Plasma cell leukaemia
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.
Overview
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.
State of the art
- 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.
Show survival figures (1)
Averages across everyone diagnosed, often years ago. Your stage, subtype, age, fitness and the treatment you receive matter more than the average, and the numbers are improving quickly.
- Bortezomib-based multi-drug induction followed by autologous transplant has lifted median survival from under a year to around three years in prospective series.
Red cards
From the labels and guidelines behind the standard of care. Your team's thresholds win.- Emergency services nowBlood clot (lenalidomide, pomalidomide, thalidomide)
A swollen painful calf, or sudden breathlessness with chest pain; venous and arterial thromboembolism is a boxed warning and blood-thinning prophylaxis is recommended.
- Emergency services nowBleeding or bruising
Bleeding that will not stop, black or bloody stools, or unexplained bruising when platelets are expected to be low.
- Emergency services nowCytokine release syndrome
Fever of 38 C or higher, chills, low blood pressure, fast heartbeat or breathlessness, especially after a step-up dose. Boxed warning on teclistamab, epcoritamab, glofitamab, tarlatamab and blinatumomab.
- Emergency services nowCytokine release syndrome
Fever of 38 C or higher is grade 1 CRS; fever with low blood pressure, fast heartbeat, breathlessness or low oxygen is grade 2 or higher. The labels carry a boxed warning and say to report fever immediately.
- Check before combiningLenalidomide with Dexamethasone: major interaction
Venous and arterial thromboembolism risk rises markedly with lenalidomide plus dexamethasone (and further with erythropoietin or oestrogens).. Thromboprophylaxis (aspirin, LMWH or a DOAC by risk) is standard.
- Check before combiningPomalidomide with Dexamethasone: major interaction
Thromboembolism risk with pomalidomide-dexamethasone.. Thromboprophylaxis is standard.
See all on the product pages:BortezomibCarfilzomibCiltacabtagene autoleucelCyclophosphamideDexamethasoneLenalidomideMelphalan (including hepatic delivery system)PomalidomideTalquetamabTeclistamab·Printable cards in the navigator
Anatomy and lymph node drainage
- Bone marrow (leukaemia, MDS, MPN, myeloma)
- Lymph node germinal centre (lymphomas)
- Spleen
- Blood (leukaemic phase)
- Lytic bone lesions (myeloma)
- Skin and extranodal sites
- Nodes: cervical
- Nodes: axillary
- Nodes: mediastinal
- Nodes: para-aortic and mesenteric
- Nodes: inguinal
Leukaemias, myeloma and MDS live in the marrow and blood; lymphomas grow in lymph nodes and spleen. The node stations are the disease map, not a route of spread, and staging counts them.
- Bone marrow (leukaemia, MDS, MPN, myeloma)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)
- Lymph node germinal centre (lymphomas)
- Spleen
- Blood (leukaemic phase)Primary plasma cell leukaemia (de novo, 5 percent or more circulating plasma cells) · Secondary plasma cell leukaemia (leukaemic transformation of established myeloma)
- Lytic bone lesions (myeloma)
- Skin and extranodal sites
- cervical
- axillary
- mediastinal
- para-aortic and mesenteric
- inguinal
In lymphoma the node stations are the disease itself; staging (Ann Arbor / Lugano) counts how many regions and sides of the diaphragm are involved.
