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<p>Chronic lymphocytic leukemia/small lymphocytic lymphoma (CLL/SLL) is the most prevalent leukemia in adults which typically follows an indolent clinical course. However, it has the potential to transform into more aggressive categories, including accelerated CLL or diffuse large B-cell lymphoma (DLBCL), a phenomenon known as Richter transformation. Bruton tyrosine kinase inhibidors (BTKI), such as ibrutinib and zanubrutinib, represent a cornerstone of CLL/SLL treatment by inhibiting B-cell receptor signaling. Temporary discontinuation of these agents are common due to surgical procedures or infections which has been associated with rapid disease progression, producing a pseudo–Richter transformation (P-RT) in tissues. We present a case with CLL/SLL who experienced clinical deterioration following ibrutinib withdrawal, resulting in an explosive disease flare and splenic rupture. The patient improved after BTKI reintroduction, and a P-RT was diagnosed. Nine similar patients were found through PubMed search, and we describe their clinicopathological characteristics.</p>

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When pseudo–Richter transformation hits the spleen: a case report and literature review

  • 期刊论文

  • 出版模式：

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  • 发表日期：

    2026年6月2日

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  • Ricard Onieva,
  • Laura Rama,
  • Carmen Blázquez,
  • Rut Astorga,
  • Laura Escudero,
  • Maria Elena Ramila,
  • M. Carmen Ramos,
  • Natalia Papaleo

摘要

Chronic lymphocytic leukemia/small lymphocytic lymphoma (CLL/SLL) is the most prevalent leukemia in adults which typically follows an indolent clinical course. However, it has the potential to transform into more aggressive categories, including accelerated CLL or diffuse large B-cell lymphoma (DLBCL), a phenomenon known as Richter transformation. Bruton tyrosine kinase inhibidors (BTKI), such as ibrutinib and zanubrutinib, represent a cornerstone of CLL/SLL treatment by inhibiting B-cell receptor signaling. Temporary discontinuation of these agents are common due to surgical procedures or infections which has been associated with rapid disease progression, producing a pseudo–Richter transformation (P-RT) in tissues. We present a case with CLL/SLL who experienced clinical deterioration following ibrutinib withdrawal, resulting in an explosive disease flare and splenic rupture. The patient improved after BTKI reintroduction, and a P-RT was diagnosed. Nine similar patients were found through PubMed search, and we describe their clinicopathological characteristics.