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Case Reports in Oncological Medicine
Volume 2017 (2017), Article ID 6428461, 5 pages
https://doi.org/10.1155/2017/6428461
Case Report

T Cell Histiocyte Rich Large B Cell Lymphoma Presenting as Hemophagocytic Lymphohistiocytosis: An Uncommon Presentation of a Rare Disease

1Department of Hematology/Oncology, Staten Island University Hospital, 475 Seaview Avenue, Staten Island, NY 10305, USA
2Department of Pulmonary/Critical Care, Staten Island University Hospital, 475 Seaview Avenue, Staten Island, NY 10305, USA
3Department of Pathology, Staten Island University Hospital, 475 Seaview Avenue, Staten Island, NY 10305, USA

Correspondence should be addressed to Uroosa Ibrahim

Received 11 April 2017; Accepted 19 July 2017; Published 21 August 2017

Academic Editor: Jeanine M. Buchanich

Copyright © 2017 Uroosa Ibrahim et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

Abstract

T cell histiocyte rich large B cell lymphoma (THRLBCL) is a rare subtype of non-Hodgkin’s lymphoma characterized by malignant B cells with reactive T lymphocytes. The pathophysiology is thought to involve cytokine-mediated evasion of T cell immune response by malignant B cells. It usually presents at an advanced stage with extranodal involvement. An extremely unusual manifestation of the disease is hemophagocytic lymphohistiocytosis (HLH) which is a hyperinflammatory disorder. We present a case of a 43-year-old male who presented with recurrent fever and recent radiologic imaging showing splenomegaly and right inguinal lymphadenopathy. On presentation, he had a fever of 105°F. Laboratory work-up was consistent with pancytopenia, elevated lactate dehydrogenase, elevated D-dimer, and a ferritin of 24,247 ng/mL. The patient was started on steroid therapy. An excisional biopsy of the right inguinal lymph node was consistent with a diagnosis of THRLBCL and the patient subsequently received six cycles of chemotherapy with R-CHOP (Rituximab, Cyclophosphamide, Doxorubicin, Vincristine, and Prednisone) after which a PET-CT scan showed no evidence of biologically active disease and ferritin was down to 822 ng/mL. We discuss the clinical manifestations and diagnostic and therapeutic considerations of this rare disease along with a review of reported cases in the literature.