Massive central nervous system infiltration by <scp>CD</scp>56‐positive plasma cells in multiple myeloma

Lopes, Annelise Correa Wengerkievicz; Xavier, Flávia Caló Aquino; Barroso, Rodrigo de Souza; Gomes, H. R.; Sales, Marcelo Augusto Oliveira de

doi:10.1111/cyt.12365

Cited by 2 publications

(1 citation statement)

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“…33 Although our own data suggested a higher incidence of CD56 loss in CNS-MM than in ND-MM, data from some other studies do not support this or the presence of a CNS-specific immunophenotype. 29,[34][35][36][37] Studies of EMD in general have revealed a putative biological signature which includes increased LDH, 7,38 along with evidence of a reduction in CD56 expression. 20,39 We found no difference in features such as cytogenetics, cytology and histopathology between CNS-MM diagnosed at the time of MM diagnosis and those diagnosed at relapse.…”

Section: Other Associationsmentioning

confidence: 99%

Multiple myeloma with central nervous system relapse

et al. 2020

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C entral nervous system involvement in multiple myeloma is a rare complication but carries a very poor prognosis. We provide a review of current literature, including presentation, treatment and survival data, and describe our experience in a regional hematologic malignancy diagnosis center where, over a 15-year period, ten cases were identified. Although the median age of onset, frequently between 50-60 years, is comparatively young, those diagnosed usually have a preceding diagnosis of multiple myeloma and often have had several lines of treatment. We discuss putative underlying factors such as prior treatment and associations including possible risk factors and features suggestive of a distinct biology. Central nervous system involvement may be challenging to diagnose in myeloma, displaying heterogeneous symptoms that can be confounded by neurological symptoms caused by the typical features of myeloma or treatment side-effects. We discuss the clinical features, imaging and laboratory methods used in diagnosis, and highlight the importance of considering this rare complication when neurological symptoms occur at presentation or, more commonly, during the disease pathway. In the absence of clinical trial data to inform an evidence-based approach to treatment, we discuss current and novel treatment options. Finally, we propose the establishment of an International Registry of such cases as the best way to collect and subsequently disseminate presentation, diagnostic and treatment outcome data on this rare complication of multiple myeloma.

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