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胰腺癌背景下的冷凝集素病

Cold Agglutinin Disease in the Setting of Pancreatic Adenocarcinoma.

作者信息

Phan Hong, Motiani Vanita, Haque Ayema, Asghar Sarrah Ali, Patel Harshank, Khan Zafir Zohab Hussain, Bannon Susan

机构信息

Western Michigan University Homer Stryker M.D. School of Medicine, Kalamazoo, USA.

Department of Internal Medicine, Western Michigan University Homer Stryker M.D. School of Medicine, Kalamazoo, USA.

出版信息

Eur J Case Rep Intern Med. 2025 Aug 13;12(9):005587. doi: 10.12890/2025_005587. eCollection 2025.

Abstract

UNLABELLED

Autoimmune haemolytic anaemia (AIHA) is caused by antibody-mediated destruction of red blood cells. There are two broad categories of AIHA: warm and cold, both categorized by the thermal reactivity of the autoantibodies. Cold agglutinin disease (CAD) occurs at temperatures below normal body temperature and primarily involves IgM antibodies. CAD typically occurs secondary to other processes: lymphoproliferative disorders or infection, although it can be rarely idiopathic. We present a case of a 65-year-old male with no past medical history who initially presented with haematuria and proteinuria, along with thrombocytopenia and leukopenia. He reported an unintentional weight loss of more than 9 kg, constipation, and pale-greasy coloured stools. Computed tomography (CT) scan of the abdomen and pelvis revealed findings suspicious for primary pancreatic neoplasm with nodal metastases and early carcinomatosis, severe right hydronephrosis, and distended gallbladder. Direct antiglobulin test (DAT) screening was negative, but an antibody screen was positive. The patient had a positive cold agglutin screen and elevated CA 19-9, carcinoembryonic antigen (CEA), and cyclic citrullinated peptide (CCP) levels, tumour markers used typically for diagnosis of pancreatic cancer. The patient presented with symptoms and laboratory findings suggestive of a complex interplay between haemolytic anaemia and a suspected solid organ malignancy, specifically pancreatic cancer. His elevated tumour markers and positive antibody screen and cold agglutinin screen suggested the possibility of paraneoplastic syndrome secondary to his pancreatic cancer. However, his infectious and autoimmune panel findings further complicated the picture and underscored the multifactorial nature of his condition.

LEARNING POINTS

This case highlights a rare association between autoimmune haemolytic anaemia (AIHA) and pancreatic adenocarcinoma.While AIHA is a known paraneoplastic syndrome of hematologic malignancies, this case report suggests that it may also be a paraneoplastic syndrome for solid tumours.This case report highlights the difficulty of treating AIHA in the setting of comorbidities, suggesting that standard treatment guidelines may not be effective.

摘要

未标注

自身免疫性溶血性贫血(AIHA)是由抗体介导的红细胞破坏引起的。AIHA主要分为两大类:温抗体型和冷抗体型,两者均根据自身抗体的热反应性进行分类。冷凝集素病(CAD)发生在低于正常体温的情况下,主要涉及IgM抗体。CAD通常继发于其他疾病:淋巴增殖性疾病或感染,尽管很少为特发性。我们报告一例65岁男性,既往无病史,最初表现为血尿、蛋白尿,伴有血小板减少和白细胞减少。他自述体重意外减轻超过9kg,便秘,大便呈灰白色油腻状。腹部和盆腔计算机断层扫描(CT)显示可疑原发性胰腺肿瘤伴淋巴结转移和早期癌病、严重右肾积水和胆囊扩张。直接抗球蛋白试验(DAT)筛查为阴性,但抗体筛查为阳性。患者冷凝集素筛查阳性,CA 19-9、癌胚抗原(CEA)和环瓜氨酸肽(CCP)水平升高,这些肿瘤标志物通常用于胰腺癌的诊断。患者出现的症状和实验室检查结果提示溶血性贫血与疑似实体器官恶性肿瘤(特别是胰腺癌)之间存在复杂的相互作用。他升高的肿瘤标志物、阳性抗体筛查和冷凝集素筛查提示胰腺癌继发副肿瘤综合征的可能性。然而,他的感染和自身免疫检查结果使情况更加复杂,并强调了其病情的多因素性质。

学习要点

本病例突出了自身免疫性溶血性贫血(AIHA)与胰腺腺癌之间的罕见关联。虽然AIHA是血液系统恶性肿瘤已知的副肿瘤综合征,但本病例报告表明它也可能是实体肿瘤的副肿瘤综合征。本病例报告突出了在合并症情况下治疗AIHA的困难,提示标准治疗指南可能无效。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/0303/12416801/cd17d3e2f9cd/5587_Fig1.jpg

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