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伪装成严重脓毒症和脓毒性休克的淋巴瘤相关噬血细胞综合征(LAHS)

Lymphoma-Associated Hemophagocytic Syndrome (LAHS) Masquerading as Severe Sepsis and Septic Shock.

作者信息

Ruelas Shea, Weerasinghe Isuri S, Woldeyohannes Samuel

机构信息

Internal Medicine, Baylor Scott & White Medical Center, Fort Worth, USA.

Neurology, University of Texas Southwestern Medical Center, Fort Worth, USA.

出版信息

Cureus. 2025 Jun 12;17(6):e85850. doi: 10.7759/cureus.85850. eCollection 2025 Jun.

Abstract

Our case involves a patient who presented with abdominal pain, nausea, and vomiting and was found to have lactic acidosis, acute liver dysfunction, and renal failure. An intra-abdominal infection was initially suspected, but the infectious workup was negative, and he did not respond to antimicrobial therapy. He rapidly deteriorated despite aggressive supportive care. Further workup revealed evidence that his symptoms were being driven by hemophagocytic lymphohistiocytosis (HLH) syndrome of an unclear cause. A liver biopsy was obtained; however, before the pathology results, the patient developed internal hemorrhage due to severe coagulopathy, and he was not able to be resuscitated despite aggressive treatment. The liver biopsy revealed T-cell lymphoma of the liver, most likely a hepatosplenic subtype of lymphoma, which is quite rare compared to the incidence of B-cell lymphomas. Lymphomas can present in a diverse range of presentations and can be accompanied by co-syndromes such as HLH. HLH can cause serious morbidity and mortality through overactivation of the immune system, affecting multiple organ systems. This case demonstrates the need for a broad differential when approaching a patient with multi-organ failure, as malignancy can be the inciting condition, and effective treatment may depend on swift diagnosis.

摘要

我们的病例涉及一名出现腹痛、恶心和呕吐症状的患者,检查发现其患有乳酸酸中毒、急性肝功能障碍和肾衰竭。最初怀疑是腹腔内感染,但感染相关检查结果为阴性,且患者对抗菌治疗无反应。尽管给予了积极的支持治疗,他的病情仍迅速恶化。进一步检查发现有证据表明其症状是由病因不明的噬血细胞性淋巴组织细胞增生症(HLH)综合征所致。进行了肝脏活检;然而,在病理结果出来之前,患者因严重凝血功能障碍发生了内出血,尽管进行了积极治疗仍未能复苏。肝脏活检显示为肝脏T细胞淋巴瘤,很可能是淋巴瘤的肝脾亚型,与B细胞淋巴瘤的发病率相比相当罕见。淋巴瘤可以有多种表现形式,并可能伴有HLH等综合征。HLH可通过免疫系统过度激活导致严重的发病率和死亡率,影响多个器官系统。该病例表明,在诊治多器官功能衰竭患者时需要进行广泛的鉴别诊断,因为恶性肿瘤可能是诱发因素,而有效治疗可能取决于快速诊断。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ef5a/12255274/cd58f8de9dc0/cureus-0017-00000085850-i01.jpg

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