Same organ: Marginal zone lymphoma, Cutaneous T-cell lymphoma (mycosis fungoides and Sezary syndrome), Primary mediastinal (thymic) large B-cell lymphoma, Leukaemia (all types), Acute myeloid leukaemia, Acute lymphoblastic leukaemia, Chronic lymphocytic leukaemia, Chronic myeloid leukaemia (CML), Diffuse large B-cell lymphoma, Follicular lymphoma, Hodgkin lymphoma, Mantle cell lymphoma, Multiple myeloma, Non-Hodgkin lymphoma (all types), Myelodysplastic syndromes / neoplasms (MDS), Myeloproliferative neoplasms (PV, ET, myelofibrosis), Polycythaemia vera (PV), Essential thrombocythaemia (ET), Waldenström macroglobulinaemia, Hairy cell leukaemia, Peripheral T-cell lymphomas (including cutaneous T-cell lymphoma), Blastic plasmacytoid dendritic cell neoplasm (BPDCN), Burkitt lymphoma, HIV-associated (AIDS-related) lymphomas, Chronic myelomonocytic leukaemia and MDS/MPN overlap neoplasms, Systemic mastocytosis, Erdheim-Chester disease, Rosai-Dorfman disease and other histiocytic neoplasms, Langerhans cell histiocytosis (LCH), Post-transplant lymphoproliferative disorder (PTLD), FLT3-mutated acute myeloid leukaemia, IDH1- and IDH2-mutated acute myeloid leukaemia, NPM1-mutated and KMT2A-rearranged acute myeloid leukaemia, Secondary and therapy-related acute myeloid leukaemia, Acute promyelocytic leukaemia, Acute myeloid leukaemia in older or unfit patients, Smouldering multiple myeloma, Newly diagnosed multiple myeloma, transplant-eligible, Newly diagnosed multiple myeloma, transplant-ineligible, Relapsed or refractory multiple myeloma, Lower-risk myelodysplastic syndromes, Higher-risk myelodysplastic syndromes, Chronic lymphocytic leukaemia, first treatment, Relapsed or refractory chronic lymphocytic leukaemia, Richter transformation of chronic lymphocytic leukaemia, Chronic myeloid leukaemia, chronic phase, Chronic myeloid leukaemia, accelerated and blast phase, Primary myelofibrosis
Show survival figures (1)
Averages across everyone diagnosed, often years ago. Your stage, subtype, age, fitness and the treatment you receive matter more than the average, and the numbers are improving quickly.
- 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.
Cases by country
No country-level case numbers. This cancer is not mapped to a GLOBOCAN site.
One section per setting: the options named, what each is for, the trials behind them, the recorded trade-offs and the questions to ask.
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.
Autologous stem cell transplant for all fit patients; tandem autologous or reduced-intensity allogeneic transplant considered in young patients; continuous maintenance after transplant.
Treated as heavily relapsed myeloma: BCMA or GPRC5D bispecific antibodies, CAR-T where feasible, selinexor or pomalidomide combinations; palliative care early.
Subtypes & biomarkers
top- 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)
- 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
How often this target appears
- 1974Kyle defines plasma cell leukaemia at 20 percent or 2 x 10^9/L circulating plasma cells
- 2013IFM 2005-01 phase 2: bortezomib-based induction and transplant lengthens survival
- 2021IMWG lowers the diagnostic threshold to 5 percent circulating plasma cells
Dated changes read from the records linked to this cancer: approvals, regulatory steps, reported trials, guideline versions and milestones. Newest first; no date is inferred.
All 4 changes by month →- 2026-09-17This recordPlasma cell leukaemiaFacts on this page last checked
When this page itself was last checked or edited.
- 2021MilestoneFlow cytometry (immunophenotyping)IMWG lowers the diagnostic threshold to 5 percent circulating plasma cells
A milestone in how this cancer is treated.
- 2013MilestoneBortezomibIFM 2005-01 phase 2: bortezomib-based induction and transplant lengthens survival
A milestone in how this cancer is treated.
- 1974MilestonePlasma cell leukaemiaKyle defines plasma cell leukaemia at 20 percent or 2 x 10^9/L circulating plasma cells
A milestone in how this cancer is treated.
What is in development for Plasma cell leukaemia, drawn from the whole corpus: 0 items. Drugs are grouped by the most advanced trial phase they have reached anywhere; approved treatments sit under standard of care. Technologies are the methods being tested for this cancer, trials are the studies recorded here, and ideas are proposals not yet in a trial.
Nothing recorded in development for this cancer yet.
Open problems and what is being done
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.
Trials
topTrials recruiting now
Country and place are remembered in this browser only. A postcode is sent to OpenStreetMap's Nominatim service to find coordinates when you press the button; nothing else leaves your device.
Expert centres
topExpert centres
New Haven, CT · consortium | United States | none recorded | 0 | 2,224 | 28,423 | none recorded | - |
Würzburg · cancer center | Germany | none recorded | 0 | 760 | 11,806 | - | |
Toulouse · cancer center | France | none recorded | 0 | 232 | 3,172 | - | |
| Italy | none recorded | 0 | 229 | 2,386 | - | ||
Indianapolis, IN · cancer center | United States | 0 | 159 | 4,238 | - | ||
Hamilton, ON · cancer center | Canada | none recorded | 0 | 103 | 1,158 | - | |
Baltimore, MD · cancer center | United States | 0 | 101 | 2,306 | - | ||
Omaha, NE · cancer center | United States | 0 | 37 | 4,159 | - | ||
Minneapolis, MN · cancer center | United States | 0 | not matched | - | - | ||
Atlanta, GA · cancer center | United States | 0 | not matched | - | - |
These are the things we can measure; they are not a ranking of quality. Each column is a field on the institution record or a count over what OnCo has linked; a centre that treats many patients with Plasma cell leukaemia but is thinly recorded here will look small.
