Leptomeningeal disease as a manifestation of CLL.

Abstract

e17019 Background: Chronic Lymphocytic Leukemia is a lymphoproliferative disorder which can have variable course from being indolent to aggressive. Reported annual incidence of Chronic Lymphocytic Leukemia is 3:100000. The most common manifestation is Lymphadenopathy and Leptomeningeal involvement is very rare with only a few reported cases in literature. Methods: This is an observational case report. Results: We report a case of 57 year old White male who presented with CNS disease as his first manifestation of CLL. He reported a 6 week history of cognitive decline with inability to perform complex tasks, difficulty with calculations, word finding trouble, progressive clumsiness of right arm and leg and a weight loss. MRi revealed Left sided Leptomeningeal enhancement. As part of his work up; patient received LP, PET scans, Infectious and autoimmune studies and eventually brain biopsy which initially showed Lymphocytic predominance.On further review by NIH immunohistochemical stains for CD5, CD20 and CD23 were positive on the leptomeningeal and parenchymal lymphocytes. His bone marrow showed CLL involvement both morphologically and immunophenotypically by means of flow cytometric analysis. He was started on treatment with High dose methotrexate and Rituximab and showed remarkable improvement in his cognitive function by the completion of his second cycle. His bone marrow repeat biopsy showed complete remission and no involvement of lymphoma. Conclusions: CLL is an indolent disease and direct CNS invasion is extremely rare. Our case becomes unique in terms of its presentation. It highlights the importance of timely diagnosis and management of leptomeningeal disease in CLL can lead to resolution of neurologic symptoms.