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

Abstract EN

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.