A Slow Burning Diagnosis: A Case Report of Hemophagocytic Lymphohistiocytosis Preceding the Diagnosis of Subcutaneous Panniculitis-Like T-Cell Lymphoma

Authors

Steven Manobianco, MD, Thomas Jefferson UniversityFollow
William Bradford, MD, Thomas Jefferson UniversityFollow
Ida Micaily, MD, Thomas Jefferson UniversityFollow
Adam Binder, MD, Thomas Jefferson UniversityFollow

Abstract

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.

Recommended Citation

Manobianco, MD, Steven; Bradford, MD, William; Micaily, MD, Ida; and Binder, MD, Adam (2021) "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.
DOI: https://doi.org/10.29046/TMF.022.1.013
Available at: https://jdc.jefferson.edu/tmf/vol22/iss1/14