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
Affiliations
Hajime Yasuda
Department of Hematology, Juntendo University School of Medicine, Tokyo, Japan; Corresponding author.
Yuji Tomizawa
Department of Neurology, Juntendo University School of Medicine, Tokyo, Japan
Sakiko Harada
Department of Hematology, Juntendo University School of Medicine, Tokyo, Japan
Makoto Sasaki
Department of Hematology, Juntendo University School of Medicine, Tokyo, Japan
Norio Komatsu
Department of Hematology, Juntendo University School of Medicine, Tokyo, Japan; Laboratory for the Development of Therapies Against MPN, Juntendo University School of Medicine, Japan; Department of Advanced Hematology, Juntendo University School of Medicine, Japan
Jun Ando
Department of Hematology, Juntendo University School of Medicine, Tokyo, Japan; Department of Cell Therapy and Transfusion Medicine, Juntendo University School of Medicine, Japan
Nobutaka Hattori
Department of Neurology, Juntendo University School of Medicine, Tokyo, Japan
Miki Ando
Department of Hematology, Juntendo University School of Medicine, Tokyo, Japan
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.
