Successful treatment of refractory cold hemagglutinemia in MYD88 L265P mutation-negative Waldenström’s macroglobulinemia with bortezomib

Mayuko Izumi, Hiroko Tsunemine, Yasuhiro Suzuki, Akihiro Tomita, Toshiko Kusumoto, Taiichi Kodaka, Kiminari Itoh, Takayuki Takahashi

研究成果: Article査読

3 被引用数 (Scopus)

抄録

We report here the successful treatment of cold agglutinin-associated refractory hemolysis with bortezomib in a patient with Waldenström’s macroglobulinemia (WM). A 78-year-old man was referred to our hospital with cold hemagglutinemia of unknown cause. Laboratory examination revealed a hemoglobin concentration of 6.9 g/dL, serum IgM concentration of 1904 mg/dL, and a titer of cold hemagglutinin of over ×8192. Serum immunoelectrophoresis demonstrated monoclonal protein of the IgM-κ type. A bone marrow aspirate showed many lymphoplasmacytic cells, which were positive for CD19, CD20, CD38, and cytoplasmic μ and κ light chains. A diagnosis of WM-associated cold hemagglutinemia was made. Because of red blood cell transfusion-dependency, we treated him with intravenous fludarabine, oral melphalan–prednisolone, cyclophosphamide, and melphalan, and two courses of R-CHOP in sequence with a marked decrease of serum IgM (928 mg). We then started weekly bortezomib plus dexamethasone (BD) therapy, as he was still transfusion-dependent. Soon after the initiation of BD, he achieved transfusion independence, with a further decrease in serum levels of IgM and marked improvement of anemia. Interestingly, his marrow abnormal lymphocytes were later found not to carry the MYD88 L265P mutation. The successful treatment with bortezomib for WM lacking this mutation is discussed.

本文言語English
ページ(範囲)238-243
ページ数6
ジャーナルInternational Journal of Hematology
102
2
DOI
出版ステータスPublished - 11-08-2015
外部発表はい

All Science Journal Classification (ASJC) codes

  • 血液学

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