An Atypical Presentation of Richter's Transformation in Chronic Lymphocytic Leukemia: Stroke-Like Onset With Multiorgan Involvement Including the Heart and Seminal Vesicles.

Chronic lymphocytic leukemia (CLL) is usually an indolent lymphoproliferative disorder. However, a small proportion of patients may develop Richter's transformation (RT), in which CLL transforms into an aggressive lymphoma. This transformation is often associated with rapid clinical progression and extranodal disease. Here, we describe a 72-year-old man diagnosed with CLL during evaluation for persistent cough and leukocytosis. Peripheral blood smear demonstrated smudge cells, and the diagnosis was confirmed by peripheral blood flow cytometry. As the patient was asymptomatic, he was managed with a watch-and-wait approach and regular follow-up. After nearly three years of observation, the patient presented with acute-onset slurring of speech and right-sided weakness, initially raising suspicion for a cerebrovascular event. Further imaging revealed multiple lesions involving the brain, lungs, stomach, and seminal vesicles, without lymph node involvement or hepatosplenomegaly. Fluorodeoxyglucose-positron emission tomography (FDG-PET) demonstrated widespread metabolically active disease. Histopathological examination of a CT-guided lung biopsy revealed high-grade B-cell lymphoma, raising suspicion for RT in the setting of known CLL. The patient was treated with R-CHOP chemotherapy, and follow-up imaging showed a favorable radiological response. This case highlights the atypical presentation of RT in patients with CLL who develop sudden clinical deterioration or unusual extranodal involvement, the role of tissue biopsy in establishing the diagnosis, and the challenges involved in its management.