Hemophagocytic syndrome and neurological involvement in a case of intravascular large B-cell lymphoma

Abstract

Intravascular large B-cell lymphoma (IVLBCL) is a rare form of non-Hodgkin’s lymphoma, and is divided into Western and Asian variants. The latter is rarely found to have neurological system involvement. In China, there have only been a few diagnosed cases of IVLBCL. Here, we present a Chinese case of Asian-variant IVLBCL with neurological symptoms. A 32-year-old Chinese man presented with bilateral lower limb numbness and persistent fever. He also complained of difficulties in urination and defecation. In addition, splenomegaly and pancytopenia were observed. We identified 3% dysplastic lymphocytes in his peripheral blood film, and his bone marrow biopsy led to a diagnosis of Asian-variant IVLBCL. Lumbar spine magnetic resonance imaging, which revealed an edematous spinal cord, further confirmed neurological involvement. The patient refused treatment from the time of diagnosis, and died 2 months after being discharged. IVLBCL is a highly aggressive but nonspecific clinical manifestation that is difficult to diagnose; therefore, a greater understanding of the disease is needed. The current first-line therapy involves R-CHOP combination therapy (cyclophosphamide, doxorubicin, vincristine, prednisone, and rituximab); however, the overall prognosis of IVLBCL remains poor.