Evidence map›Paper›PMID 42156574›Full record

ArticleAnnals of hematology2026

Pseudo-Richter transformation in a patient with chronic lymphocytic leukemia after cessation of fixed duration venetoclax/ibrutinib treatment.

Raphael Battegay, Dominik Heim, Stefan Dirnhofer

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In one paragraph

Article in Annals of hematology, 2026. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Not yet cited in PubMed.

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1 · What the graph read from it

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The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.

2 · The registry

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3 · Its place in the literature

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4 · The record

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5 · Who and what money

Authors and funding

3 authors.

Raphael BattegayDepartment of hematology, University Hospital Basel, Basel, Switzerland. Raphael.battegay@usb.ch.
Dominik HeimDepartment of hematology, University Hospital Basel, Basel, Switzerland.
Stefan DirnhoferInstitute of Medical Genetics and Pathology, University Hospital Basel, Basel, Switzerland.

Funding

No grant is acknowledged in the PubMed record.

6 · The paper itself

Abstract

Pseudo-Richter transformation (Pseudo-RT) in chronic lymphocytic leukemia (CLL) refers to rapid clinical progression resembling Richter transformation (RT), DLBCL - type. It presents with systemic symptoms and lymphadenopathy. Distinguishing Pseudo-Richter from true Richter transformation is challenging but clinically essential due to differences in prognosis and treatment approaches. A 69-year-old Caucasian man was diagnosed with symptomatic CLL. Treatment was initiated with ibrutinib (cycles 1-15) and venetoclax (cycles 4-15). The patient responded well, with clinical and hematological complete response. One week after the last cycle was completed, the patient developed fever, abdominal pain, and recurrent lymphadenopathy. Imaging revealed partial regression and new progression of lymph nodes, splenic infarction, and an eventual splenic rupture requiring emergency splenectomy. Histology showed a diffuse infiltrate of large cells and paraimmunoblasts with high proliferation index (~ 50%), raising suspicion of RT. However, molecular testing was negative for typical findings of high-grade lymphoma and PET-CT did not support it as well. Suspecting pseudo-RT ibrutinib was reintroduced 14 days after cessation, leading to rapid normalization of leukocyte counts and clinical improvement. We report a case of pseudo-RT in a CLL patient treated with ibrutinib/venetoclax combination therapy, contrasting prior reports involving Bruton tyrosine kinase inhibitor (BTKi) monotherapy. The rapid onset of transformation-like symptoms following recent BTKi interruption/cessation should raise suspicion for pseudo-RT, though true RT must be excluded. As BTKi use in CLL increases, this phenomenon may become more common. Prompt interdisciplinary collaboration is essential for accurate diagnosis and appropriate management. Further research is needed to develop rapid diagnostic tools to distinguish pseudo-RT from true RT.

Indexed as

AdenineAntineoplastic Combined Chemotherapy ProtocolsBridged Bicyclo Compounds, HeterocyclicLeukemia, Lymphocytic, Chronic, B-CellSulfonamidesAgedHumansMalePiperidinesPyrazolesPyrimidinesTreatment InterruptionAdenineBridged Bicyclo Compounds, HeterocyclicibrutinibPiperidinesPyrazolesPyrimidinesSulfonamidesvenetoclaxBruton Tyrosine kinase inhibitorChronic lymphatic leukemiaIbrutinibPseudo-Richter transformationVenetoclax

Identifiers

PMID42156574
PMCPMC13357450

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Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.