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[造血干细胞移植治疗的原发性纵隔生殖细胞肿瘤相关血液系统肿瘤]

[Hematological neoplasia associated with primary mediastinal germ-cell tumor treated with hematopoietic stem cell transplantation].

作者信息

Ajimi Chihiro, Suzuki Yuri, Horigome Akihisa, Tsuchida Yuko, Takasago Satoshi, Uryu Hideko, Yamanaka Junko, Shichino Hiroyuki

机构信息

National Center for Global Health and Medicine, Pediatrics.

出版信息

Rinsho Ketsueki. 2021;62(10):1482-1487. doi: 10.11406/rinketsu.62.1482.

DOI:10.11406/rinketsu.62.1482
PMID:34732620
Abstract

The occurrence of a primary mediastinal germ cell tumor and hematological neoplasia provides a poor prognosis that is known to be fatal at a median of 6 months after onset. We report the case of a 15-year-old male who was treated with chemotherapy and hematopoietic cell transplantation based on a report of a surviving case. At diagnosis, the patient had an unresectable mediastinal tumor with elevated alpha-fetoprotein and human chorionic gonadotropin levels and acute megakaryoblastic leukemia. We prioritized treatment with chemotherapy for the tumor owing to the oncological emergency. We then performed leukemia induction therapy and achieved complete remission. Although we used CDDP in combination with intensive therapy, the mediastinal tumor grew too large for it to be safely resected. We transplanted bone marrow from the patient's human leukocyte antigen-haploidentical sibling upon conditioning with busulfan-melphalan. After 44 days, the leukemia recurred in the patient's central nervous system. This was followed by various post-transplant complications, and the patient died of organ failure that was associated with infectious diseases. At necropsy, a poorly engrafted bone marrow was observed. The mediastinal tumor was primarily necrotic, although some immature teratoma components were observed. No leukemic precursor cells were detected. Residual mediastinal tumors may be associated with the recurrence of leukemias. We seek a treatment strategy that enables early tumor resection and high-dose chemotherapy. Further case studies are warranted along with the development of effective treatment methods.

摘要

原发性纵隔生殖细胞肿瘤与血液系统肿瘤同时发生时预后较差,已知发病后中位生存期为6个月,常导致死亡。我们报告一例15岁男性患者,根据一例存活病例的报道,对其进行了化疗和造血细胞移植治疗。诊断时,患者患有无法切除的纵隔肿瘤,甲胎蛋白和人绒毛膜促性腺激素水平升高,同时患有急性巨核细胞白血病。由于肿瘤急症,我们优先对肿瘤进行化疗。然后我们进行了白血病诱导治疗并实现了完全缓解。尽管我们将顺铂与强化治疗联合使用,但纵隔肿瘤长得太大,无法安全切除。在用白消安-美法仑预处理后,我们从患者的人类白细胞抗原半相合同胞那里移植了骨髓。44天后,患者中枢神经系统白血病复发。随后出现了各种移植后并发症,患者死于与传染病相关的器官衰竭。尸检时,观察到骨髓植入不良。纵隔肿瘤主要为坏死性,尽管观察到一些不成熟的畸胎瘤成分。未检测到白血病前体细胞。残留的纵隔肿瘤可能与白血病复发有关。我们寻求一种能够早期切除肿瘤并进行大剂量化疗的治疗策略。随着有效治疗方法的发展,有必要进行进一步的病例研究。

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