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Review
. 2019 Jan 28;21(1):8.
doi: 10.1007/s11912-019-0754-x.

Plasma Cell Leukemia: Definition, Presentation, and Treatment

Michael Tveden Gundesen  1 , Thomas Lund  1 , Hanne E H Moeller  2 , Niels Abildgaard  3   4
Affiliations
Review

Plasma Cell Leukemia: Definition, Presentation, and Treatment

Michael Tveden Gundesen et al. Curr Oncol Rep. .

Abstract

Purpose of review: We discuss current topics on the definition of plasma cell leukemia and the distinction between plasma cell leukemia and multiple myeloma. Moreover, we review the latest literature on how to treat plasma cell leukemia.

Recent findings: Plasma cell leukemia is clinically and genetically distinct from multiple myeloma. Plasma cell leukemia is defined by the observation in blood of more than 20% clonal plasma cells by differential count of the leucocytes or by counting more than 2 × 109 per liter circulating clonal plasma cells. However, patients with lower levels of circulating plasma cells have the same adverse prognosis, which challenges the disease definition. Survival has improved after implementation of high-dose chemotherapy with stem-cell support, bortezomib, and lenalidomide in the treatment; yet, the prognosis remains poor. The results of allo-transplants have been disappointing. The diagnostic criteria of PCL are currently discussed in the international myeloma community. Despite some improvement in survival, the prognosis remains adverse. New, more targeted treatment modalities, including immunotherapies, will hopefully improve the outcome in the near future.

Keywords: Cytogenetics; Diagnosis; Molecular biology; Plasma cell leukemia; Prognosis; Treatment.

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Conflict of interest statement

Conflict of Interest

The authors declare that they have no conflict of interest.

Human and Animal Rights and Informed Consent

All reported studies/experiments with human or animal subjects performed by the authors have been previously published and complied with all applicable ethical standards (including the Helsinki Declaration and its amendments, institutional/national research committee standards, and international/national/institutional guidelines).

Figures

Fig. 1
Fig. 1
Left: Blood smear showing plasma cells constituting > 20% of total leukocytes. The plasma cells are atypical with high nuclear/cytoplasmic ratio. Right: flow cytometry histograms of blood. The neoplastic plasma cells indicated in red and purple (CD56 positive and negative fraction respective) express CD138, bright CD38, CD45, cytoplasmic kappa and are negative for CD19 and cytoplasmic lambda
See this image and copyright information in PMC

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