Secondary Hemophagocytic Lymphohistiocytosis (SHLH) in the Context of Newly Diagnosed Philadelphia Positive, CD 20+ B-Cell Acute Lymphoblastic Lymphoma (B-ALL): A Case Report and Brief Review of Literature

Hemophagocytic Lymphohistiocytosis (HLH) is a clinical syndrome that develops due to the unregulated activation of the inflammatory system and is considered when a patient presents with a constellation of symptoms and objective findings consisting of fevers, splenomegaly, hepatomegaly, cytopenias, hepatitis, hypertriglyceridemia and/or hypofibrinogenemia, hyperferritinemia, biopsies of the bone marrow, spleen or lymph node demonstrating hemophagocytosis, and elevated soluble CD25 receptor levels. This clinical diagnosis requires a high index of suspicion as well as prompt initiation of therapy. HLH can be classified as primary or secondary. Primary HLH can be categorized into familial HLH due to specific gene mutations or hereditary immune deficiencies. Secondary HLH is often reactive due to malignancy, infection, autoimmunity or suppression of the immune system. We report a case of a 57 year old African American female who presents with secondary HLH in the context of newly diagnosed acute leukemia, specifically Philadelphia positive, CD20 positive, B-ALL. This case highlights the importance of recognizing the rare association of secondary HLH as a presenting feature in a patient with newly diagnosed acute leukemia and further reiterates the need to immediately begin appropriate targeted therapy concurrently for HLH and the underlying malignancy.