Viral-associated hemophagocytic syndrome

✍ Scribed by D'Angio, Giulio J. ;Evans, Audrey E. ;Close, Pamelyn ;Friedman, David ;Uri, Antonia

• Publisher: John Wiley and Sons
• Year: 1990
• Tongue: English
• Weight: 447 KB
• Volume: 18
• Category: Article
• ISSN: 0098-1532
• DOI: 10.1002/mpo.2950180206

✦ Synopsis

J.C. is a 2-year-old boy referred for 5 weeks of unremitting daily fever as high as 41.5"C. His fevers were unresponsive to courses of intravenous cephalosporins and Bactrim, and the illness did not change after a course of intravenous gamma-globulin. He had two seizures associated with fever, and CSF was reportedly normal on two occasions. In the week prior to admission, he developed prominent bilateral cervical lymphadenopathy and pancytopenia, and he was referred here to rule out a malignant process. He was previously in good health and without the need for any medications. There was no history of unusual animal or travel exposures. There were no joint symptoms, but there was a history of a peeling rash on the hands and feet.

Physical examination on admission revealed an illappearing and lethargic child with a temperature of 40°C. There was bilateral anterior cervical lymphadenopathy with considerable soft tissue swelling around the nodes. The nodes themselves were soft, mobile and slightly tender, and the largest was 3 cm by 2 cm. Breath sounds were decreased at both bases; the liver was palpable 2 cm below the right costal margin, and the spleen was palpable 2 cm below the left. There were no other enlarged nodes, and there was no arthritis. A macular red rash was noted on the trunk and extremities. Neurologic examination was remarkable for lethargy and irritability, but was non-focal.

The initial laboratory findings were as follows: