CD5 negative leukemic mantle cell lymphoma with prolymphocyte morphology

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CD5 negative leukemic mantle cell lymphoma with prolymphocyte morphology

#00065571

Author: Ke Xu, MD
Category: Lymphoma: Mature B-cell and Plasma cell Neoplasms > Mantle cell Lymphoma
Published Date: 03/28/2025

A 70-year-old man presented with asymptomatic lymphocytosis and splenomegaly. Hb 75 g/L, WCC 354 x10⁹/L, lymphocytes 294 x10⁹/L, platelet 133 x 10⁹/L. The blood film showed many large prolymphocytes. Flow cytometry showed 90% CD19+CD5-CD10-CD20+CD23-CD79b+CD22+FMC7- kappa-restricted B lymphocytes. Target FISH showed IGH::CCND1 fusion and TP53 loss. Molecular karyotyping identified complex karyotypes and chromoanagenesis events. Lymphoid NGS detected CCND1 p.Glu36Gly (VAF 47%) and TP53 p.Asp184ValfsTer63 (VAF 90%) variants. PET/CT showed avid splenomegaly and minimal lymphadenopathy. He was diagnosed with mantle cell lymphoma and treated with acalabrutinib, with a good response.