# Splenic B-cell lymphoma/leukaemia with prominent nucleoli (formerly B-cell prolymphocytic leukaemia and hairy cell leukaemia variant)

Source: https://onco.cc/cancers/splenic-b-cell-lymphoma-leukaemia-prominent-nucleoli/  
OnCo record `splenic-b-cell-lymphoma-leukaemia-prominent-nucleoli` (Cancer). Data CC BY-NC 4.0, attribute "Data from OnCo (onco.cc)"; commercial use needs a licence.

## TL;DR

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).

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.

## Fields

- Kind: Cancer
- Last checked: 2026-09-24
- Also known as: 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
- Tags: subtype-page; wave4; haematologic; rare
- 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

## Sections of this record

The page is a hub with ten sections in reading order; large sections have their own page. The same plan as JSON: https://onco.cc/api/v1/cancers/splenic-b-cell-lymphoma-leukaemia-prominent-nucleoli/sections.json

- Overview (on the hub): The TL;DR, the family this cancer belongs to, the organ, who gets it and what the state of the art is. https://onco.cc/cancers/splenic-b-cell-lymphoma-leukaemia-prominent-nucleoli/#overview
- What it is (on the hub): Anatomy, the subtypes and how they differ, how it is staged, and where advanced disease spreads. https://onco.cc/cancers/splenic-b-cell-lymphoma-leukaemia-prominent-nucleoli/#what-it-is [3 subtypes]
- Finding it (on the hub): How it shows itself, how it is confirmed, what screening exists, and the biomarkers clinicians test for. https://onco.cc/cancers/splenic-b-cell-lymphoma-leukaemia-prominent-nucleoli/#finding-it [4 biomarkers]
- Treating it (on the hub): The standard of care by setting, the medicines, surgery and radiotherapy named in it, and the regimens behind them. https://onco.cc/cancers/splenic-b-cell-lymphoma-leukaemia-prominent-nucleoli/#treating-it [1 setting, 1 decision with options]
- Evidence (on the hub): Trials recruiting now, the landmark trials, the key papers and what they mean, the latest literature, and the milestones year by year. https://onco.cc/cancers/splenic-b-cell-lymphoma-leukaemia-prominent-nucleoli/#evidence
- The science (on the hub): The molecular landscape: the targets and how often each appears, the pathways, the mechanics stages and the preclinical models. https://onco.cc/cancers/splenic-b-cell-lymphoma-leukaemia-prominent-nucleoli/#science [3 targets]
- Where you are (own page): Cases by country, the UK and NHS pathway and other country lenses, and the expert centres with trials on record. https://onco.cc/cancers/splenic-b-cell-lymphoma-leukaemia-prominent-nucleoli/where-you-are/
- Living with it (on the hub): The decisions you may face, the aids that walk through them, the warnings on record, the first sixty days and the questions to ask. https://onco.cc/cancers/splenic-b-cell-lymphoma-leukaemia-prominent-nucleoli/#living-with-it [8 questions, 6 red cards]
- What is coming (own page): Everything in development, the open problems and what is being done about them, the roadmaps, and what changed on this record. https://onco.cc/cancers/splenic-b-cell-lymphoma-leukaemia-prominent-nucleoli/coming/ [4 medicines]
- Data (own page): Every connected record, the notes, the JSON, Markdown and RDF twins, and where the record came from and when it was checked. https://onco.cc/cancers/splenic-b-cell-lymphoma-leukaemia-prominent-nucleoli/data/ [20 connected records]

## Standard of care

- All cases: 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. ([Rituximab](https://onco.cc/drugs/rituximab/), [Bendamustine](https://onco.cc/drugs/bendamustine/), [Ibrutinib](https://onco.cc/drugs/ibrutinib/), [Venetoclax](https://onco.cc/drugs/venetoclax/), [Chronic lymphocytic leukaemia](https://onco.cc/cancers/cll/), [Hairy cell leukaemia](https://onco.cc/cancers/hairy-cell-leukemia/))

## Sources

- Wikipedia: https://en.wikipedia.org/wiki/B-cell_prolymphocytic_leukemia
- NCI PDQ: chronic lymphocytic leukaemia treatment: https://www.cancer.gov/types/leukemia/patient/cll-treatment-pdq
- Alaggio 2022, Leukemia: the 5th edition WHO classification of haematolymphoid tumours, lymphoid neoplasms: https://doi.org/10.1038/s41375-022-01620-2
- British Journal of Haematology 2024: understanding splenic B-cell lymphoma/leukaemia with prominent nucleoli: https://doi.org/10.1111/bjh.19754
- Lens 1997, Blood: p53 abnormalities in B-cell prolymphocytic leukaemia: https://doi.org/10.1182/blood.v89.6.2015
- J Cancer Res Ther 2024: splenic B-cell lymphoma/leukaemia with prominent nucleoli, three cases and review: https://doi.org/10.4103/jcrt.jcrt_1931_22

## Connected records

- cancers: [Chronic lymphocytic leukaemia](https://onco.cc/cancers/cll/), [Hairy cell leukaemia](https://onco.cc/cancers/hairy-cell-leukemia/), [Leukaemia (all types)](https://onco.cc/cancers/leukaemia/), [Mantle cell lymphoma](https://onco.cc/cancers/mantle-cell-lymphoma/), [Splenic marginal zone lymphoma](https://onco.cc/cancers/splenic-marginal-zone-lymphoma/), [T-cell prolymphocytic leukaemia](https://onco.cc/cancers/t-cell-prolymphocytic-leukaemia/)
- drugs: [Bendamustine](https://onco.cc/drugs/bendamustine/), [Ibrutinib](https://onco.cc/drugs/ibrutinib/), [Rituximab](https://onco.cc/drugs/rituximab/), [Venetoclax](https://onco.cc/drugs/venetoclax/)
- terms: [Allogeneic stem cell transplant (allo-SCT)](https://onco.cc/terms/allogeneic-transplant/)

---
JSON: https://onco.cc/api/v1/entities/splenic-b-cell-lymphoma-leukaemia-prominent-nucleoli.json