Infektassoziiertes hämophagozytisches Syndrom mit letalem Verlauf

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Abstract
A 53-year-old man fell ill with myalgia, and 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 preceeding infection, often a viral one.