Atypical Case of Chronic Lymphocytic Leukemia Treated With R-CHOP
Posted: Wednesday, July 1, 2020
A rare case of chronic lymphocytic leukemia (CLL)/small lymphocytic lymphoma with atypical immunophenotype and increased prolymphocytes was described in the American Journal of Case Reports. Although treatment with rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisolone (R-CHOP) appeared to be effective, Mohamed A. Yassin, PhD, of the National Center for Cancer Care and Research, Doha, Qatar, and colleagues noted the disease prognosis is still unclear.
In brief, Richter syndrome is characterized by the development of a secondary aggressive lymphoma. Most often, patients with CLL develop a diffuse large B-cell lymphoma. Such patients are frequently administered R-CHOP as first-line treatment.
“The high proliferation index (Ki67) and the increase of large cells are suggestive of aggressive progression of the disease; however, no frank features of Richter’s transformation were noted,” the investigators remarked. “To date, our patient has responded well to treatment with R-CHOP, with complete remission in the subcutaneous masses and on PET scan, but further follow-up is needed.”
A 71-year-old man presented with an increased white blood cell count, among other symptoms consistent with hematologic malignancies. He was subsequently diagnosed with atypical CLL with increased prolymphocytes and Binet stage C disease. He achieved complete remission with six cycles of a bendamustine/rituximab regimen. However, 6 months after treatment, the patient presented with several subcutaneous lumps on his abdomen, upper limbs, and back.
Based on the results of a histologic analysis, the soft tissue-masses appeared to be caused by an infiltration of the adipose tissue, with small lymphoid cells and lymphocytes exhibiting monocytoid characteristics. As a result of the unexpected disease progression and aggressive tumor histology, the investigators opted to administer six cycles of systemic R-CHOP chemotherapy. The patient subsequently experienced a complete remission of his subcutaneous masses, both clinically and radiologically.
Disclosure: The study authors reported no conflicts of interest.
