Insights from a patient with chronic lymphocytic leukemia complicating ALK+ anaplastic large cell lymphoma

• Published in: Intractable & Rare Diseases Research Vol. 11; no. 4; pp. 196 - 201
• Main Authors: Lin, Wuqiang, Chen, Xiuli, Cai, Zhenjie, Zheng, Heyong, Huang, Hanxing, Yang, Huanxing, Hu, Jianda, Zheng, Jing, Asakawa, Tetsuya
• Format: Journal Article
• Language: English
• Published: International Research and Cooperation Association for Bio & Socio-Sciences Advancement 01.11.2022
• Subjects: ALK; anaplastic large cell lymphoma; chronic lymphocytic leukemia; Communication; fludarabine; Richter syndrome
• ISSN: 2186-3644; 2186-361X
• DOI: 10.5582/irdr.2022.01086

Chronic lymphocytic leukemia (CLL) that transforms into a more aggressive lymphoma has been termed Richter syndrome (RS). CLL with T-cell neoplasia is rarely reported; those with ALK+ anaplastic large cell lymphoma (ALCL) are also exceedingly rarely reported. A 63-year-old woman from the south of China presented with generalized lymphadenectasis and fever; she already had a prior diagnosis of CLL 9 years ago. As per her current diagnosis, it was CLL with ALK+ ALCL. The two-lymph node and bone marrow biopsies presented two types of cellular groups: i) left cervical lymph node biopsy suggested CLL (Ki67: 10%), along with bone marrow biopsy exhibited enhancement of the small lymphocytes (30%) with scant cytoplasm, round or irregular cell nuclei, and massive amounts of chromatin. Large cells (< 1%) that expressed CD30 and ALK were visible; The results of immunohistochemistry were as follows: CD20 (weak positive); PAX5 (positive); CD23 and CD5 (weak positive); and CD3, CD10, and CyclinD1 (negative); ii) left supraclavicular lymph node biopsy suggested ALK+ ALCL (Ki67: 70%). The final diagnosis was CLL with ALCL. The mechanisms of this condition are not fully understood, which might be associated with chronic stimulation of T cells by CLL cells along with immune dysfunction.