Hemophagocytic lymphohistiocytosis secondary to T-cell/histiocyte-rich large B-cell lymphoma

Katherine Devitt; Jan Cerny; Bradley Switzer; Muthalagu Ramanathan; Rajneesh Nath; Hongbo Yu; Bruce A Woda; Benjamin J Chen

doi:10.1016/j.lrr.2014.05.004

Hemophagocytic lymphohistiocytosis secondary to T-cell/histiocyte-rich large B-cell lymphoma

Leuk Res Rep. 2014 Jun 2;3(2):42-5. doi: 10.1016/j.lrr.2014.05.004. eCollection 2014.

Authors

Katherine Devitt¹, Jan Cerny², Bradley Switzer², Muthalagu Ramanathan², Rajneesh Nath², Hongbo Yu¹, Bruce A Woda¹, Benjamin J Chen¹

Affiliations

¹ Department of Pathology UMass Memorial Medical Center and University of Massachusetts Medical School, 1 Innovation Drive, Biotech 3, Worcester, MA 01605, USA.
² Department of Hematology/Oncology, UMass Memorial Medical Center and University of Massachusetts Medical School, Worcester, MA 01605, USA.

Abstract

Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening clinical syndrome characterized by dysregulation of the immune system. Impaired function of cytotoxic T cells and natural killer cells is often seen, and T-cell malignancies represent most cases of lymphoma-associated HLH. HLH associated with B-cell lymphoma is rare. We describe a case of a 30-year-old man who presented with fever, splenomegaly, and hyperferritinemia. Bone marrow biopsy revealed T-cell/histiocyte-rich large B-cell lymphoma, a rare, aggressive B-cell malignancy. This case highlights the interplay between a pro-inflammatory cytokine microenvironment and tumor-mediated immune suppression, and addresses the importance of accurately diagnosing these entities for appropriate clinical management.

Keywords: Chemotherapy; Hemophagocytic lymphohistiocytosis (HLH); Stem cell transplant; T-cell/histiocyte rich large B-cell lymphoma (THRLBCL).