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Virus‐associated hemophagocytic syndrome A benign histiocytic proliferation distinct from malignant histiocytosis
Author(s) -
Risdall Robert J.,
McKenna Robert W.,
Nesbit Mark E.,
Krivit William,
Balfour Henry H.,
Simmons Richard L.,
Brunning Richard D.
Publication year - 1979
Publication title -
cancer
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 3.052
H-Index - 304
eISSN - 1097-0142
pISSN - 0008-543X
DOI - 10.1002/1097-0142(197909)44:3<993::aid-cncr2820440329>3.0.co;2-5
Subject(s) - hemophagocytosis , malignant histiocytosis , medicine , histiocyte , hepatosplenomegaly , pathology , histiocytosis , bone marrow , pancytopenia , disease
Nineteen patients whose bone marrow smears showed histiocytic hyperplasia with prominent hemophagocytosis were found to have a clinicopathologic syndrome associated with active viral infection. High fever, constitutional symptoms, liver function, and coagulation abnormalities and peripheral blood cytopenias were characteristic findings. Hepatosplenomegaly, lymphadenopathy, bilateral pulmonary infiltrates, and skin rash were often present. Fourteen of the patients were immunosuppressed. Active infection by herpes group viruses was documented in 14 patients and by adenovirus in 1. The bone marrow of most patients also showed decreased granulopoiesis and erythropoiesis with normal to increased numbers of megakaryocytes. Treatment generally consisted of supportive therapy and withdrawal of immunosuppressive drugs. Thirteen patients recovered. Lymph node biopsy and autopsy material showed generalized histiocytic hyperplasia with hemophagocytosis. The relationship of this disorder to familial hemophagocytic reticulosis, familial erythrophagocytic lymphohistiocytosis, histiocytic medullary reticulosis, and malignant histiocytosis is discussed. Immunosuppressive and cytotoxic therapy may be contraindicated in the treatment of this virus‐associated syndrome.