Aggressive Central Nervous System Relapse after Autologous Stem Cell Transplant in Multiple Myeloma: Case Reports and Literature Review

Extramedullary disease is an aggressive presentation at diagnosis and relapse for multiple myeloma (MM) patients. Central nervous system (CNS) is a very rare manifestation of the extramedullary disease, accounting for less than 1% of MM on diagnosis and relapse. Neurological symptoms are unspecific...

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Main Authors: Rui Bergantim, Juliana Bastos, Maria José Soares, Bruno Carvalho, Pedro Soares, Cristina Marques, Jennifer Costa, José Eduardo Guimarães, Fernanda Trigo
Format: Article
Language:English
Published: Wiley 2020-01-01
Series:Case Reports in Hematology
Online Access:http://dx.doi.org/10.1155/2020/8563098
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author Rui Bergantim
Juliana Bastos
Maria José Soares
Bruno Carvalho
Pedro Soares
Cristina Marques
Jennifer Costa
José Eduardo Guimarães
Fernanda Trigo
author_facet Rui Bergantim
Juliana Bastos
Maria José Soares
Bruno Carvalho
Pedro Soares
Cristina Marques
Jennifer Costa
José Eduardo Guimarães
Fernanda Trigo
author_sort Rui Bergantim
collection DOAJ
description Extramedullary disease is an aggressive presentation at diagnosis and relapse for multiple myeloma (MM) patients. Central nervous system (CNS) is a very rare manifestation of the extramedullary disease, accounting for less than 1% of MM on diagnosis and relapse. Neurological symptoms are unspecific and usually attributed to other causes. We present two patients with CNS-MM at relapse after autologous stem cell transplant highlighting the importance of clinical suspicion and interdisciplinarity at diagnostic workup as well as the need for intensive therapeutic options on such rare and aggressive cases. The presence of neurological abnormalities in anamnesis and physical examination on a patient with MM should always prompt to suspect of a CNS involvement, and active investigation must be undertaken. MRI is the standard radiological method to detect CNS-MM, with histopathological corroboration by stereotactic biopsy and CSF evaluation alongside. Treatment of CNS-MM should include two essential approaches—be able to cross the BBB and treat the systemic disease. There is no standard therapy for this extramedullary relapse, and a tailored and multiple therapy should be promptly started—intrathecal therapy, radiotherapy, and systemic therapy, including an immunomodulator.
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institution Kabale University
issn 2090-6560
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language English
publishDate 2020-01-01
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series Case Reports in Hematology
spelling doaj-art-5ecff1366de64e50aee3b333b3e8a9ad2025-02-03T06:43:39ZengWileyCase Reports in Hematology2090-65602090-65792020-01-01202010.1155/2020/85630988563098Aggressive Central Nervous System Relapse after Autologous Stem Cell Transplant in Multiple Myeloma: Case Reports and Literature ReviewRui Bergantim0Juliana Bastos1Maria José Soares2Bruno Carvalho3Pedro Soares4Cristina Marques5Jennifer Costa6José Eduardo Guimarães7Fernanda Trigo8Hematology Department, Centro Hospitalar São João, Porto, PortugalHematology Department, Centro Hospitalar São João, Porto, PortugalHematology Department, Centro Hospitalar São João, Porto, PortugalHematology & Oncology Unit, Department of Medicine, Faculty of Medicine of University of Porto, Porto, PortugalRadiotherapy Department, Centro Hospitalar São João, Porto, PortugalFlow Cytometry Laboratory, Clinical Pathology Department, Centro Hospitalar São João, Porto, PortugalPathologic Anatomy Department, Centro Hospitalar São João, Porto, PortugalHematology Department, Centro Hospitalar São João, Porto, PortugalHematology Department, Centro Hospitalar São João, Porto, PortugalExtramedullary disease is an aggressive presentation at diagnosis and relapse for multiple myeloma (MM) patients. Central nervous system (CNS) is a very rare manifestation of the extramedullary disease, accounting for less than 1% of MM on diagnosis and relapse. Neurological symptoms are unspecific and usually attributed to other causes. We present two patients with CNS-MM at relapse after autologous stem cell transplant highlighting the importance of clinical suspicion and interdisciplinarity at diagnostic workup as well as the need for intensive therapeutic options on such rare and aggressive cases. The presence of neurological abnormalities in anamnesis and physical examination on a patient with MM should always prompt to suspect of a CNS involvement, and active investigation must be undertaken. MRI is the standard radiological method to detect CNS-MM, with histopathological corroboration by stereotactic biopsy and CSF evaluation alongside. Treatment of CNS-MM should include two essential approaches—be able to cross the BBB and treat the systemic disease. There is no standard therapy for this extramedullary relapse, and a tailored and multiple therapy should be promptly started—intrathecal therapy, radiotherapy, and systemic therapy, including an immunomodulator.http://dx.doi.org/10.1155/2020/8563098
spellingShingle Rui Bergantim
Juliana Bastos
Maria José Soares
Bruno Carvalho
Pedro Soares
Cristina Marques
Jennifer Costa
José Eduardo Guimarães
Fernanda Trigo
Aggressive Central Nervous System Relapse after Autologous Stem Cell Transplant in Multiple Myeloma: Case Reports and Literature Review
Case Reports in Hematology
title Aggressive Central Nervous System Relapse after Autologous Stem Cell Transplant in Multiple Myeloma: Case Reports and Literature Review
title_full Aggressive Central Nervous System Relapse after Autologous Stem Cell Transplant in Multiple Myeloma: Case Reports and Literature Review
title_fullStr Aggressive Central Nervous System Relapse after Autologous Stem Cell Transplant in Multiple Myeloma: Case Reports and Literature Review
title_full_unstemmed Aggressive Central Nervous System Relapse after Autologous Stem Cell Transplant in Multiple Myeloma: Case Reports and Literature Review
title_short Aggressive Central Nervous System Relapse after Autologous Stem Cell Transplant in Multiple Myeloma: Case Reports and Literature Review
title_sort aggressive central nervous system relapse after autologous stem cell transplant in multiple myeloma case reports and literature review
url http://dx.doi.org/10.1155/2020/8563098
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