Diffuse large b-cell lymphoma with hemophagocytic syndrome in an HIV+ patient treated with CCR5- bone marrow transplant

Abstract

Hemophagocytic lymphohistiocytosis (HLH) is a life threatening systemic disease characterized by proliferation of activated lymphocytes/macrophages leading to an exaggerated but ineffective immune response. HLH clinically presents with fever, pancytopenia, splenomegaly and hemophagocytosis in the bone marrow, lymph nodes or liver. HLH has been associated with viral, bacterial, fungal, parasitic infections, and malignancies, particularly T-cell lymphomas, and collagen-associated vascular diseases. We describe a case of an HIV positive patient who presented to the emergency department with fever, cough, diarrhea and abdominal pain. A CT scan revealed diffuse lymphadenopathy. Excisional biopsy of the lymph node showed almost complete effacement of the normal lymph node architecture. The effaced areas were composed of small lymphocytes, medium size cells with features of centroblasts, several immunoblasts and ReedSternberg like cells. Increased number of histiocytes with hemophagocytosis was also observed. Diagnosis of EBV positive diffuse large B cell lymphoma was made. In addition, clinical presentation, laboratory results and morphology led to the diagnosis of HLH. This is a unique case describing a well-controlled HIV positive patient who developed HLH triggered by reactivation of EBV with diffuse large B cell lymphoma. The patient underwent allogeneic homozygous CCR5-transplant with a possibility of curing his large B cell lymphoma and HIV. He is currently under surveillance for his HIV status and lymphoma.