealed dense collections of mast cells, as seen in systemic mastocytosis. A skin biopsy was diagnostic of urticaria pigmentosa.
一名41岁男性因病因不明的循环性休克（收缩压50mmHg）入院，其身体上部明显发红，全身出现斑丘疹。输注1500ml胶体溶液后，血压升至患者病情不再危险的水平。他报告在过去几年中每次饮用3 - 4升啤酒后都会出现类似的潮红和循环性虚脱发作。实验室检查显示凝血酶原时间为56%，部分凝血活酶时间为130秒，凝血酶时间>180秒。入院3小时后凝血时间进一步恶化，但在20小时内恢复正常。休息及激发试验（热水浴）后，血清浓度分别为：肝素0.21U/ml和0.85U/ml；组胺1.9μg/ml和2.3μg/ml；5-羟色胺23μg/ml和38μg/ml。髂嵴骨髓活检的组织学检查显示有密集的肥大细胞聚集，这在系统性肥大细胞增多症中可见。皮肤活检诊断为色素性荨麻疹。
