{"entity":{"id":"plasma-cell-leukaemia","kind":"cancer","name":"Plasma cell leukaemia","aka":["PCL","Primary plasma cell leukaemia","Secondary plasma cell leukaemia","Leukaemic myeloma"],"tldr":"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.\n\nNo 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.\n\nResearch 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.","asOf":"2026-09-17","wikipedia":"https://en.wikipedia.org/wiki/Plasma_cell_leukemia","links":[{"label":"Wikipedia","url":"https://en.wikipedia.org/wiki/Plasma_cell_leukemia"},{"label":"NCCN Guidelines: Multiple Myeloma","url":"https://www.nccn.org/guidelines/guidelines-detail?category=1&id=1445"}],"tags":["subtype-page"],"related":[],"cancers":[],"sections":[],"technologies":["autologous-stem-cell-transplant","allogeneic-hsct","flow-cytometry-mrd"],"targets":["cd38","bcma","proteasome","tp53"],"drugs":[],"companies":[],"institutions":[],"pathways":[],"terms":["high-risk-myeloma","tp53-mutated","autologous-transplant","leukaemia-type","proteasome-inhibitor"],"trials":[],"people":[],"bottlenecks":[],"keyPapers":[],"journals":[],"dependsOn":[],"notes":[],"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"],"standardOfCare":[{"setting":"Primary plasma cell leukaemia, induction","approach":"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.","refs":["bortezomib","daratumumab","lenalidomide","cyclophosphamide","dexamethasone","carfilzomib","tumor-lysis-syndrome"],"guideline":{"version":"NCCN Guidelines: Multiple Myeloma","url":"https://www.nccn.org/guidelines/guidelines-detail?category=1&id=1445"}},{"setting":"Consolidation","approach":"Autologous stem cell transplant for all fit patients; tandem autologous or reduced-intensity allogeneic transplant considered in young patients; continuous maintenance after transplant.","refs":["autologous-stem-cell-transplant","allogeneic-hsct","melphalan","lenalidomide","maintenance-therapy"],"guideline":{"version":"NCCN Guidelines: Multiple Myeloma","url":"https://www.nccn.org/guidelines/guidelines-detail?category=1&id=1445"}},{"setting":"Secondary plasma cell leukaemia or relapse","approach":"Treated as heavily relapsed myeloma: BCMA or GPRC5D bispecific antibodies, CAR-T where feasible, selinexor or pomalidomide combinations; palliative care early.","refs":["teclistamab","talquetamab","ciltacabtagene-autoleucel","selinexor","pomalidomide"],"guideline":{"version":"NCCN Guidelines: Multiple Myeloma","url":"https://www.nccn.org/guidelines/guidelines-detail?category=1&id=1445"}}],"stateOfArt":["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."],"history":[{"year":1974,"title":"Kyle defines plasma cell leukaemia at 20 percent or 2 x 10^9/L circulating plasma cells","refs":[]},{"year":2013,"title":"IFM 2005-01 phase 2: bortezomib-based induction and transplant lengthens survival","refs":["bortezomib","autologous-stem-cell-transplant"]},{"year":2021,"title":"IMWG lowers the diagnostic threshold to 5 percent circulating plasma cells","refs":["flow-cytometry"]}],"pipeline":["teclistamab","ciltacabtagene-autoleucel","daratumumab","carfilzomib"],"openProblems":["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."],"parent":"multiple-myeloma"},"route":"/cancers/plasma-cell-leukaemia/","neighbours":{"technology":[{"id":"allogeneic-hsct","kind":"technology","name":"Allogeneic stem cell transplantation","route":"/technologies/allogeneic-hsct/"},{"id":"autologous-stem-cell-transplant","kind":"technology","name":"Autologous stem cell transplant (high-dose therapy)","route":"/technologies/autologous-stem-cell-transplant/"},{"id":"flow-cytometry-mrd","kind":"technology","name":"Multiparameter flow cytometry MRD","route":"/technologies/flow-cytometry-mrd/"}],"target":[{"id":"bcma","kind":"target","name":"BCMA","route":"/targets/bcma/"},{"id":"cd38","kind":"target","name":"CD38","route":"/targets/cd38/"},{"id":"proteasome","kind":"target","name":"Proteasome (PSMB5)","route":"/targets/proteasome/"},{"id":"tp53","kind":"target","name":"TP53","route":"/targets/tp53/"}],"term":[{"id":"autologous-transplant","kind":"term","name":"Autologous stem cell transplant (ASCT)","route":"/terms/autologous-transplant/"},{"id":"flow-cytometry","kind":"term","name":"Flow cytometry (immunophenotyping)","route":"/terms/flow-cytometry/"},{"id":"high-risk-myeloma","kind":"term","name":"High-risk cytogenetics (myeloma)","route":"/terms/high-risk-myeloma/"},{"id":"leukaemia-type","kind":"term","name":"Leukaemia (tissue type)","route":"/terms/leukaemia-type/"},{"id":"maintenance-therapy","kind":"term","name":"Maintenance therapy","route":"/terms/maintenance-therapy/"},{"id":"proteasome-inhibitor","kind":"term","name":"Proteasome inhibitor (bortezomib, carfilzomib, ixazomib)","route":"/terms/proteasome-inhibitor/"},{"id":"tp53-mutated","kind":"term","name":"TP53-mutated (p53-abnormal)","route":"/terms/tp53-mutated/"},{"id":"tumor-lysis-syndrome","kind":"term","name":"Tumour lysis syndrome (TLS)","route":"/terms/tumor-lysis-syndrome/"}],"drug":[{"id":"bortezomib","kind":"drug","name":"Bortezomib","route":"/drugs/bortezomib/"},{"id":"carfilzomib","kind":"drug","name":"Carfilzomib","route":"/drugs/carfilzomib/"},{"id":"ciltacabtagene-autoleucel","kind":"drug","name":"Ciltacabtagene autoleucel","route":"/drugs/ciltacabtagene-autoleucel/"},{"id":"cyclophosphamide","kind":"drug","name":"Cyclophosphamide","route":"/drugs/cyclophosphamide/"},{"id":"daratumumab","kind":"drug","name":"Daratumumab","route":"/drugs/daratumumab/"},{"id":"dexamethasone","kind":"drug","name":"Dexamethasone","route":"/drugs/dexamethasone/"},{"id":"lenalidomide","kind":"drug","name":"Lenalidomide","route":"/drugs/lenalidomide/"},{"id":"melphalan","kind":"drug","name":"Melphalan (including hepatic delivery system)","route":"/drugs/melphalan/"},{"id":"pomalidomide","kind":"drug","name":"Pomalidomide","route":"/drugs/pomalidomide/"},{"id":"selinexor","kind":"drug","name":"Selinexor","route":"/drugs/selinexor/"},{"id":"talquetamab","kind":"drug","name":"Talquetamab","route":"/drugs/talquetamab/"},{"id":"teclistamab","kind":"drug","name":"Teclistamab","route":"/drugs/teclistamab/"}],"cancer":[{"id":"multiple-myeloma","kind":"cancer","name":"Multiple myeloma","route":"/cancers/multiple-myeloma/"}]}}