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June 1991

Ophthalmic Manifestations of Virus-Associated Hemophagocytic Syndrome

Author Affiliations

New Haven, Conn

Arch Ophthalmol. 1991;109(6):777. doi:10.1001/archopht.1991.01080060033015

Virus-associated hemophagocytic syndrome (VAHS) is characterized by high fever, malaise, hepatosplenomegaly, and pancytopenia. Patients usually have a viral-like illness prior to its onset. The diagnosis is based on a bone marrow biopsy specimen that demonstrates histiocytic hyperplasia with prominent hemophagocytosis of red blood cells, platelets, and nucleated hematopoietic cells. Hypocellularity of the bone marrow is frequently seen. This syndrome is distinct from malignant histiocytosis, in which the histiocytes show neoplastic proliferation.1 This is the first report, to our knowledge, of the ophthalmic manifestations of VAHS.

Report of a Case.  —A 14-year-old, previously healthy boy presented with a 3-week history of increasing fatigue, fever, chills, and dry cough. On admission, his temperature was 41°C and throat, blood, and urine cultures were negative for bacteria. A chest roentgenogram was equivocal. Laboratory values were as follows: hemoglobin, 43 g/L; hematocrit, 0.126; white blood cell count, 2.4×109/L; and platelets, 97 ×

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