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Genetic and Rare Diseases Information Center (GARD)

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Virus associated hemophagocytic syndrome


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Overview


Virus associated hemophagocytic syndrome is a very serious complication of a viral infection. Signs and symptoms of virus associated hemophagocytic syndrome, include high fever, liver problems, enlarged liver and spleen, coagulation factor abnormalities, decreased red or white blood cells and platelets (pancytopenia), and a build-up of histiocytes, a type of immune cell, in various tissues in the body resulting in the destruction of blood-producing cells (histiocytic proliferation with prominent hemophagocytosis).[1] 

Diagnosis is based upon the signs and symptoms of the patient. The cause of the condition is not known. Treatment is challenging and approach will vary depending on the age and medical history of the patient. Complications of this syndrome can become life threatening. Related conditions (conditions with overlapping signs and symptoms), include histiocytic medullary reticulosis (HMR), familial hemophagocytic lymphohistiocytosis (FHL), and X-linked lymphoproliferative syndrome.[1]
Last updated: 5/8/2013

References

  1. Kikuta H et al.,. Fatal Epstein-Barr virus-associated hemophagocytic syndrome. . Blood. 1993;82:3259-3264; http://bloodjournal.hematologylibrary.org/content/82/11/3259.full.pdf. Accessed 5/8/2013.
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