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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.
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).
How 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.
How common: no figure for the new category; the constituent diagnoses were uncommon (British Journal of Haematology 2024).
Treatment: 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.
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.
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.
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: Extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue (MALT lymphoma), Splenic marginal zone lymphoma, Nodal marginal zone lymphoma, Primary cutaneous marginal zone lymphoma, Primary cutaneous follicle centre lymphoma, Sezary syndrome, Nodal T-follicular helper cell lymphoma, angioimmunoblastic type (angioimmunoblastic T-cell lymphoma), Hepatosplenic T-cell lymphoma, Intravascular large B-cell lymphoma, Lymphomatoid granulomatosis, T-cell prolymphocytic leukaemia, T-cell large granular lymphocytic leukaemia, Mixed-phenotype acute leukaemia, Myeloid leukaemia of Down syndrome, Burkitt leukaemia, 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, Plasma cell leukaemia, 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, Standard-risk B-cell acute lymphoblastic leukaemia in children, High-risk acute lymphoblastic leukaemia in children (high-risk B-ALL and T-ALL), Philadelphia chromosome-positive acute lymphoblastic leukaemia in children (Ph-positive ALL), Philadelphia chromosome-like acute lymphoblastic leukaemia (Ph-like or BCR::ABL1-like ALL), Infant acute lymphoblastic leukaemia (KMT2A-rearranged, under one year), Relapsed and refractory acute lymphoblastic leukaemia in children, Acute myeloid leukaemia in children, Erdheim-Chester disease, Rosai-Dorfman-Destombes disease, Single-system Langerhans cell histiocytosis (bone, skin or one other organ), Multisystem Langerhans cell histiocytosis (with or without risk-organ involvement), Indolent and smouldering systemic mastocytosis, Advanced systemic mastocytosis (aggressive SM, SM with an associated haematological neoplasm, mast cell leukaemia), Early-stage classical Hodgkin lymphoma (stage I to II), Advanced-stage classical Hodgkin lymphoma (stage III to IV), Nodular lymphocyte-predominant Hodgkin lymphoma (nodular lymphocyte-predominant B-cell lymphoma), Relapsed and refractory classical Hodgkin lymphoma
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.
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Query for this cancer: (TITLE:"Splenic B-cell lymphoma/leukaemia with prominent nucleoli" OR ABSTRACT:"Splenic B-cell lymphoma/leukaemia with prominent nucleoli" OR TITLE:"formerly B-cell prolymphocytic leukaemia and hairy cell leukaemia variant" OR ABSTRACT:"formerly B-cell prolymphocytic leukaemia and hairy cell leukaemia variant" OR TITLE:"SBLPN" OR ABSTRACT:"SBLPN" OR TITLE:"B-cell prolymphocytic leukaemia" OR ABSTRACT:"B-cell prolymphocytic leukaemia" OR TITLE:"B-cell prolymphocytic leukemia" OR ABSTRACT:"B-cell prolymphocytic leukemia" OR TITLE:"B-PLL" OR ABSTRACT:"B-PLL") AND (treatment OR therapy OR trial OR survival OR diagnosis). Results are unfiltered search hits about Splenic B-cell lymphoma/leukaemia with prominent nucleoli (formerly B-cell prolymphocytic leukaemia and hairy cell leukaemia variant), not a curated reading list.
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Bleeding that will not stop, black or bloody stools, or unexplained bruising when platelets are expected to be low.
Blood in stool or urine, vomiting blood, a bleed that will not stop, or a severe headache; fatal bleeding events have occurred and the labels advise considering the risk around surgery and with blood thinners.
Nausea, vomiting, muscle cramps, palpitations, seizures, confusion or passing much less urine in the first days of a new dose; the label requires hydration, anti-hyperuricaemic drugs and blood tests around each ramp-up step.
Avoid grapefruit and Seville oranges.
Take with a meal and water. Avoid grapefruit, Seville oranges and starfruit.
Possible QT prolongation. Check ECG and electrolytes; review other QT-prolonging drugs.
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