Occasionally a patient with Epstein-Barr Virus (EBV) infection will develop secondary hemophagocytic lymphohistiocytosis (HLH). This is considered a severe form of infectious mononucleosis and can be fatal.
Most cases occur during an initial infection with EBV. It may develop in patients with the X-linked lymphoproliferative disease (XLP).
Features:
(1) systemic symptoms which may be severe
(2) generalized lymphadenopathy
(3) hepatosplenomegaly
(4) elevated blood levels of cytokines (interferon-gamma, macrophage-colony stimulating factor, interleuken-12, other), which cause a proliferation of T cells and monocytes
(5) presence of hemophagocytic histiocytes in lymph nodes, spleen, liver and bone marrow
(6) evidence of EBV within T cells
(7) exclusion of other explanations for HLH
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