Not known: OnCo holds no case-volume or outcome figures for centres, so none are shown. Where a national audit or registry publishes them, the centre's page links to it. Default order: Newsweek rank, then trials for this cancer, then research output.
Questions to ask
topQuestions to ask your oncologist about Plasma cell leukaemia
Newly diagnosed
- What is my exact diagnosis, stage, and grade, and which tests established them?Why: Everything else follows from an accurate stage and subtype.
- Which biomarkers have been tested on my tumour (for example Circulating plasma cells on blood filmand by flow cytometry, Lactate dehydrogenase, FISH: del, t, t, 1q gain, del, TP53 mutation, Renal function and calcium), and what were the results?Why: These results decide eligibility for targeted therapy, immunotherapy, and trials.
- Which subtype is my cancer, and does that change the recommended treatment?Why: Recognised subtypes for this cancer include Primary plasma cell leukaemia, Secondary plasma cell leukaemia, Plasma cell leukaemia with high-risk cytogenetics, t, 1q gain).
- Is germline (inherited) genetic testing recommended for me or my family?Why: Inherited variants can change treatment and matter for relatives.
Primary plasma cell leukaemia, induction
- For my situation (primary plasma cell leukaemia, induction), which of the standard options do you recommend and why?Why: Guideline options include: 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.
- Am I a candidate for Bortezomib, Daratumumab, Lenalidomide or related drugs, and what side effects should I expect?Why: Knowing the expected toxicities helps you plan work, family, and supportive care.
Consolidation
- For my situation (consolidation), which of the standard options do you recommend and why?Why: Guideline options include: Autologous stem cell transplant for all fit patients; tandem autologous or reduced-intensity allogeneic transplant considered in young patients; continuous maintenance after transplant.
- Am I a candidate for Melphalan (including hepatic delivery system), Lenalidomide, and what side effects should I expect?Why: Knowing the expected toxicities helps you plan work, family, and supportive care.
Secondary plasma cell leukaemia or relapse
- For my situation (secondary plasma cell leukaemia or relapse), which of the standard options do you recommend and why?Why: Guideline options include: Treated as heavily relapsed myeloma: BCMA or GPRC5D bispecific antibodies, CAR-T where feasible, selinexor or pomalidomide combinations; palliative care early.
- Am I a candidate for Teclistamab, Talquetamab, Ciltacabtagene autoleucel or related drugs, and what side effects should I expect?Why: Knowing the expected toxicities helps you plan work, family, and supportive care.
Any stage
- Are there clinical trials I could join, for example of Teclistamab, Ciltacabtagene autoleucel, Daratumumab, Carfilzomib?Why: Trials are how the next standard of care is set; asking early keeps options open.
- Would a second opinion at a high-volume centre change anything, and can you help arrange it?Why: Rare or high-stakes decisions benefit from a centre that treats many similar patients.
- What supportive care (symptom control, nutrition, exercise, mental health, financial help) is available from the start?Why: Supportive care improves quality of life and helps patients complete treatment.
- I read that “No randomised trials; treatment is extrapolated from high-risk myeloma”. How does that affect my plan?Why: Open problems are where trials and second opinions matter most.
- I read that “Secondary plasma cell leukaemia has no effective standard”. How does that affect my plan?Why: Open problems are where trials and second opinions matter most.
Newly diagnosed? Read the first 60 days with Plasma cell leukaemia, then print the one-page appointment sheet with room for the answers.
Print this page for your appointment (your browser's print command). These prompts are for discussion; your clinical team knows your case.
Direct links plus the targets, companies, and technologies of this cancer's products.
technologies
12targets
6drugs
12companies
10terms
8Latest papers
topQuery for this cancer: (TITLE:"Plasma cell leukaemia" OR ABSTRACT:"Plasma cell leukaemia" OR TITLE:"PCL" OR ABSTRACT:"PCL" OR TITLE:"Primary plasma cell leukaemia" OR ABSTRACT:"Primary plasma cell leukaemia" OR TITLE:"Secondary plasma cell leukaemia" OR ABSTRACT:"Secondary plasma cell leukaemia" OR TITLE:"Leukaemic myeloma" OR ABSTRACT:"Leukaemic myeloma") AND (treatment OR therapy OR trial OR survival OR diagnosis). Results are unfiltered search hits about Plasma cell leukaemia, not a curated reading list.
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