Leptomeningeal disease in chronic lymphocytic leukemia

Abstract

Chronic lymphocytic leukemia (CLL) is the most common lymphoproliferative disorder in the western hemisphere, with an annual incidence of 3:100 000. Commonly patients are asymptomatic but not rarely disease progression occurs in the setting of lymphadenopathy and extensive leukemic burden. Leptomeningeal involvement in patients with CLL is infrequent, with presenting symptoms of headache (23%), acute or chronic changes in mental status (28%), cranial nerve abnormalities (54%) including optic neuropathy (28%), weakness of lower extremities (23%) and cerebellar signs (18%). In this report, we discuss a CLL patient with leptomeningeal involvement, who presented with neurological symptoms as the first clinical sign, and a diagnosis of leptomeningeal was made based on CSF cytology and flow cytometry. Treatment consisted of radiation therapy and intrathecal chemotherapy with arabinoside–cytosine and systemic chemotherapy. On the basis of this patient-report together with 37 other previously reported cases, the clinical characteristics together with treatment options and outcome of leptomeningeal involvement in CLL are reviewed. Our case together with data from the literature indicate that a timely diagnosis and intensive treatment of leptomeningeal disease of CLL may lead to longstanding and complete resolution of neurological symptoms.

Introduction

Chronic lymphocytic leukemia (CLL) is the most common lymphoproliferative disorder in the western world, with an annual frequency of 3: 100 000. At present, the median age is 65–70 years, males being affected twice as much as females [1]. The most frequent complication of CLL is an opportunistic infection, often in the lung parenchyma [2]. Leptomeningeal disease in CLL is distinctly rare. In the past three decades, 37 cases have been reported, while leptomeningeal involvement as the first clinical sign of CLL is even more sporadic. We encountered a patient with leptomeningeal involvement as the first clinical sign of CLL. Her history is described together with a review of the literature focussing on the diagnosis and treatment of leptomeningeal CLL.