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Rare disease
Case report
Rare case of non-producer variant of plasma cell dyscrasias with circulating plasma cells
  1. Sukesh Manthri1,
  2. Rabia Zafar2,
  3. Robert Frank Cornell3 and
  4. Kanishka Chakraborty1
  1. 1Division of Hematology/Oncology, Department of Internal Medicine, East Tennessee State University, Johnson City, Tennessee, USA
  2. 2Pathology, East Tennessee State University, Johnson City, Tennessee, USA
  3. 3Division of Hematology/Oncology, Department of Medicine, Vanderbilt University, Nashville, Tennessee, USA
  1. Correspondence to Dr Kanishka Chakraborty; chakrabk{at}etsu.edu

Abstract

Non-producing variant of plasma cell disorders with circulating plasma cells is an aggressive variant of plasma cell dyscrasias with relatively poor outcomes. A 75-year-old man was admitted due to anaemia (90 g/L) and thrombocytopenia (9×109/L). Comprehensive metabolic panel showed creatinine of 1.34 mg/dL, total protein of 6 g/dL, and corrected calcium was normal. Peripheral smear review showed 8% circulating atypical plasmacytoid cells. Bone marrow biopsy (BMB) confirmed plasma cell myeloma involving 90%–95% of bone marrow cellularity. Serum protein electrophoresis showed no monoclonal protein. Due to aggressive biology of non-producer variant and outcomes based on circulating plasma cells, he was started on VD-PACE (bortezomib, dexamethasone, cisplatin, doxorubicin, cyclophosphamide and etoposide) chemotherapy. BMB after cycle 1 chemotherapy showed no morphologic, immunophenotypic, or flow cytometric features of a plasma cell neoplasm. Given excellent treatment response cycle 2 was changed to VRD (bortezomib, lenalidomide and dexamethasone). Following two cycles of VRD, he underwent autologous haematopoietic cell transplantation. Day 80 BMB suggested stringent complete response.

  • cancer intervention
  • haematology (incl blood transfusion)
  • oncology

https://doi.org/10.1136/bcr-2019-231314

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    Footnotes

    • Contributors SM worked on the background, case report and discussion. RZ provided us with pathology images. RFC and KC reviewed and edited the manuscript.

    • Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.

    • Competing interests None declared.

    • Patient consent for publication Obtained.

    • Provenance and peer review Not commissioned; externally peer reviewed.