Rare Richter Transformation of Chronic Lymphocytic Lymphoma to Hodgkin Lymphoma

Patient: Female, 68-year-old Final Diagnosis: Richter transformation of CLL to HL Symptoms: Abdominal pain • ascites • diffuse lymphadenopathy • fever • hepatosplenomegaly • pleural effusion • weakness Medication: — Clinical Procedure: — Specialty: Forensic Medicine • Hematology • General and Internal Medicine • Oncology • Pathology OBJECTIVE: Rare disease BACKGROUND: Richter transformation (RT) is an uncommon clinicopathological condition referring to the development of aggressive lymphoma from chronic lymphocytic lymphoma/small lymphocytic lymphoma (CLL/SLL) and characterized by sudden clinical deterioration with marked multifocal lymphadenopathy. Transformation of CLL/SLL to diffuse large B-cell lymphoma is the most common (2–9%), but T-cell and Hodgkin transformation (HL) (0.4%) occur, although less frequently. CASE REPORT: A 68-year-old woman initially diagnosed with CLL/SLL presented to the hospital with fever, weakness, abdominal pain, and vomiting. The physical examination showed hepatosplenomegaly and extensive abdominal lymphadenopathy on radiological imaging. The laboratory work-up revealed pancytopenia and a markedly increased alkaline phosphatase. In the setting of extensive granulomatous hepatitis, the development of aggressive Hodgkin lymphoma Richter transformation with multi-organ involvement within a few months led to the patient’s sudden death. Autopsy findings led to the post-mortem diagnosis of Richter transformation of CLL. CONCLUSIONS: Here, we describe a rare case of Hodgkin lymphoma RT from progressive CLL, with transformation occurring at approximately 12 years after initial diagnosis, despite treatment. Our case report underscores the diagnostic challenges and pitfalls associated with the granulomatous presentation masking RT transformation of CLL to Hodgkin lymphoma. The purpose of this report is to raise suspicion for the clinicopathological signs of Richter transformation in the presence of an atypical granulomatous presentation.