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- Ryo Kikuchi, Masahiro Onozawa, Jun Nagai, Satomi Okada, Yuta Hasegawa, Hiroyuki Ohigashi, Shintaro Mitamura, Taku Maeda, Emi Takakuwa, Yuichiro Fujieda, Hideki Goto, Daigo Hashimoto, Yoshihiro Matsuno, and Takanori Teshima.
- Department of Hematology, Hokkaido University Hospital, Japan.
- Intern. Med. 2024 Feb 15; 63 (4): 541545541-545.
AbstractCryoglobulins are immunoglobulins that precipitate in cold conditions. Type I cryoglobulinemic vasculitis is associated with hematological malignancies. We herein report a case of steroid-resistant type 1 cryoglobulinemic vasculitis associated with monoclonal gammopathy of undetermined significance (MGUS) in a 47-year-old woman. By immunofixation of cryoglobulin, we found that the main component of cryoglobulin was the M protein due to MGUS, so treatment of MGUS was needed. Bortezomib+dexamethasone therapy resulted in a rapid decrease in cryoglobulin and improvement in the symptoms of cryoglobulinemic vasculitis. In refractory type I cryoglobulinemic vasculitis, treatment of the underlying gammaglobulinopathy should be considered.
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