Abstract Details

Small lymphocytic lymphoma (SLL) is an insidious form of non-Hodgkin lymphoma. Like chronic lymphocytic leukemia (CLL), SLL is a proliferative disorder of monoclonal B cells. While SLL involves the lymphoid tissues, CLL involves the blood. SLL and CLL thus represent distinct products of the same underlying disease process. SLL in particular is an exceptionally rare clinical entity, and a paucity of epidemiological data on isolated SLL exists in the literature.

 A 70-year-old woman with a history of Stage IV CLL/SLL presented to an outside institution with four weeks of bilateral, ascending lower extremity paresthesias and proximal weakness, as well as a right oculomotor nerve palsy. Workup included a negative brain MRI and lumbar puncture (LP). On transfer to our institution, a repeat LP contained many nucleated cells and elevated protein. A lumbar spine MRI showed an enlarging marrow lesion of the L4 vertebral body which was subsequently biopsied, revealing a low-grade B-cell lymphoproliferative process. The right sural nerve was also biopsied, and demonstrated neurolymphomatosis with probable vasculitic neuropathy involving infiltration of the epineurium and perineurium. Hematology was consulted, and per the biopsy results, determined that lymphocytic infiltration was the cause of the neuropathy. A chemotherapeutic regimen of venetoclax and rituximab was initiated.   

This case describes the presentation and progression of a case of probable SLL vasculitic neuropathy, while illustrating the complex diagnostic methodology on both a clinical and histopathological basis. SLL alone is a rare clinical phenomenon, but SLL infiltrative neuropathy and vasculitis are exceedingly rare. In this case of SLL-associated probable vasculitic neuropathy with B-cell predominance, a chemotherapeutic regimen of venetoclax and rituximab was selected for anti-apoptotic and B-cell inhibitory properties, respectively. An important future consideration involves early identification of vascular involvement, which could guide the selection of therapies that specifically target vasculitic neuropathies, such as cyclophosphamide.