{"entity":{"id":"splenic-b-cell-lymphoma-leukaemia-prominent-nucleoli","kind":"cancer","name":"Splenic B-cell lymphoma/leukaemia with prominent nucleoli (formerly B-cell prolymphocytic leukaemia and hairy cell leukaemia variant)","aka":["SBLPN","B-cell prolymphocytic leukaemia","B-cell prolymphocytic leukemia","B-PLL","Hairy cell leukaemia variant","HCL-v","Splenic B-cell lymphoma/leukemia with prominent nucleoli"],"tldr":"Splenic B-cell lymphoma/leukaemia with prominent nucleoli is the new WHO name for a rare group of B-cell leukaemias of older adults with a big spleen, large cells with obvious nucleoli and a poor response to standard treatment; it absorbs the old diagnoses B-cell prolymphocytic leukaemia and hairy cell leukaemia variant. It is treated with rituximab-based chemotherapy or newer targeted drugs.","summary":"WHO-HAEM5 discontinued B-cell prolymphocytic leukaemia as an entity and reframed hairy cell leukaemia variant, placing cases previously classified as CD5-negative B-PLL and as hairy cell leukaemia variant, together with splenic marginal zone and splenic diffuse red pulp lymphomas with many medium or large nucleolated cells, in a new category, splenic B-cell lymphoma/leukaemia with prominent nucleoli, as a temporary holding ground for cases that do not fit the existing classification; B-PLL had overlapped with chronic lymphocytic leukaemia and mantle cell lymphoma, and hairy cell leukaemia variant lacked consistent features (Alaggio 2022; British Journal of Haematology 2024). The old B-PLL literature described an aggressive mature B-cell disorder with p53 abnormalities in about half: loss of heterozygosity at 17p in 53 percent and TP53 mutations in 53 percent of 19 cases, a pattern distinct from CLL (Lens 1997). A three-case series of the renamed entity describes middle-aged men with cytopenias and CD25-negative or dim hairy cells that respond worse to therapy than classic hairy cell leukaemia (J Cancer Res Ther 2024).\n\nHow it differs from its parent: it is a diagnosis of exclusion among the splenic B-cell leukaemias, made after CLL with prolymphocytoid progression, mantle cell lymphoma (cyclin D1, SOX11) and classic hairy cell leukaemia (BRAF V600E, CD25, annexin A1) have been ruled out, and it behaves worse than any of them.\n\nHow common: no figure for the new category; the constituent diagnoses were uncommon (British Journal of Haematology 2024).\n\nTreatment: no trial exists; rituximab with chemotherapy (bendamustine or cladribine-based regimens borrowed from hairy cell leukaemia variant practice), BTK inhibitors and venetoclax by analogy with TP53-disrupted CLL, and allogeneic transplant for fit patients, following the CLL and hairy cell leukaemia pages.","asOf":"2026-09-24","wikipedia":"https://en.wikipedia.org/wiki/B-cell_prolymphocytic_leukemia","links":[{"label":"NCI PDQ: chronic lymphocytic leukaemia treatment","url":"https://www.cancer.gov/types/leukemia/patient/cll-treatment-pdq"},{"label":"Alaggio 2022, Leukemia: the 5th edition WHO classification of haematolymphoid tumours, lymphoid neoplasms","url":"https://doi.org/10.1038/s41375-022-01620-2"},{"label":"British Journal of Haematology 2024: understanding splenic B-cell lymphoma/leukaemia with prominent nucleoli","url":"https://doi.org/10.1111/bjh.19754"},{"label":"Lens 1997, Blood: p53 abnormalities in B-cell prolymphocytic leukaemia","url":"https://doi.org/10.1182/blood.v89.6.2015"},{"label":"J Cancer Res Ther 2024: splenic B-cell lymphoma/leukaemia with prominent nucleoli, three cases and review","url":"https://doi.org/10.4103/jcrt.jcrt_1931_22"}],"tags":["subtype-page","wave4","haematologic","rare"],"related":["leukaemia","cll","hairy-cell-leukemia","splenic-marginal-zone-lymphoma","mantle-cell-lymphoma","t-cell-prolymphocytic-leukaemia"],"cancers":[],"sections":[],"technologies":[],"targets":[],"drugs":["rituximab","bendamustine","ibrutinib","venetoclax"],"companies":[],"institutions":[],"pathways":[],"terms":["allogeneic-transplant"],"trials":[],"people":[],"bottlenecks":[],"keyPapers":[],"journals":[],"dependsOn":[],"notes":[],"group":"haematologic","burden":"Rare: B-PLL and hairy cell leukaemia variant diagnoses were uncommon and the new category is a holding ground for cases that fit neither the classic types nor CLL (British Journal of Haematology 2024). No registry figure exists for the new category.","subtypes":["Former CD5-negative B-cell prolymphocytic leukaemia","Former hairy cell leukaemia variant (CD25-negative, BRAF wild-type)","Splenic marginal zone or diffuse red pulp lymphoma with prominent nucleolated cells"],"biomarkers":["Large nucleolated B cells in blood and marrow; CD5 negative, CD25 negative or dim, BRAF V600E absent","TP53 mutation or 17p deletion (about half of former B-PLL)","Exclusion of cyclin D1 and SOX11 (mantle cell lymphoma) and of CLL immunophenotype","Splenomegaly with lymphocytosis"],"standardOfCare":[{"setting":"All cases","approach":"Rituximab with chemotherapy borrowed from hairy cell leukaemia variant practice; BTK inhibitors or venetoclax by analogy with TP53-disrupted CLL; allogeneic transplant for fit patients; no trial exists.","refs":["rituximab","bendamustine","ibrutinib","venetoclax","cll","hairy-cell-leukemia"]}],"stateOfArt":[],"history":[],"pipeline":[],"openProblems":[],"parent":"leukaemia"},"route":"/cancers/splenic-b-cell-lymphoma-leukaemia-prominent-nucleoli/","neighbours":{"cancer":[{"id":"cll","kind":"cancer","name":"Chronic lymphocytic leukaemia","route":"/cancers/cll/"},{"id":"hairy-cell-leukemia","kind":"cancer","name":"Hairy cell leukaemia","route":"/cancers/hairy-cell-leukemia/"},{"id":"leukaemia","kind":"cancer","name":"Leukaemia (all types)","route":"/cancers/leukaemia/"},{"id":"mantle-cell-lymphoma","kind":"cancer","name":"Mantle cell lymphoma","route":"/cancers/mantle-cell-lymphoma/"},{"id":"splenic-marginal-zone-lymphoma","kind":"cancer","name":"Splenic marginal zone lymphoma","route":"/cancers/splenic-marginal-zone-lymphoma/"},{"id":"t-cell-prolymphocytic-leukaemia","kind":"cancer","name":"T-cell prolymphocytic leukaemia","route":"/cancers/t-cell-prolymphocytic-leukaemia/"}],"drug":[{"id":"bendamustine","kind":"drug","name":"Bendamustine","route":"/drugs/bendamustine/"},{"id":"ibrutinib","kind":"drug","name":"Ibrutinib","route":"/drugs/ibrutinib/"},{"id":"rituximab","kind":"drug","name":"Rituximab","route":"/drugs/rituximab/"},{"id":"venetoclax","kind":"drug","name":"Venetoclax","route":"/drugs/venetoclax/"}],"term":[{"id":"allogeneic-transplant","kind":"term","name":"Allogeneic stem cell transplant (allo-SCT)","route":"/terms/allogeneic-transplant/"}]}}