Paraneoplastic Pemphigus Associated with B-cell Chronic Lymphocytic Leukemia Treated with Ibrutinib and Rituximab

  • Ito Yuta
    Department of Hematology, National Cancer Center Hospital, Japan
  • Makita Shinichi
    Department of Hematology, National Cancer Center Hospital, Japan
  • Maeshima Akiko Miyagi
    Department of Pathology, National Cancer Center Hospital, Japan
  • Hatta Shunsuke
    Department of Hematology, National Cancer Center Hospital, Japan
  • Suzuki Tomotaka
    Department of Hematology, National Cancer Center Hospital, Japan
  • Yuda Sayako
    Department of Hematology, National Cancer Center Hospital, Japan
  • Fukuhara Suguru
    Department of Hematology, National Cancer Center Hospital, Japan
  • Munakata Wataru
    Department of Hematology, National Cancer Center Hospital, Japan Rare Cancer Center, National Cancer Center Hospital, Japan
  • Suzuki Tatsuya
    Department of Hematology, National Cancer Center Hospital, Japan
  • Maruyama Dai
    Department of Hematology, National Cancer Center Hospital, Japan
  • Izutsu Koji
    Department of Hematology, National Cancer Center Hospital, Japan

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抄録

<p>Paraneoplastic pemphigus (PNP) is a severe autoimmune blistering disease associated with an underlying malignancy, and its prognosis is poor. We herein report the first patient with B-cell chronic lymphocytic leukemia/small lymphocytic lymphoma (B-CLL/SLL)-associated PNP successfully treated with the Bruton's tyrosine kinase inhibitor ibrutinib and rituximab. Although his PNP lesions did not improve with ibrutinib monotherapy, the combination of ibrutinib and rituximab was effective against B-CLL/SLL-associated PNP. This case suggests that ibrutinib plus rituximab may be a potent therapeutic option for B-CLL/SLL-associated PNP that is hard to control with ibrutinib alone. </p>

収録刊行物

  • Internal Medicine

    Internal Medicine 57 (16), 2395-2398, 2018-08-15

    一般社団法人 日本内科学会

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