# Plasma cell leukaemia

Source: https://onco.cc/cancers/plasma-cell-leukaemia/  
OnCo record `plasma-cell-leukaemia` (Cancer). Data CC BY-NC 4.0, attribute "Data from OnCo (onco.cc)"; commercial use needs a licence.

## TL;DR

Plasma cell leukaemia is myeloma in which the cancerous plasma cells spill into the bloodstream in large numbers. It is the most aggressive plasma cell cancer and is treated urgently with several myeloma drugs at once followed by a stem cell transplant.

## Summary

The International Myeloma Working Group redefined plasma cell leukaemia in 2021 as 5 percent or more circulating plasma cells on a blood film in a patient with myeloma, replacing the older 20 percent threshold, because outcomes are equally poor above 5 percent. Primary plasma cell leukaemia presents de novo, often in younger patients with high tumour burden, kidney failure, hypercalcaemia, extramedullary disease and a high lactate dehydrogenase; secondary plasma cell leukaemia arises late in the course of established myeloma, and is worse still. High-risk cytogenetics, del(17p), t(14;16), 1q gain and del(1p), and TP53 mutations are far commoner than in ordinary myeloma.

No randomised trial exists. Treatment follows the most intensive myeloma regimens: a bortezomib-based induction with an immunomodulatory drug, dexamethasone and a CD38 antibody, or multi-agent chemotherapy combinations such as VTD-PACE or hyper-CVAD for rapid control, then autologous stem cell transplant in every fit patient, with tandem transplant or a reduced-intensity allogeneic transplant considered in the young, followed by maintenance. Prospective phase 2 series from the French and European myeloma groups with bortezomib-based induction and transplant report median survivals of around three years, against under a year with older chemotherapy. Secondary plasma cell leukaemia is treated as heavily relapsed myeloma with bispecific antibodies or CAR-T where available, though data are limited to small series.

Research is limited by rarity: registries, retrospective series and inclusion in high-risk myeloma trials are the evidence base. Whether BCMA-directed immunotherapy in the front line can change the outlook, as it has in relapsed myeloma, is the open question.

## Fields

- Kind: Cancer
- Last checked: 2026-09-17
- Also known as: PCL; Primary plasma cell leukaemia; Secondary plasma cell leukaemia; Leukaemic myeloma
- Tags: subtype-page
- Group: haematologic
- Burden: A rare and aggressive form of plasma cell cancer, a few percent of myeloma presentations at most; historically fatal within a year, now often controlled for several years with multi-drug induction and transplant.
- Subtypes: Primary plasma cell leukaemia (de novo, 5 percent or more circulating plasma cells); Secondary plasma cell leukaemia (leukaemic transformation of established myeloma); Plasma cell leukaemia with high-risk cytogenetics (del(17p), t(14;16), 1q gain)
- Biomarkers: Circulating plasma cells on blood film (5 percent or more) and by flow cytometry; Lactate dehydrogenase; FISH: del(17p), t(14;16), t(11;14), 1q gain, del(1p); TP53 mutation; Renal function and calcium; Serum free light chains and M-protein; Extramedullary disease on PET-CT

## Sections of this record

The page is a hub with ten sections in reading order; large sections have their own page. The same plan as JSON: https://onco.cc/api/v1/cancers/plasma-cell-leukaemia/sections.json

