Myelomatous Meningitis: a Report of Six Cases from a Single Centre
摘要
Myelomatous meningitis is a rare extramedullary manifestation of plasma cell dyscrasias, with an estimated incidence of less than 1%. The hallmark of this condition is the involvement of cerebrospinal fluid (CSF) with myeloma cells. This is a retrospective study of six patients diagnosed with myelomatous meningitis at a tertiary cancer care centre in India. This case series included patients having CSF cytology-positive results. Six patients were diagnosed with myelomatous meningitis, accounting for 0.18% of all multiple myeloma cases. The median age was 44.5 years (range: 35 to 62 years), with 4 males and 2 females. Five patients had multiple myeloma, and one patient had plasma cell leukemia at the time of diagnosis. Two patients presented with myelomatous meningitis at initial diagnosis, while four developed it at relapse. The median time from initial diagnosis to the development of myelomatous meningitis was 9 months. The most common central nervous system symptoms were cranial nerve palsy in 4 patients, headache in 3, encephalopathy in 3, and seizures in 2 patients. Imaging with either computed tomography or magnetic resonance imaging revealed leptomeningeal enhancement or deposits in 3 patients. Among the two patients with myelomatous meningitis at initial presentation, one with plasma cell leukemia received VTD-PACE chemotherapy, while the other was treated with high-dose steroids. Of the four patients who developed myelomatous meningitis at relapse, two patients received high-dose steroids, two received triple intrathecal chemotherapy, and one received whole-brain radiation followed by the KRd regimen. The median survival after the development of myelomatous meningitis was 3 months. Myelomatous meningitis is a rare, aggressive extramedullary manifestation of plasma cell dyscrasias, associated with a dismal prognosis.