Hemophagocytic Syndromes and Infection

, Beth Israel Deaconess Medical Center, Boston, Massachusetts, USA


Emerging Infectious Diseases. 2000;6(6) 

In This Article


HLH and related hemophagocytic syndromes are uncommon but severe illnesses associated with a variety of infectious agents, as well as genetic, neoplastic, and autoimmune diseases. HLH in the context of infection is best described as part of a spectrum of EBV-associated illness resulting in clonal proliferation of T-lymphocytes, with excessive activation of macrophages. This syndrome may be difficult to distinguish from T-cell lymphoma and should be treated aggressively with etoposide-based chemotherapeutic regimens.

Hemophagocytic syndromes associated with other infectious illnesses, including sepsis, typhoid fever, tuberculosis, and leishmaniasis, may resolve with treatment of the underlying infection, and their recognition is important as they may mimic malignant disease. Further study of these reactive hemophagocytic syndromes may yield important insights into the biology of macrophage activation.


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