Anti-myelin-associated-glycoprotein neuropathy successfully treated with tirabrutinib

Hajime Yasuda; Yuji Tomizawa; Sakiko Harada; Makoto Sasaki; Norio Komatsu; Jun Ando; Nobutaka Hattori; Miki Ando

doi:10.1016/j.heliyon.2022.e10928

Anti-myelin-associated-glycoprotein neuropathy successfully treated with tirabrutinib

Heliyon. 2022 Oct 5;8(10):e10928. doi: 10.1016/j.heliyon.2022.e10928. eCollection 2022 Oct.

Authors

Hajime Yasuda¹, Yuji Tomizawa², Sakiko Harada¹, Makoto Sasaki¹, Norio Komatsu^{1

3

4}, Jun Ando^{1

5}, Nobutaka Hattori², Miki Ando¹

Affiliations

¹ Department of Hematology, Juntendo University School of Medicine, Tokyo, Japan.
² Department of Neurology, Juntendo University School of Medicine, Tokyo, Japan.
³ Laboratory for the Development of Therapies Against MPN, Juntendo University School of Medicine, Japan.
⁴ Department of Advanced Hematology, Juntendo University School of Medicine, Japan.
⁵ Department of Cell Therapy and Transfusion Medicine, Juntendo University School of Medicine, Japan.

Abstract

Background: Anti-myelin-associated-glycoprotein (MAG) neuropathy is a distal, predominantly demyelinating, sensory or sensory-motor polyneuropathy most often developing in the context of an IgM-type monoclonal gammopathy due to monoclonal gammopathy of undetermined significance or lymphoplasmacytic lymphoma. Rituximab is considered standard therapy for treatment naïve patients, but optimal treatment methods for relapsed/refractory patients have not been established.

Case presentation: We demonstrate that tirabrutinib, a second-generation Burton kinase inhibitor, led to drastic improvements of polyneuropathy that were affirmed by nerve conduction studies in a rituximab-refractory anti-MAG neuropathy patient. Tirabrutinib continues to give excellent disease control with no apparent adverse events at 11 months since initiation, and the patient remains free of plasmapheresis sessions which were originally mandatory.

Conclusion: Tirabrutinib is an extremely promising treatment option for anti-MAG neuropathy.

Keywords: Anti-MAG antibody; BTK inhibitor; Bruton tyrosine kinase inhibitor; Ibrutinib; IgM MGUS; Lymphoplasmacytic lymphoma; Plasmapheresis; Rituximab; SGPG glycolipid; Sensory-motor type polyneuropathy.

Publication types

Case Reports