Richter transformation: chronic lymphocytic leukemia to diffuse large B-cell lymphoma
Presentation
Started hematologic follow-up for CLL two years ago. New-onset cervical lymphadenopathy for the past several weeks. Lymph node biopsy confirmed Richter transformation to diffuse large cell lymphoma. The treating hematologist requested neck-chest-abdomen CT before commencing with R-CHOP therapy.
Patient Data
- parotid glands infiltrated by many lymph nodes, some enlarged
- prominent cervical lymphadenopathy in all stations, large conglomerate in right parapharyngeal space with hypodense foci, most probably necrosis
- enlarged left pharyngeal tonsil
- additional prominent lymphadenopathy: axillary, perihilar (liver), para-aortic, right external iliac chain, left inguinal
- lymphadenopathy: supraclavicular, mediastinal, right hilar (lung), in gastrohepatic ligament, hilar (liver), mesenteric
- two small oval subpleural nodules in RML, adjacent to horizontal fissure; possibly represent intraparenchymal lymph nodes
- spleen slightly enlarged, with numerous hypodense lesions, likely representing lymphoma
- left renal angiomyolipoma
Previous abdominal CT
Previous abdominal CT preceding current study by 1 year and 9 months (done for CLL follow-up), included for comparison.
Case Discussion
Started follow-up two years ago for a clinical and laboratory picture of chronic lymphocytic leukemia (CLL). Did not require a specific treatment.
Several months ago, hospitalized due to unexplained new-onset anemia, lymphocytosis was gone, transient aggravation of lymphadenopathy. Accepted workup was done, with the working assumption that the cause was an intercurrent viral infection. New cervical lymphadenopathy for the last few weeks.
Pathology report, cervical lymph node biopsy:
Macroscopic description: Segments of grey flexible tissue measuring 3X2.5X1.5 cm overall.
Microscopic description: Lymph node showing diffuse large B-cell lymphoma, non-germinal center type. Tumor cells were positive for CD20, MUM-1 and BcL-6, and negative for CD5, CD10, BcL-2, and CD23. The proliferation index (Ki-67 stain) is about 80%. C-myc stains about 30% of tumor cells. In addition, peripheral salivary gland tissue (non-involved) is present.
On the current CT study, the lymphadenopathy is far more striking than on the previous one and reflects the patient's diagnosis.
Richter transformation denotes development of aggressive lymphoid disease, usually high-grade non-Hodgkin lymphoma, in patients with CLL or small lymphocytic lymphoma.
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