Early development of extramedullary disease in multiple myeloma with no high-risk chromosomal abnormalities and good clinical course

DOI
  • TAKESHITA Masataka
    Department of Hematology, Tokyo-Kita Medical Center, Kita-ku, Tokyo, Japan International Myeloma Center for Advanced Research and Treatment, Kita-ku, Tokyo, Japan
  • KODAMA Nobuyuki
    Department of Hematology, Tokyo-Kita Medical Center, Kita-ku, Tokyo, Japan International Myeloma Center for Advanced Research and Treatment, Kita-ku, Tokyo, Japan
  • HISHIMA Tomoko
    Department of Hematology, Tokyo-Kita Medical Center, Kita-ku, Tokyo, Japan International Myeloma Center for Advanced Research and Treatment, Kita-ku, Tokyo, Japan
  • HIRAI Risen
    Department of Hematology, Tokyo-Kita Medical Center, Kita-ku, Tokyo, Japan
  • TANIMURA Akira
    Department of Hematology, Tokyo-Kita Medical Center, Kita-ku, Tokyo, Japan
  • OKUDA Yuko
    Department of Hematology, Tokyo-Kita Medical Center, Kita-ku, Tokyo, Japan
  • OKAZAKI Kouji
    Department of Hematology, Tokyo-Kita Medical Center, Kita-ku, Tokyo, Japan
  • KUDO Daisuke
    Department of Hematology, Tokyo-Kita Medical Center, Kita-ku, Tokyo, Japan
  • MIWA Akiyoshi
    Department of Hematology, Tokyo-Kita Medical Center, Kita-ku, Tokyo, Japan International Myeloma Center for Advanced Research and Treatment, Kita-ku, Tokyo, Japan

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<p>The treatment of multiple myeloma (MM) has made dramatic advance over the past decade with novel agents and cell therapy. However, prognosis is still poor for relapsed MM patients. Extramedullary disease (EMD), mostly seen at later stages of refractory or relapsed multiple myeloma (RRMM), has higher incidence of chromosomal abnormalities with poor prognosis and is actually resistant to anti-myeloma drugs. Overcoming EMDs is one of the unmet medical needs in the treatment of MM, especially in RRMM. We experienced a rare case of MM, who developed multiple EMDs early after diagnosis, without high-risk chromosomal abnormalities. Although the EMDs affected intracranial nerve, suggesting poorer prognosis, the patient showed drastic response to initial therapies, and maintained complete response for two years after autologous transplantation. The other cases we experienced, who developed progressive EMDs early in their clinical courses, had one or more high-risk chromosomal abnormalities and showed definite intractable clinical course to induction therapy, which showed sharp contrast with the present case. This case with an unexpected clinical course suggests that extramedullary mass formation is not only triggered by accumulation of chromosomal abnormalities in MM cells, but also influenced by certain factors in the genetic background or microenvironment of the patient.</p>

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