Clonally unrelated HL-type RS manifested as hemophagocytic syndrome: a case report and literature review

Xiaoman Zheng; Wenshuang Ding; Zhigang Zhu; Junping Li; Weijie Zhong

doi:10.3389/fonc.2024.1472560

Clonally unrelated HL-type RS manifested as hemophagocytic syndrome: a case report and literature review

Front Oncol. 2024 Dec 5:14:1472560. doi: 10.3389/fonc.2024.1472560. eCollection 2024.

Authors

Xiaoman Zheng¹, Wenshuang Ding², Zhigang Zhu¹, Junping Li¹, Weijie Zhong¹

Affiliations

¹ Department of Geriatrics, Hematology & Oncology Ward, Guangzhou First People's Hospital, Guangdong Medical University, Guangzhou, Guangdong, China.
² Department of Pathology, Guangzhou First People's Hospital, Guangdong Medical University, Guangzhou, Guangdong, China.

Abstract

Hodgkin lymphoma variant of Richter syndrome (HL-type RS) is a very rare disease, in which chronic lymphocytic leukemia (CLL) or small lymphocytic lymphoma (SLL) is transformed into novel Hodgkin lymphoma. The most important prognostic factor of HL-type RS is the clonal relationship between HL-type RS and the preexisting CLL/SLL. Detailed confirmation of clonally unrelated HL-type RS cases have not been reported. To the best of our knowledge, this is the first case of HL-type RS confirmed as clone independent by a detailed comparison of immunoglobulin gene rearrangement clones and gene mutations. A 76-year-old man, diagnosed with SLL 1 year before transformation was treated with Zanubrutinib 3 months before transformation. When diagnosed with HL-type RS, he presented with symptoms of hemophagocytic syndrome. Positive therapeutic effects were achieved using a modified rituximab-doxorubicin, bleomycin, vinblastine, dacarbazine regimen in combination with Zanubrutinib. We also discuss a thorough review of the relevant literature we performed to help us better understand this rare disease.

Keywords: Hodgkin lymphoma variant of Richter syndrome; chronic lymphocytic leukemia; clonal relationship; hemophagocytic syndrome; small lymphocytic lymphoma.

Publication types

Case Reports

Grants and funding

The author(s) declare financial support was received for the research, authorship, and/or publication of this article. This work was supported by the Guangzhou Planned Project of Science and Technology, China (2023A03J0963).