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

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.

T-Cell/Histiocyte-Rich Large B-Cell Lymphoma: Report of the First Case in the Mandible

“…In the present case, malignant B-cells were positive for CD20 and Bcl-2 and negative for CD30, and the reactive background infiltrate was positive for CD5, CD45RO and CD68. This corroborates the immunohistochemical findings reported in the literature for THRLBCL in other locations [8,[21][22][23][24][25][26][27]. The immunohistochemistry findings in the present case were fundamental to the direction of the final diagnosis of this rare variant of DLBCL localized in the jaw, owing to the similarity of the clinical and radiographic characteristics of this case with those of other typical cases of DLBCL described in the literature [13,16].…”

L-LOP/LOPP for the treatment of canine gastrointestinal/hepatosplenic lymphoma

“…Although there are only scarce reports of HS lymphoma in dogs it is likely to arise from splenic cytotoxic γδ-T-cells, which represents a specific syndrome in people known as hepatosplenic gamma-delta T-cell lymphoma ( 4 , 8 , 9 ). Nevertheless, primary or secondary HS large B-cell lymphomas are also reported in humans and may also occur in dogs ( 24 , 25 ). Lack of immunolabeling for both T- and B-cell markers may also occur as a result of loss of T/B-cell antigen receptor complex expression ( 4 ).…”

Unique presentation of T-cell/histiocyte-rich large B cell lymphoma complicated with hemophagocytic lymphohistiocytosis: Case report and review of the literature

“…To the best of our knowledge, eight cases of HLH secondary to TCHRLBCL were described in the English literature (19)(20)(21)(22)(23)(24)(25)(26). Their summary is displayed in Table II.…”