ArticleFrontiers in oncology2024
Clonally unrelated HL-type RS manifested as hemophagocytic syndrome: a case report and literature review.
Article in Frontiers in oncology, 2024. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Not yet cited in PubMed.
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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.
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