- Overview (on the hub): The TL;DR, the family this cancer belongs to, the organ, who gets it and what the state of the art is. https://onco.cc/cancers/plasma-cell-leukaemia/#overview [3 state-of-the-art points]
- Types and stages (on the hub): Anatomy, the subtypes and how they differ, how it is staged, and where advanced disease spreads. https://onco.cc/cancers/plasma-cell-leukaemia/#what-it-is [3 subtypes]
- Symptoms and diagnosis (on the hub): How this cancer shows itself, how the diagnosis is confirmed, and the biomarkers clinicians test for. https://onco.cc/cancers/plasma-cell-leukaemia/#finding-it [7 biomarkers]
- Treatment (on the hub): The standard of care by setting, the medicines, surgery and radiotherapy named in it, and the regimens behind them. https://onco.cc/cancers/plasma-cell-leukaemia/#treating-it [3 settings, 3 decisions with options]
- Trials and papers (on the hub): Trials recruiting now, the landmark trials, the trials held by this cancer's subtypes, the key papers and what they mean, the latest literature, and the milestones year by year. https://onco.cc/cancers/plasma-cell-leukaemia/#evidence [1 trial, 4 key papers, 3 milestones]
- Biology and targets (on the hub): The molecular landscape: the targets and how often each appears, the pathways, the mechanics stages and the preclinical models. https://onco.cc/cancers/plasma-cell-leukaemia/#science [8 targets]
- Countries and centres (own page): Cases by country, the UK and NHS pathway and other country lenses, the expert centres with trials on record, and the centres named on this cancer's subtypes. https://onco.cc/cancers/plasma-cell-leukaemia/where-you-are/
- Decisions and support (on the hub): The decisions you may face, the aids that walk through them, the warnings on record, the first sixty days and the questions to ask. https://onco.cc/cancers/plasma-cell-leukaemia/#living-with-it [15 questions, 6 red cards]
- Pipeline and open problems (own page): Everything in development, the medicines held by this cancer's subtypes, the open problems and what is being done about them, the roadmaps, and what changed on this record. https://onco.cc/cancers/plasma-cell-leukaemia/coming/ [12 medicines, 1 trial, 3 open problems]
- Data (own page): Every connected record, the notes, the JSON, Markdown and RDF twins, and where the record came from and when it was checked. https://onco.cc/cancers/plasma-cell-leukaemia/data/ [57 connected records]

## Standard of care

- Primary plasma cell leukaemia, induction: Bortezomib-based quadruplet (daratumumab with bortezomib, lenalidomide or cyclophosphamide, and dexamethasone), or VTD-PACE-type multi-agent chemotherapy for rapid control, with tumour lysis and renal precautions. ([Bortezomib](https://onco.cc/drugs/bortezomib/), [Daratumumab](https://onco.cc/drugs/daratumumab/), [Lenalidomide](https://onco.cc/drugs/lenalidomide/), [Cyclophosphamide](https://onco.cc/drugs/cyclophosphamide/), [Dexamethasone](https://onco.cc/drugs/dexamethasone/), [Carfilzomib](https://onco.cc/drugs/carfilzomib/), [Tumour lysis syndrome (TLS)](https://onco.cc/terms/tumor-lysis-syndrome/))
- Consolidation: Autologous stem cell transplant for all fit patients; tandem autologous or reduced-intensity allogeneic transplant considered in young patients; continuous maintenance after transplant. ([Autologous stem cell transplant (high-dose therapy)](https://onco.cc/technologies/autologous-stem-cell-transplant/), [Allogeneic stem cell transplantation](https://onco.cc/technologies/allogeneic-hsct/), [Melphalan (including hepatic delivery system)](https://onco.cc/drugs/melphalan/), [Lenalidomide](https://onco.cc/drugs/lenalidomide/), [Maintenance therapy](https://onco.cc/terms/maintenance-therapy/))
- Secondary plasma cell leukaemia or relapse: Treated as heavily relapsed myeloma: BCMA or GPRC5D bispecific antibodies, CAR-T where feasible, selinexor or pomalidomide combinations; palliative care early. ([Teclistamab](https://onco.cc/drugs/teclistamab/), [Talquetamab](https://onco.cc/drugs/talquetamab/), [Ciltacabtagene autoleucel](https://onco.cc/drugs/ciltacabtagene-autoleucel/), [Selinexor](https://onco.cc/drugs/selinexor/), [Pomalidomide](https://onco.cc/drugs/pomalidomide/))

## State of the art

- Bortezomib-based multi-drug induction followed by autologous transplant has lifted median survival from under a year to around three years in prospective series.
- The 2021 IMWG definition at 5 percent circulating plasma cells identifies more patients early.
- Immune therapies are being borrowed from relapsed myeloma, with only small series to guide them.

## Open problems

- No randomised trials; treatment is extrapolated from high-risk myeloma.
- Secondary plasma cell leukaemia has no effective standard.
- Whether front-line BCMA immunotherapy or allogeneic transplant improves long-term survival.

