Hematology Reports (Feb 2017)

Successful intrathecal chemotherapy combined with radiotherapy followed by pomalidomide and low-dose dexamethasone maintenance therapy for a primary plasma cell leukemia patient

  • Yusuke Yamashita,
  • Shinobu Tamura,
  • Takehiro Oiwa,
  • Hiroshi Kobata,
  • Kodai Kuriyama,
  • Toshiki Mushino,
  • Shogo Murata,
  • Hiroki Hosoi,
  • Akinori Nishikawa,
  • Nobuyoshi Hanaoka,
  • Takashi Sonoki

DOI
https://doi.org/10.4081/hr.2017.6986
Journal volume & issue
Vol. 9, no. 1

Abstract

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Primary plasma cell leukemia (PPCL) is a rare aggressive variant of plasma cell disorder and frequently presents with extramedullary disease. Central nervous system (CNS) involvement with PPCL has an extremely poor prognosis. We describe a 46-year-old man with PPCL treated with a combination of lenalidomide, bortezomib, and dexamethasone as induction therapy following upfront allogeneic stem cell transplantation (allo-SCT). Despite achieving a very good partial response, the patient suffered from an isolated CNS relapse 12 months after allo-SCT. He was immediately started on concurrent intrathecal chemotherapy (IT) and cranial irradiation (RT). Subsequently, pomalidomide and low-dose dexamethasone (Pd) were given as maintenance therapy. He has been without CNS recurrence for more than 18 months. Our case suggests that concurrent IT and RT followed by Pd maintenance therapy may be an effective option to control CNS relapse of PPCL after allo-SCT.

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