异基因干细胞移植后特发性难治性腹水依鲁替尼和诊断性肝活检的疗效。

IF 1.8 4区 医学 Q3 HEMATOLOGY
Shota Takahata, Kazuki Sakatoku, Masanori Shiohara, Yosuke Nakaya, Mirei Horiuchi, Kentaro Ido, Yosuke Makuuchi, Masatomo Kuno, Hiroshi Okamura, Mitsutaka Nishimoto, Yasuhiro Nakashima, Mika Nakamae, Masayuki Hino, Hirohisa Nakamae
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引用次数: 0

摘要

特发性门静脉高压相关难治性腹水(IRA)是异基因造血细胞移植(alloo - hct)后罕见但可能危及生命的并发症。其发病机制尚不清楚,最佳的诊断和治疗策略尚未建立。在此,我们报告一例58岁男性患者在接受同种异体hct后出现进行性腹水。虽然临床表现符合肝静脉闭塞性疾病/窦状静脉阻塞综合征(VOD/SOS)的诊断标准,但肝脏组织学未发现VOD/SOS的标志性特征,如窦扩张或中心静脉纤维化。相反,伴有淋巴细胞浸润和纤维化的门静脉病变提示亚临床慢性移植物抗宿主病(GVHD)样病理,通过排除支持IRA的诊断。使用白莫硫地尔治疗慢性GVHD是无效的。随后用依鲁替尼(一种布鲁顿酪氨酸激酶抑制剂)治疗,三天内腹水消退。这是用依鲁替尼成功治疗IRA的第二例报道,并强调了在移植后腹水的鉴别诊断中考虑依鲁替尼反应性免疫介导机制的重要性。鉴于IRA和VOD/SOS之间治疗策略的根本差异,我们的研究结果强调了在这种具有挑战性的临床情况下,需要准确的诊断,包括组织病理学评估,以指导有效的治疗和改善患者的预后。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Efficacy of ibrutinib and diagnostic liver biopsy for idiopathic refractory ascites after allogeneic stem cell transplantation.

Idiopathic portal hypertension-related refractory ascites (IRA) is a rare but potentially life-threatening complication following allogeneic hematopoietic cell transplantation (allo-HCT). Its pathogenesis remains unclear, and optimal diagnostic and therapeutic strategies have yet to be established. Here, we report a case of a 58-year-old man who developed progressive ascites after allo-HCT. Although the clinical findings met diagnostic criteria for hepatic veno-occlusive disease/sinusoidal obstruction syndrome (VOD/SOS), liver histology revealed no hallmark features of VOD/SOS, such as sinusoidal dilatation or central venous fibrosis. Instead, portal venopathy with lymphocytic infiltration and fibrosis suggested a subclinical chronic graft-versus-host disease (GVHD)-like pathology, supporting a diagnosis of IRA by exclusion. Treatment with belumosudil, one of the available treatment options for chronic GVHD, was ineffective. Subsequent treatment with ibrutinib, a Bruton's tyrosine kinase inhibitor, led to resolution of ascites within three days. This is the second reported case of successful treatment of IRA with ibrutinib, and highlights the importance of considering ibrutinib-responsive immune-mediated mechanisms in the differential diagnosis of post-transplant ascites. Given the fundamental differences in treatment strategies between IRA and VOD/SOS, our findings underscore the need for accurate diagnosis, including histopathological evaluation, to guide effective therapy and improve patient outcomes in this challenging clinical scenario.

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来源期刊
CiteScore
3.90
自引率
4.80%
发文量
223
审稿时长
6 months
期刊介绍: The International Journal of Hematology, the official journal of the Japanese Society of Hematology, has a long history of publishing leading research in hematology. The journal comprises articles that contribute to progress in research not only in basic hematology but also in clinical hematology, aiming to cover all aspects of this field, namely, erythrocytes, leukocytes and hematopoiesis, hemostasis, thrombosis and vascular biology, hematological malignancies, transplantation, and cell therapy. The expanded [Progress in Hematology] section integrates such relevant fields as the cell biology of stem cells and cancer cells, and clinical research in inflammation, cancer, and thrombosis. Reports on results of clinical trials are also included, thus contributing to the aim of fostering communication among researchers in the growing field of modern hematology. The journal provides the best of up-to-date information on modern hematology, presenting readers with high-impact, original work focusing on pivotal issues.
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