D'Addona Matteo, Giudice Valentina, Pezzullo Luca, Ciancia Giuseppe, Baldi Carlo, Gorrese Marisa, Bertolini Angela, Campana Annapaola, Fresolone Lucia, Manzo Paola, Zeppa Pio, Serio Bianca, Selleri Carmine
Hematology and Transplant Center, University Hospital "San Giovanni di Dio e Ruggi d'Aragona", 84131 Salerno, Italy.
Department of Medicine, Surgery and Dentistry, University of Salerno, 84081 Baronissi, Italy.
J Clin Med. 2022 Aug 10;11(16):4674. doi: 10.3390/jcm11164674.
Richter's syndrome represents the progression of chronic lymphocytic leukemia (CLL) to more aggressive diseases, most frequently diffuse large B-cell lymphoma, while Hodgkin's lymphoma (HL) and hairy cell leukemia (HCL) are rarely described. The first case involved a 67-year-old man with a diagnosis of a high-risk stage-II CLL treated with rituximab and ibrutinib, developed a HL nodular sclerosis variant after three months of therapy for CLL. After achieving a complete remission for HL and ibrutinib cessation because of drug-related cardiotoxicity, the patient relapsed after five months off-therapy and died due to disease progression after two cycles of brentuximab-vedotin. The second case involved an 83-year-old female with a diagnosis of stage-IV CLL treated with rituximab plus bendamustine who developed a HCL eight years later. Pentostatin was unsuccessfully employed as upfront HCL therapy, and the patient was then switched to rituximab while in remission for CLL. In conclusion, Richter's transformation risk rate might be higher in patients treated with novel targeted therapies, and multiparametric flow cytometry and lymph node biopsy at relapse could help in early identifying small clones. The treatment of predominant neoplasia is mandatory, and disease-specific drugs are administered; however, clinical efficacy might be lower in these patients.
里氏综合征代表慢性淋巴细胞白血病（CLL）进展为侵袭性更强的疾病，最常见的是弥漫性大B细胞淋巴瘤，而霍奇金淋巴瘤（HL）和毛细胞白血病（HCL）则很少见。首例病例为一名67岁男性，诊断为高危II期CLL，接受利妥昔单抗和伊布替尼治疗，在CLL治疗三个月后发展为HL结节硬化型。在HL达到完全缓解且因药物相关心脏毒性停用伊布替尼后，患者在停药五个月后复发，在接受两个周期的本妥昔单抗治疗后因疾病进展死亡。第二例病例为一名83岁女性，诊断为IV期CLL，接受利妥昔单抗加苯达莫司汀治疗，八年后发展为HCL。喷司他丁作为HCL的一线治疗未成功，患者在CLL缓解期改用利妥昔单抗。总之，接受新型靶向治疗的患者里氏转化风险率可能更高，复发时的多参数流式细胞术和淋巴结活检有助于早期识别小克隆。必须对主要肿瘤进行治疗，并给予针对特定疾病的药物；然而，这些患者的临床疗效可能较低。
