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成人严重血小板减少症患者的横纹肌肉瘤:临床管理挑战与治疗并发症

Rhabdomyosarcoma in adults with severe thrombocytopenia: Challenges in clinical management and therapeutic complications.

作者信息

Estela-Zape Jose Luis, Sanclemente-Cardoza Valeria, Arzayus-Patiño Leonardo, Noreña-Buitrón Lizeth Dayana, Espinosa María Alejandra

机构信息

Faculty of Health, Universidad Santiago de Cali, Cali, Colombia.

Faculty of Health Sciences, Fundación Universitaria Maria Cano, Cali, Colombia.

出版信息

Respir Med Case Rep. 2025 Apr 17;55:102211. doi: 10.1016/j.rmcr.2025.102211. eCollection 2025.

DOI:10.1016/j.rmcr.2025.102211
PMID:40343148
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC12059698/
Abstract

Alveolar rhabdomyosarcoma (ARMS) in adults is a rare condition with a poor prognosis compared to other subtypes. The management of this pathology is complex due to the lack of standardized guidelines and the limited response to multimodal treatments, including chemotherapy, radiotherapy, and surgery. We report the case of a 40-year-old male with stage IV ARMS and pulmonary metastasis, who underwent resection of right supraclavicular sarcoma and cervical lymph node dissection. He was later referred for palliative chemotherapy following an acute clinical presentation lasting two days, characterized by right facial edema, inflammation, ulceration, and severe pain. Initial treatment included ampicillin/sulbactam, trimethoprim/sulfamethoxazole, and dexamethasone. Chemotherapy with doxorubicin and ifosfamide was delayed due to thrombocytopenia, elevated D-dimer and fibrinogen levels, and an echocardiographic finding that required differentiation between thrombus and metastatic lesion. Anticoagulation with fondaparinux and transfusions were initiated, and the patient received 10 sessions of radiotherapy. During hospitalization, the patient developed orthopnea, pleural effusion, superior vena cava syndrome, and hemodynamic deterioration, necessitating vasopressor support and mechanical ventilation. Despite these interventions, the patient progressed to refractory shock, severe hypoxemia, and died from cardiac arrest. This case highlights the challenges in managing ARMS in adults with severe thrombocytopenia.

摘要

成人肺泡横纹肌肉瘤(ARMS)是一种罕见疾病,与其他亚型相比预后较差。由于缺乏标准化指南以及对包括化疗、放疗和手术在内的多模式治疗反应有限,这种病理类型的管理较为复杂。我们报告了一例40岁男性IV期ARMS伴肺转移患者,该患者接受了右锁骨上肉瘤切除术和颈部淋巴结清扫术。在出现持续两天的急性临床表现后,其特征为右面部水肿、炎症、溃疡和剧痛,随后他被转诊接受姑息化疗。初始治疗包括氨苄西林/舒巴坦、甲氧苄啶/磺胺甲恶唑和地塞米松。由于血小板减少、D-二聚体和纤维蛋白原水平升高以及超声心动图检查发现需要鉴别血栓和转移灶,多柔比星和异环磷酰胺化疗被推迟。开始使用磺达肝癸钠抗凝和输血,患者接受了10次放疗。住院期间,患者出现端坐呼吸、胸腔积液、上腔静脉综合征和血流动力学恶化,需要血管活性药物支持和机械通气。尽管采取了这些干预措施,患者仍进展为难治性休克、严重低氧血症,最终死于心脏骤停。该病例突出了成人严重血小板减少的ARMS管理中的挑战。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/9d46/12059698/efd63f1a3986/gr3.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/9d46/12059698/e18ed967fbb2/gr1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/9d46/12059698/0ca0c4357945/gr2.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/9d46/12059698/efd63f1a3986/gr3.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/9d46/12059698/e18ed967fbb2/gr1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/9d46/12059698/0ca0c4357945/gr2.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/9d46/12059698/efd63f1a3986/gr3.jpg

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本文引用的文献

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Haematologica. 2024 Jun 1;109(6):1648-1650. doi: 10.3324/haematol.2023.284291.
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Clinicopathological features and prognosis of primary pulmonary rhabdomyosarcoma in middle-aged and elderly patients: a case report and literature review.中老年原发性肺横纹肌肉瘤的临床病理特征及预后:病例报告及文献复习。
J Int Med Res. 2023 Mar;51(3):3000605231159782. doi: 10.1177/03000605231159782.
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Alveolar rhabdomyosarcoma of epididymis: A case report.
附睾肺泡状横纹肌肉瘤:一例报告。
Front Oncol. 2022 Nov 2;12:1027504. doi: 10.3389/fonc.2022.1027504. eCollection 2022.
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All-cause mortality in cancer patients treated for sepsis in intensive care units: a systematic review and meta-analysis.重症监护病房中因败血症接受治疗的癌症患者的全因死亡率:系统评价和荟萃分析。
Support Care Cancer. 2022 Dec;30(12):10099-10109. doi: 10.1007/s00520-022-07392-w. Epub 2022 Oct 10.
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A Rare Occurrence of Relapse Malignant Vascular Mesenchymal Tumors (Rhabdomyosarcoma).复发性恶性血管间叶肿瘤(横纹肌肉瘤)的罕见病例。
Cureus. 2022 Jul 31;14(7):e27525. doi: 10.7759/cureus.27525. eCollection 2022 Jul.
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Understanding and Managing Sepsis in Patients With Cancer in the Era of Antimicrobial Resistance.在抗菌药物耐药时代理解和管理癌症患者的脓毒症
Front Med (Lausanne). 2021 Mar 31;8:636547. doi: 10.3389/fmed.2021.636547. eCollection 2021.
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