Korean J Med.  2016 Feb;90(2):163-168. 10.3904/kjm.2016.90.2.163.

Rare Presentation of Richter's Transformation to Diffuse Large B Cell Lymphoma: a Case Report

• Goo EJ
• Kim MK
• Kong EJ
• Koh SA
• Gu MJ
• Jung JY
• Hyun MS

• KMID: 2268877
• DOI: http://doi.org/10.3904/kjm.2016.90.2.163

Abstract

Richter's syndrome refers to the development of aggressive lymphoma in a patient with chronic lymphocytic leukemia (CLL). It occurs in about 2% to 10% of patients with CLL. The most frequent manifestation of Richter's syndrome is diffuse large B cell lymphoma (DLBCL). Extranodal involvement is rare but can occur. The prognosis of Richter's syndrome is very poor. We herein report a case of a rare presentation of Richter's syndrome. A 42-year-old man diagnosed with CLL 2 years previously developed nodules on the bilateral thighs and buttocks. A positron emission tomography (PET)-CT scan revealed high fluorodeoxyglucose uptake in multiple lymph nodes, skeletal muscles, and the myocardium. An ultrasonography-guided biopsy confirmed Richter's syndrome from CLL to DLBCL. The patient was treated with rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisone chemotherapy. After six cycles of chemotherapy, we performed a PET-CT scan that revealed a complete response. However, 3 months later, the syndrome recurred. The patient was undergoing salvage chemotherapy at the time of this writing.

Keyword

Chronic lymphocytic leukemia; Diffuse large B cell lymphoma

MeSH Terms

Adult
Biopsy
Buttocks
Cyclophosphamide
Doxorubicin
Drug Therapy
Humans
Leukemia, Lymphocytic, Chronic, B-Cell
Lymph Nodes
Lymphoma
Lymphoma, B-Cell*
Muscle, Skeletal
Myocardium
Positron-Emission Tomography
Prednisone
Prognosis
Thigh
Vincristine
Writing
Rituximab
Cyclophosphamide
Doxorubicin
Prednisone
Vincristine