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5 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.
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.
| Setting | Approach | Guideline |
|---|---|---|
| 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. | not mapped |