HLH is a severe inflammatory syndrome characterized by primary or secondary immune dysregulation causing excess activation of macrophages and cytotoxic lymphocytes, leading to multi-system dysfunction. Diagnosing and managing HLH can be challenging for clinicians, with HLH-2004 criteria for diagnosis requiring a molecular diagnosis or the presence of at least five of the following: fever, splenomegaly, cytopenia involving two or more cell lineages, hypertriglyceridemia and/or hypofibrinogenemia, hemophagocytosis in the bone marrow, spleen or lymph nodes with no evidence of malignancy, low or no NK cell activity, elevated ferritin, or elevated soluble IL-2 receptor1. These criteria have been utilized to develop the HScore, a tool used to assist in determining the probability of HLH based on the aforementioned abnormalities2. After diagnosis, treatment typically includes chemotherapy and immunosuppression, followed by allogeneic bone marrow transplant3.
Manobianco, MD, Steven; Bradford, MD, William; Micaily, MD, Ida; and Binder, MD, Adam
"A Slow Burning Diagnosis: A Case Report of Hemophagocytic Lymphohistiocytosis Preceding the Diagnosis of Subcutaneous Panniculitis-Like T-Cell Lymphoma,"
The Medicine Forum: Vol. 22
, Article 14.
Available at: https://jdc.jefferson.edu/tmf/vol22/iss1/14