Brentuximab vedotin for refractory anaplastic lymphoma kinase-negative anaplastic large cell lymphoma in leukemic phase with RUNX3 overexpression

  • Yusuke Yamashita Department of Hematology/Oncology, Wakayama Medical University, Wakayama, Japan.
  • Yoshikazu Hori Department of Hematology/Oncology, Wakayama Medical University, Wakayama, Japan.
  • Hideki Kosako Department of Hematology/Oncology, Wakayama Medical University, Wakayama, Japan.
  • Takehiro Oiwa Department of Hematology/Oncology, Wakayama Medical University, Wakayama, Japan.
  • Kenji Warigaya Department of Diagnostic Pathology, Wakayama Medical University, Wakayama, Japan.
  • Toshiki Mushino Department of Hematology/Oncology, Wakayama Medical University, Wakayama, Japan.
  • Shogo Murata Department of Hematology/Oncology, Wakayama Medical University, Wakayama, Japan.
  • Masakazu Fujimoto Department of Diagnostic Pathology, Wakayama Medical University, Wakayama, Japan.
  • Akinori Nishikawa Department of Hematology/Oncology, Wakayama Medical University, Wakayama, Japan.
  • Shin-ichi Murata Department of Diagnostic Pathology, Wakayama Medical University, Wakayama, Japan.
  • Takashi Sonoki Department of Hematology/Oncology, Wakayama Medical University, Wakayama, Japan.
  • Shinobu Tamura | stamura@wakayama-med.ac.jp Department of Hematology/Oncology, Wakayama Medical University, Wakayama, Japan.

Abstract

Anaplastic lymphoma kinase (ALK)- negative anaplastic large cell lymphoma (ALCL) is an aggressive CD30-positive non- Hodgkin lymphoma. ALK-ALCL rarely manifests with extensive bone marrow and peripheral blood involvement (known as “leukemic phase”). A 54-year-old woman was diagnosed with ALK-ALCL in leukemic phase, characterized by an extremely poor prognosis. Lymphoma cells in this case showed chromosomal translocation 1p36.1- encoded RUNX3 and overexpression of its protein. She was refractory to CHOP and salvage chemotherapy. Fortunately, she achieved complete remission with three cycles of Brentuximab vedotin (BV) and underwent umbilical cord blood transplantation. However, she died due to treatment-related mortality on day 129. The autopsy findings showed no lymphoma cells. Treatment strategy for ALK-ALCL is controversial, but the efficacy of BV in CD30-positive peripheral T-cell lymphoma not only as salvage regimens, but also in first line, has been reported in recent years. BV may be an effective option for ALK-ALCL in leukemic phase.

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Published
2020-05-15
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Section
Case Reports
Keywords:
ALK-negative anaplastic large cell lymphoma, Brentuximab vedotin, leukemic phase, RUNX3
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How to Cite
Yamashita, Y., Hori, Y., Kosako, H., Oiwa, T., Warigaya, K., Mushino, T., Murata, S., Fujimoto, M., Nishikawa, A., Murata, S.- ichi, Sonoki, T., & Tamura, S. (2020). Brentuximab vedotin for refractory anaplastic lymphoma kinase-negative anaplastic large cell lymphoma in leukemic phase with <em>RUNX3</em&gt; overexpression. Hematology Reports, 12(1). https://doi.org/10.4081/hr.2020.8368

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