Symptomatic central nervous system (CNS) involvement in chronic lymphocytic
leukemia (CLL) or its variants is rare. We report two cases of CLL with le
ptomeningeal involvement. Patient one was an 81-year-old male who had CLL s
tage C (IV) at diagnosis and developed meningeal disease 29 months later. P
atient 2 was a 42-year-old male with a diagnosis of CLL stage ii (II) that
evolved into mixed-cell CLL/prolymphocytic leukemia (PLL) 1.5 years later,
with leptomeningeal infiltration of prolymphocytes developing 26 months aft
er initial diagnosis. Meningeal leukemia was diagnosed by cerebrospinal flu
id examination, with flow cytometry showing the same immunophenotypic findi
ngs of lambda-light chain restriction as the lymphocytes in bone marrow in
one patient, and with morphologic characteristics exhibiting exclusively pr
olymphocytes in the other patient. The CNS disease of both patients respond
ed effectively to intrathecal chemotherapy and cranial irradiation. However
, both patients died of infection, a major cause of morbidity and mortality
in patients with CLL. The clinicopathologic features of these two patients
indicate that, despite the rarity of CNS involvement in CLL patients, any
neurologic manifestation in CLL patients should arouse suspicion of meninge
al leukemia and patients should be examined and managed accordingly.