Intravascular Large B-Cell Lymphoma Complicated by Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis that was Successfully Treated with Rituximab-Containing Chemotherapy

  • Tsuda Mariko
    Department of Medicine and Bioregulatory Science, Graduate School of Medical Sciences, Kyushu University
  • Nakashima Yasuhiro
    Department of Medicine and Bioregulatory Science, Graduate School of Medical Sciences, Kyushu University
  • Ikeda Motohiko
    Department of Medicine and Bioregulatory Science, Graduate School of Medical Sciences, Kyushu University
  • Shimada Shingo
    Department of Medicine and Bioregulatory Science, Graduate School of Medical Sciences, Kyushu University
  • Nomura Masatoshi
    Department of Medicine and Bioregulatory Science, Graduate School of Medical Sciences, Kyushu University
  • Matsushima Takamitsu
    Department of Medicine and Bioregulatory Science, Graduate School of Medical Sciences, Kyushu University
  • Takahashi Shunsuke
    Department of Anatomic Pathology, Graduate School of Medical Sciences, Kyushu University
  • Aishima Shinichi
    Department of Anatomic Pathology, Graduate School of Medical Sciences, Kyushu University
  • Oda Yoshinao
    Department of Anatomic Pathology, Graduate School of Medical Sciences, Kyushu University
  • Shiratsuchi Motoaki
    Department of Medicine and Bioregulatory Science, Graduate School of Medical Sciences, Kyushu University
  • Takayanagi Ryoichi
    Department of Medicine and Bioregulatory Science, Graduate School of Medical Sciences, Kyushu University

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A 64-year-old woman had suffered from painful livedo reticularis for 2 years and was referred to us due to fever, anasarca and paresthesia of the lower limbs. Serum proteinase-3-anti-neutrophil cytoplasmic antibody (ANCA) was positive. Abnormal lymphocytes were found in the cerebrospinal fluid and bone marrow. Skin biopsy revealed large atypical lymphoid cells with CD20 positivity lodged in the small vessels and neutrophilic infiltration into the arterial vascular wall with fibrinoid degeneration. A diagnosis of intravascular large B-cell lymphoma complicated by ANCA-associated vasculitis was made, and rituximab-containing chemotherapy followed by prednisolone was quite effective for both lymphoma and ANCA-associated vasculitis.

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