The accelerated phase of Chediak-Higashi syndrome. An expression of the virus-associated hemophagocytic syndrome? NLM AIDSLINE Important note: Information in this article was accurate in 1985. The state of the art may have changed since the publication date.

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The accelerated phase of Chediak-Higashi syndrome. An expression of the virus-associated hemophagocytic syndrome?

Cancer. 1985 Aug 1;56(3):524-30. Unique Identifier : AIDSLINE MED/85227909
Rubin CM; Burke BA; McKenna RW; McClain KL; White JG; Nesbit ME Jr; Filipovich AH

Abstract: The clinical and pathologic findings of four patients with Chediak-Higashi syndrome in the accelerated phase were studied in order to clarify the nature of this enigmatic process. Fever, lymphadenopathy, hepatosplenomegaly, and cytopenias were present in every patient. All cases demonstrated extensive parenchymal infiltrates in many organs composed of benign-appearing histiocytes manifesting hemophagocytosis accompanied by lymphocytes and plasma cells. Studies in one patient suggested a viral etiology with the findings of a low blood lymphocyte OKT4 to OKT8 ratio, acquired loss of lymphocyte response to mitogens, the presence of Epstein-Barr virus genome in the mononuclear cells of lymph node, blood, and bone marrow, and possible clinical responses to acyclovir. It is concluded that the accelerated phase of Chediak-Higashi syndrome may be the clinicopathologic expression of the virus-associated hemophagocytic syndrome.
Keywords: Antibodies, Viral/ANALYSIS Biopsy Bone Marrow/PATHOLOGY Case Report Chediak-Higashi Syndrome/*IMMUNOLOGY/PATHOLOGY Female Herpesviridae Infections/*IMMUNOLOGY/PATHOLOGY Herpesvirus 4, Human/IMMUNOLOGY Histiocytes/IMMUNOLOGY/ULTRASTRUCTURE Human Immunoglobulins/METABOLISM Infant Killer Cells, Natural/IMMUNOLOGY Lymph Nodes/PATHOLOGY Lymphocyte Transformation *Phagocytosis Spleen/PATHOLOGY Support, Non-U.S. Gov't Support, U.S. Gov't, P.H.S. JOURNAL ARTICLE

KWDantibodies,viral/analysisbiopsybonemarrow/pathologycasereportchediak-higashisyndrome/KWDimmunology/pathologyfemaleherpesviridaeinfections/KWDimmunology/pathologyherpesvirus4,human/immunologyhistiocytes/immunology/ultrastructurehumanimmunoglobulins/metabolisminfantkillercells,natural/immunologylymphnodes/pathologylymphocytetransformationKWDphagocytosisspleen/pathologysupport,non-uKWDsKWDgov'tsupport,uKWDsKWDgov't,pKWDhKWDsKWDjournalarticle
851030
M85A0081

Copyright © 1985 - National Library of Medicine. Reproduced under license with the National Library of Medicine, Bethesda, MD.

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