关键词: Richter transformation breast tumor chronic lymphocytic leukemia small lymphocytic lymphoma zanubrutinib

来  源:   DOI:10.1515/biol-2022-0889   PDF(Pubmed)

Abstract:
Richter transformation (RT) represents the development of intrusive lymphoma in individuals previously or concurrently diagnosed with chronic lymphocytic leukemia (CLL) and is characterized by lymph node enlargement. However, cases involving extra-nodal organ involvement as the first symptom are rare. There are no reports of RT with breast lesions as the first symptom. Nonspecific and atypical clinical manifestations represent key challenges in the accurate diagnosis and appropriate treatment of RT. This case report describes an elderly female patient who presented with breast lesions as the first RT symptom. The patient was admitted with a painless mass in the left breast. Examination revealed multiple lymphadenopathies and abnormally high white blood cell levels. The patient was diagnosed with CLL after hematological tests, assessments of bone marrow morphology, and tissue biopsy. Mammography and B-ultrasonography showed solid space-occupying lesions (BI-RADS category 5) in the left breast. Initially, the patient declined a breast biopsy and was therefore prescribed ibrupotinib treatment, which showed limited efficacy. A needle biopsy of the affected breast indicated the presence of diffuse large B-cell lymphoma. Based on auxiliary and pathological examinations and medical history, the final diagnosis was RT with breast involvement. Zanubrutinib with rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisone treatment provided initial control; however, the treatment strategy required adjustment because of the patient\'s fluctuating condition. The current status of the patient is marked as stable, showing an overall achievement of partial alleviation. The patient is in the process of receiving follow-up treatment. We also performed a comprehensive literature review on RT, with particular emphasis on its biological paradigm, prognosis implications, existing therapeutic approaches, and emerging directions in treatment modalities.
摘要:
Richter转化(RT)代表先前或同时诊断为慢性淋巴细胞白血病(CLL)的个体中侵入性淋巴瘤的发展,其特征是淋巴结肿大。然而,以结外器官受累为首发症状的病例很少见.没有以乳腺病变为首发症状的RT的报道。非特异性和非典型的临床表现是RT的准确诊断和适当治疗的关键挑战。此病例报告描述了一名老年女性患者,该患者以乳腺病变为首发症状。患者入院时左乳房有无痛肿块。检查发现多发性淋巴结病和异常高的白细胞水平。患者经血液学检查确诊为CLL,骨髓形态学评估,和组织活检.钼靶和B超显示左乳实性占位性病变(BI-RADS5类)。最初,患者拒绝乳腺活检,因此接受了伊布替尼治疗,显示出有限的疗效。受累乳房的穿刺活检表明存在弥漫性大B细胞淋巴瘤。根据辅助和病理检查和病史,最终诊断为RT伴乳腺受累.扎努布替尼联合利妥昔单抗,环磷酰胺,阿霉素,长春新碱,和泼尼松治疗提供初始控制;然而,由于患者病情的波动,治疗策略需要调整。患者的当前状态被标记为稳定,显示出部分缓解的总体成就。患者正在接受后续治疗。我们还对RT进行了全面的文献综述,特别强调它的生物学范式,预后影响,现有的治疗方法,以及治疗方式的新兴方向。
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