Myeloma of the central nervous system: association with high‐risk chromosomal abnormalities, plasmablastic morphology and extramedullary manifestations

Abstract

Summary. Involvement of the central nervous system (CNS) by multiple myeloma, as defined by the detection of malignant plasma cells in the cerebrospinal fluid in the presence of suggestive symptoms, is considered extremely rare. We report on the characteristics of 18 such patients diagnosed and treated at the University of Arkansas over the last 10 years for an overall incidence of approximately 1%. Their evaluation revealed association of CNS involvement with unfavourable cytogenetic abnormalities (especially translocations and deletion of the chromosome 13), high tumour mass, plasmablastic morphology, additional extramedullary myeloma manifestations and circulating plasma cells. The presence of these features should alert clinicians to the possibility of CNS involvement. The outcome of these patients was extremely poor despite the use of aggressive local and systemic treatment including autologous stem cell transplants. Given this universally poor prognosis, the application of allogeneic transplants should be studied in this clinical setting.

Effective treatment of leptomeningeal multiple myeloma with total craniospinal irradiation supported by second allogeneic donor stem cell infusion

“…The small number of cases reported generally occurred in the setting of pre-existing stage III MM 2-4 associated with an elevated LDH level, 5,6 unfavourable cytogenetic abnormalities 6 and plasma cell leukemia. 4,6 Not surprisingly, the prognosis is extremely poor. Based on previously reported cases and accepting the bias for reporting only responding patients, Peterson et al 7 estimated the median overall survival to be only 1.5 months from the time of diagnosis of meningeal involvement.…”

“…[2][3][4][5][6][7][8] Often, transient neurological improvement is seen, but progression and death inevitably follow shortly after.…”

Intracranial plasmacytoma as an initial presentation of multiple myeloma

“…There are two reviews summarizing the profile of multiple myeloma cases with CNS involvement [1,3]. In the study of Nieuwenhuizen et al a better outcome with kappa CNS multiple myeloma when compared with the group with lambda CNS multiple myeloma had been suggested (P = 0.22).…”

“…There are only few reported cases in literature. Central nervous system involvement in multiple myeloma is only 1% [1]. Even more interesting is the presentation of multiple myeloma with CNS symptoms.…”

Unusual Manifestation of Multiple Myeloma: Focal Affection of Central Nervous System in a Patient With Chronic Lymphocytic Leukaemia

“…This has been referred by other studies based on cytogenetic and molecular analysis, as well [7][8] . The invasion of the central system by the multiple myeloma in the form of a leptomeningeal myelomatosis, an extramedullar propagation, or more rarely an intracerebral expansion, are frequently related to a less differentiated cellular morphology, frequently of the plasmablastic type, with a high-risk genetic profile, and also intensive previous therapy; the further course of the disease in these patients is usually very complicated and difficult to improve therapeutically [9][10][11] . In our observation, the primomanifestation of multiple myeloma in form of a focal neurologic symptoms allowed effective treatment of the disease, i.e.…”