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Lopes C, Costa A, Bergantim R, et al. Refractory CIDP successfully treated with autologous haematopoietic stem cell transplantation in a patient with monoclonal gammopathy of undetermined significance (MGUS) and rheumatoid arthritis. Neuromuscul Disord. 2021;doi: 10.1016/j.nmd.2021.11.008.
Lopes, C., Costa, A., Bergantim, R., Silveira, F., & Nadais, G. (2021). Refractory CIDP successfully treated with autologous haematopoietic stem cell transplantation in a patient with monoclonal gammopathy of undetermined significance (MGUS) and rheumatoid arthritis. Neuromuscular disorders : NMD, . https://doi.org/10.1016/j.nmd.2021.11.008
Lopes, Carolina, et al. "Refractory CIDP successfully treated with autologous haematopoietic stem cell transplantation in a patient with monoclonal gammopathy of undetermined significance (MGUS) and rheumatoid arthritis." Neuromuscular disorders : NMD vol. (2021). doi: https://doi.org/10.1016/j.nmd.2021.11.008
Lopes C, Costa A, Bergantim R, Silveira F, Nadais G. Refractory CIDP successfully treated with autologous haematopoietic stem cell transplantation in a patient with monoclonal gammopathy of undetermined significance (MGUS) and rheumatoid arthritis. Neuromuscul Disord. 2021 Nov 19; doi: 10.1016/j.nmd.2021.11.008. Epub 2021 Nov 19. PMID: 34920930.
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Neuromuscul Disord. 2021 Nov 19; doi: 10.1016/j.nmd.2021.11.008. Epub 2021 Nov 19.

Refractory CIDP successfully treated with autologous haematopoietic stem cell transplantation in a patient with monoclonal gammopathy of undetermined significance (MGUS) and rheumatoid arthritis.

Neuromuscular disorders : NMD

Carolina Lopes, Andreia Costa, Rui Bergantim, Fernando Silveira, Goreti Nadais

Affiliations

  1. Department of Neurology, Centro Hospitalar e Universitário de São João, Porto, Portugal; Neurology and Neurosurgery Unit, Department of Clinical Neurosciences and Mental Health, Faculty of Medicine, University of Porto, Porto, Portugal. Electronic address: [email protected].
  2. Department of Neurology, Centro Hospitalar e Universitário de São João, Porto, Portugal; Neurology and Neurosurgery Unit, Department of Clinical Neurosciences and Mental Health, Faculty of Medicine, University of Porto, Porto, Portugal.
  3. Department of Hematology, Centro Hospitalar e Universitário de São João, Porto, Portugal; i3S - Instituto de Investigação e Inovação em Saúde, University of Porto, Porto, Portugal; Cancer Drug Resistance Group, IPATIMUP - Institute of Molecular Pathology and Immunology, University of Porto, Porto, Portugal; Hematology & Oncology Unit, Department of Medicine, Faculty of Medicine, University of Porto, Porto, Portugal.
  4. Department of Neurology, Centro Hospitalar e Universitário de São João, Porto, Portugal.

PMID: 34920930 DOI: 10.1016/j.nmd.2021.11.008

Abstract

Autologous haematopoietic stem cell transplantation is an emerging treatment option in refractory chronic inflammatory demyelinating polyradiculoneuropathy. We describe a case of a 46-year-old male, with history of IgG/lambda monoclonal gammopathy, who was diagnosed with chronic inflammatory demyelinating polyradiculoneuropathy at 27 years of age. After an initial 10-year period of corticotherapy response, the patient experienced severe relapses and disease progression, evolving to a refractory state. First-line and escalating treatment could not achieve clinical stabilization, leading to severe disability. Pre-treatment with ibrutinib was initiated and autologous haematopoietic stem cell transplantation was performed without significant complications. Marked clinical improvement was observed in the following months, both subjective and objective. A significant proportion of the patients who respond to the first-line immunosuppressive therapy eventually become treatment-refractory. Autologous haematopoietic stem cell transplantation may be a treatment option, offering long-term remission with an overall acceptable side effect and risk profile.

Copyright © 2021 Elsevier B.V. All rights reserved.

Keywords: CIDP; Refractory; Stem cell transplantation

Conflict of interest statement

Declaration of Competing Interest The authors have no conflicts of interest related to this publication.

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