Gollub H, Machill K, Sander P
Pathologisches Institut, Bezirkskrankenhauses Schwerin.
Dtsch Med Wochenschr. 1989 Nov 3;114(44):1697-701. doi: 10.1055/s-2008-1066816.
A 53-year-old man fell ill with myalgia, an haemorrhagic diathesis, fever, renal failure and circulatory shock. Anaemia, thrombocytopenia and lymphadenopathy quickly developed, and he died after 13 days of bronchopneumonia in treatment-resistant cardiocirculatory failure. The underlying cause of the illness, in the two weeks during which he received intensive treatment with prolonged mechanical ventilation, haemodialysis, administration of clotting factors and blood derivatives as well as high doses of cortisone and antibiotics, remained uncertain. Histological examination of lymph-node biopsy and bone-marrow obtained at autopsy demonstrated massive increase in the number of benign haemophagocytic histiocytes, which--together with the clinical picture--gave the diagnosis of an infection-associated haemophagocytic syndrome. This is a rare disease and apparently the expression of an abnormal immune reaction with potentially reversible phagocytosis of the body's own blood cells after preceding infection, often a viral one.
一名53岁男性出现肌痛、出血素质、发热、肾衰竭和循环性休克。很快出现贫血、血小板减少和淋巴结病，在因难治性心循环衰竭接受支气管肺炎治疗13天后死亡。在他接受长时间机械通气、血液透析、凝血因子和血液制品输注以及大剂量皮质类固醇和抗生素强化治疗的两周时间里，疾病的根本原因仍不明确。尸检时获取的淋巴结活检和骨髓组织学检查显示良性噬血细胞组织细胞数量大量增加，结合临床表现，诊断为感染相关性噬血细胞综合征。这是一种罕见疾病，显然是先前感染（通常为病毒感染）后机体自身血细胞发生潜在可逆性吞噬的异常免疫反应的表现。
