Blood, Vol. 93 No. 6 (March 15), 1999:
pp. 1991-1991
PHOTOGRAPH
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Virus-associated hemophagocytic syndrome. A healthy 18-year-old
senior high school student presented with several weeks of myalgias,
fevers, and sore throat. She subsequently developed pancytopenia and
hepatic dysfunction. A thorough evaluation was unrevealing except for a
positive monospot and Epstein-Barr virus (EBV) VCA-IgG with a negative
EBV VCA-IgG and EBNA (both became positive several weeks later). A
diagnosis of acute EBV infection was made. Over the next 3 weeks she
had a waxing and waning course but finally required hospital admission
due to worsening pancytopenia (WBC, 300/µL; hematocrit, 16.2%; and
platelet count, 44,000/µL) and hepatic dysfunction (ALT, 636 U/L;
AST, 608 U/L; LDH, 2,788 U/L; direct bilirubin, 32.2 mg/dL; and
prothrombin time, 16 seconds). Her physical exam was notable for a
fever (39.5°C), a diffuse fine scaly rash, severe icterus, no
adenopathy, a liver span of 14 cm in the midclavicular line, and a
spleen tip palpable 2 cm below the costal margin. Biopsy of her bone
marrow showed overall hypocellularity with multiple nests of RBCs
(arrows in figure on left; original magnification ×100). These nests
of RBCs, as seen in the bone marrow aspirate (figure on right; original
magnification ×2,500), represent histiocytes with engulfed
erythrocytes characteristic of hemophagocytosis. Despite intense
immunosuppressive therapy (including steroids, cyclosporin A, IVIG, and
ATG), she developed respiratory failure, gastrointestinal bleeding,
renal failure (requiring dialysis), cardiac dysfunction (with global
hypokinesis), and pancreatitis. She finally succumbed to an
intracranial hemorrhage. Virus-associated hemophagocytic syndrome is
typically associated with acute EBV infection. It is characterized by
constitutional symptoms, including fever, rash, lymphadenopathy, and
hepatosplenomegaly. Therapy is directed at the immune dysregulation and
is often ineffective, as in this unfortunate case. (Courtesy of Lindsey
Baden, MD, and Frank Evangelista, MD, Beth Israel Deaconess Medical
Center, Departments of Medicine and Pathology, 330 Brookline Ave,
SL-435, Boston, MA 02215.)
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