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9 slides generated from the cancer page, with a quiz from the open benchmark and speaker notes that cite the sources. Arrow keys move between slides; Print gives one slide per page.
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.
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.
| Setting | Approach | Guideline |
|---|---|---|
| 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. | not mapped |
| Consolidation | Autologous stem cell transplant for all fit patients; tandem autologous or reduced-intensity allogeneic transplant considered in young patients; continuous maintenance after transplant. | not mapped |
| 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. | not mapped |