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Fatal sclerosing peritonitis associated with primary effusion lymphoma after liver transplantation: a case report.

机译:肝移植后伴发原发性渗出性淋巴瘤的致命性硬化性腹膜炎:一例报告。

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摘要

Sclerosing peritonitis (SP) after liver transplantation has been described in 10 cases in the literature. The etiology is still unknown; however, SP is considered a consequence of chronic irritation and inflammation. It can be classified as primary (idiopathic) or secondary form. Although pathologically benign, it has a negative course, resulting in unrelenting abdominal pain, small bowel obstruction, malnutrition, and death. Posttransplantation lymphoproliferative disease (PTLD) is one of the leading causes of late death. Its development is related to complex interactions between immunosuppressive drugs and environmental agents. Primary effusion lymphoma (PEL) as an onset presentation of PTLD is relatively uncommon. Most examples of effusion-based PTLD have been secondary to widespread solid organ involvement and associated with Human herpes virus 8 (HHV-8) recurrence. Here in, we report a case of a 55-year-old man who rapidly developed refractory ascites and bacterial peritonitis at 1-year after orthotopic liver transplantation (OLT) with a fatal clinical course at the beginning of the second follow-up year after an uncomplicated liver transplantation due to cryptogenic cirrhosis. The diagnosis of HHV-8-positive lymphoma was established by postmortem examination with multiple solid localizations and massive dense fibrotic adhesions encompassing the small intestine, colon, liver, and porta hepatis without any involvement of body cavities.
机译:已有10例文献报道了肝移植后的硬化性腹膜炎(SP)。病因尚不明确。但是,SP被认为是慢性刺激和炎症的结果。它可以分为主要形式(特发性)或次要形式。尽管在病理上是良性的,但其病程为阴性,导致持续的腹痛,小肠梗阻,营养不良和死亡。移植后淋巴细胞增生性疾病(PTLD)是晚期死亡的主要原因之一。它的发展与免疫抑制药物和环境药物之间的复杂相互作用有关。原发性渗出性淋巴瘤(PEL)作为PTLD的发作形式相对较少见。基于积液的PTLD的大多数实例是继发于广泛的实体器官受累并与人类疱疹病毒8(HHV-8)复发有关。在此,我们报道一例55岁的男性,该患者在原位肝移植(OLT)后1年迅速发展为难治性腹水和细菌性腹膜炎,并在术后第二个随访年开始时出现致命的临床病程由于隐源性肝硬化而进行的简单肝移植。通过死后检查确定HHV-8阳性淋巴瘤的诊断,该检查具有多个实体定位和大量密集的纤维化粘连,包括小肠,结肠,肝和肝门,而无任何体腔参与。

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