Virus-associated hemophagocytic syndromeA benign histiocytic proliferation distinct from malignant histiocytosis

Abstract

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.

Histiocytic sarcoma: a study of five cases including the histiocyte marker CD163

“…2,13,17,20,28,41,[50][51][52][53] Malignant cytologic features with evidence of tissue invasion or destruction were constant findings in this study and are necessarily present in order to distinguish from benign histiocytic proliferations. [18][19][20] Hemophagocytosis, a feature previously felt to be important in the diagnosis, may be present but is nonspecific 29,54,55 and frequently is not prominent in bona fide cases. 1,17,19,20,41,51 Likewise, this feature was inconspicuous in all but one of our cases.…”

“…Since these original papers, increasing emphasis has been placed on cytochemical and immunohistochemical characterization of these neoplasms in an attempt to establish true histiocytic lineage. [12][13][14][15][16][17] This diagnostic challenge is further complicated as it is necessary to distinguish HS from other histiocytic processes, both benign and malignant, to include the hemophagocytic syndrome, [18][19][20] malignant histiocytosis, 10,[18][19][20][21] and monocytic leukemia. 17,19 The diagnosis of HS relies predominantly on the verification of histiocytic lineage and the exclusion of other, poorly differentiated, large cell malignancies (ie lymphoma, carcinoma, melanoma) by way of extensive immunophenotypic investigation.…”

Síndrome hemofagocítico asociado con dengue hemorrágico

“…El síndrome hemofagocítico secundario ha sido informado asociado con infecciones virales principalmente por virus Epstein Barr (12-16), aunque también hay informes con herpes simple (15,17), virus de la hepatitis B y C (24) y VIH (25); se ha informado en pocas oportunidades asociado con virus del dengue (3,4).…”

Hemophagocytic Syndrome in a Patient with Adult-onset Still’s Disease

“…[7] Risdall et al [8] first described a group of patients with HS. It's a clinicopathological entity that has clinical diagnostic criteria such as fever (>39 °C), hepatosplenomegaly pancytopenia, raised levels of liver enzymes, serum ferritin, triglycerides and hemophagocytosis.…”