## Sources

- Wikipedia: https://en.wikipedia.org/wiki/Plasma_cell_leukemia
- Wikipedia: https://en.wikipedia.org/wiki/Plasma_cell_leukemia
- NCCN Guidelines: Multiple Myeloma: https://www.nccn.org/guidelines/guidelines-detail?category=1&id=1445

## Connected records

- cancers: [Multiple myeloma](https://onco.cc/cancers/multiple-myeloma/), [Newly diagnosed multiple myeloma, transplant-eligible](https://onco.cc/cancers/myeloma-transplant-eligible/), [Newly diagnosed multiple myeloma, transplant-ineligible](https://onco.cc/cancers/myeloma-transplant-ineligible/), [Relapsed or refractory multiple myeloma](https://onco.cc/cancers/myeloma-relapsed-refractory/), [Smouldering multiple myeloma](https://onco.cc/cancers/smouldering-myeloma/)
- technologies: [Allogeneic stem cell transplantation](https://onco.cc/technologies/allogeneic-hsct/), [Autologous stem cell transplant (high-dose therapy)](https://onco.cc/technologies/autologous-stem-cell-transplant/), [Multiparameter flow cytometry MRD](https://onco.cc/technologies/flow-cytometry-mrd/)
- targets: [BCMA](https://onco.cc/targets/bcma/), [CD38](https://onco.cc/targets/cd38/), [Proteasome (PSMB5)](https://onco.cc/targets/proteasome/), [TP53](https://onco.cc/targets/tp53/)
- terms: [Autologous stem cell transplant (ASCT)](https://onco.cc/terms/autologous-transplant/), [Flow cytometry (immunophenotyping)](https://onco.cc/terms/flow-cytometry/), [High-risk cytogenetics (myeloma)](https://onco.cc/terms/high-risk-myeloma/), [Leukaemia (tissue type)](https://onco.cc/terms/leukaemia-type/), [M-protein, immunofixation and serum free light chains](https://onco.cc/terms/m-protein-free-light-chains/), [Maintenance therapy](https://onco.cc/terms/maintenance-therapy/), [Proteasome inhibitor (bortezomib, carfilzomib, ixazomib)](https://onco.cc/terms/proteasome-inhibitor/), [t(11;14), cyclin D1 and SOX11](https://onco.cc/terms/cyclin-d1-t11-14/), [TP53-mutated (p53-abnormal)](https://onco.cc/terms/tp53-mutated/), [Tumour lysis syndrome (TLS)](https://onco.cc/terms/tumor-lysis-syndrome/)
- key papers: [IFM 2006 prospective trial: bortezomib-based induction and transplantation for primary plasma cell leukaemia](https://onco.cc/key-papers/paper-royer-plasma-cell-leukaemia-ifm-jco-2016/), [IMWG consensus definition of primary plasma cell leukaemia at 5 percent circulating plasma cells](https://onco.cc/key-papers/paper-imwg-plasma-cell-leukaemia-definition-bcj-2021/), [IMWG updated criteria for the diagnosis of multiple myeloma (2014)](https://onco.cc/key-papers/paper-imwg-criteria-rajkumar-lancet-oncol-2014/), [Revised International Staging System (R-ISS) for multiple myeloma](https://onco.cc/key-papers/paper-r-iss-palumbo-jco-2015/)
- drugs: [Bortezomib](https://onco.cc/drugs/bortezomib/), [Carfilzomib](https://onco.cc/drugs/carfilzomib/), [Ciltacabtagene autoleucel](https://onco.cc/drugs/ciltacabtagene-autoleucel/), [Cyclophosphamide](https://onco.cc/drugs/cyclophosphamide/), [Daratumumab](https://onco.cc/drugs/daratumumab/), [Dexamethasone](https://onco.cc/drugs/dexamethasone/), [Lenalidomide](https://onco.cc/drugs/lenalidomide/), [Melphalan (including hepatic delivery system)](https://onco.cc/drugs/melphalan/), [Pomalidomide](https://onco.cc/drugs/pomalidomide/), [Selinexor](https://onco.cc/drugs/selinexor/), [Talquetamab](https://onco.cc/drugs/talquetamab/), [Teclistamab](https://onco.cc/drugs/teclistamab/)
- trials: [Panobinostat, Gemcitabine Hydrochloride, Busulfan, and Melphalan Before Stem Cell Transplant in Treating Patients With Refractory or Relapsed Multiple Myeloma](https://onco.cc/trials/nct02506959/)

---
JSON: https://onco.cc/api/v1/entities/plasma-cell-leukaemia.json