T Cell Histiocyte Rich Large B Cell Lymphoma Presenting as Hemophagocytic Lymphohistiocytosis: An Uncommon Presentation of a Rare Disease. (21st August 2017)

• Record Type: Journal Article
• Authors: Ibrahim, Uroosa; Garcia, Gwenalyn; Saqib, Amina; Hussein, Shafinaz; Dai, Qun
• Other Names: Buchanich Jeanine M. Academic Editor.
• Is Part Of: Case reports in oncological medicine. Volume 2017(2017)
• Publication Date: 2017-08-21
• Subjects: Oncology -- Periodicals; Tumors -- Periodicals; Oncology -- Case studies -- Periodicals; Neoplasms; Oncology; Tumors; Electronic journals; Periodicals; Case studies; Periodicals; Case Reports; Fulltext; Internet Resources; Periodicals; 616.994
• DOI: 10.1155/2017/6428461
• Languages: English
• ISSNs: 2090-6706

Abstract

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.