Transformation of Chronic Lymphocytic Leukemia/Small Lymphocytic Lymphoma to Histiocytic/Dendritic Cell Sarcoma.

IF 1.3 Q4 HEMATOLOGY Journal of hematology Pub Date : 2024-10-01 Epub Date: 2024-09-16 DOI:10.14740/jh1310
Jennifer Cai, David Fernandez-Hazoury, Gene Yoshikawa, Amani Minja, Hehua Huang, Andrew Hwang, Xin Qing
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Abstract

Chronic lymphocytic leukemia/small lymphocytic lymphoma (CLL/SLL) transforming into a more aggressive lymphoma (i.e., Richter syndrome) is well documented in the literature. In recent years, transdifferentiation of CLL/SLL to histiocytic/dendritic/Langerhans cell sarcomas has also been reported. We hereby describe a case of a 55-year-old female who was incidentally diagnosed with CLL after presenting to the hospital for symptoms of undiagnosed rheumatoid arthritis. At the time of presentation, CLL was stage 1, and the patient was placed on observation. Eight years after being diagnosed with CLL, and after several treatment modalities for her rheumatoid arthritis, the patient re-presented with progression of adenopathy, intermittent fevers, 5-pound weight loss, and worsening respiratory status requiring airway management. Computed tomography (CT) imaging revealed a soft tissue mass in the nasopharynx, lingual tonsillar hypertrophy with airway compromise, and bulky cervical, supraclavicular, and axillary lymphadenopathy. A biopsy of an enlarged cervical lymph node yielded a diagnosis of histiocytic/dendritic cell sarcoma favoring interdigitating dendritic cell sarcoma, likely representing transdifferentiation from CLL/SLL, of which there are no standard of care treatment guidelines. The patient was treated with ifosfamide, carboplatin, and etoposide (ICE) for three cycles, followed by rituximab, etoposide, prednisone, vincristine, cyclophosphamide, and doxorubicin (R-EPOCH) in combination with zanubrutinib. She then underwent haploidentical hematopoietic stem cell transplantation. At the time of the making of this manuscript, the patient was 45 days post-transplant without any notable complications.

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慢性淋巴细胞白血病/小淋巴细胞淋巴瘤向组织细胞/树突状细胞肉瘤转化。
慢性淋巴细胞白血病/小淋巴细胞淋巴瘤(CLL/SLL)转化为侵袭性更强的淋巴瘤(即里氏综合征)的文献记载很多。近年来,CLL/SLL 向组织细胞/树突状细胞/朗格汉斯细胞肉瘤的转化也有报道。我们在此描述了一例 55 岁女性的病例,她因未确诊的类风湿性关节炎症状到医院就诊,被偶然诊断为 CLL。就诊时,CLL 为 1 期,患者被安排接受观察。确诊为 CLL 八年后,在对其类风湿性关节炎进行了多次治疗后,患者再次就诊时出现腺病进展、间歇性发热、体重下降 5 磅,呼吸状况恶化,需要进行气道管理。计算机断层扫描(CT)成像显示鼻咽部有软组织肿块,舌扁桃体肥大,气道受损,颈部、锁骨上和腋窝淋巴结肿大。对肿大的颈淋巴结进行活检后,诊断结果为组织细胞/树突状细胞肉瘤,倾向于间变性树突状细胞肉瘤,很可能是从 CLL/SLL 转移而来,目前尚无标准治疗指南。患者接受了伊佛酰胺、卡铂和依托泊苷(ICE)三个周期的治疗,随后接受了利妥昔单抗、依托泊苷、泼尼松、长春新碱、环磷酰胺和多柔比星(R-EPOCH)联合扎努替尼的治疗。随后,她接受了单倍体造血干细胞移植。在撰写本手稿时,患者已移植45天,没有出现任何明显的并发症。
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来源期刊
Journal of hematology
Journal of hematology HEMATOLOGY-
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