Immunome Research
Open Access
ISSN: 1745-7580
ISSN: 1745-7580
Department of Clinical, Anaesthesiology and Cardiovascular Sciences, Sapienza University of Rome, Rome, Italy
Review Article
Hemophagocytic Lymphohistiocytosis and Macrophage Activation Syndrome: Review of the Literature
Author(s): Chiara Gioia*
HLH is a life-threatening condition characterized by the uncontrolled activation of cytotoxic T lymphocytes, NK cells, and macrophages, resulting in an overproduction of pro-inflammatory cytokines. A primary and a secondary form are distinguished. Clinical manifestations include fever, splenomegaly, neurological changes, coagulopathy, hepatic dysfunction, cytopenia, hypertriglyceridemia, hyperferritinemia, and hemophagocytosis. In adults, therapy, although aggressive, is often unsuccessful. We previously reported a case of HLH related to EBV-infection and hematologic malignancies in a Burkina Faso 41-year-old man, presented acute onset of fever, fatigue, and weight loss. EBV-DNA load of more than 90000 copies/mL was found. Bone marrow aspirate showed hemophagocytosis while biopsy revealed a marrow localization of peripheral T lymphoma. High doses of glucocorticoids were immediately ad.. View More»