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酷似克罗恩病的周期性中性粒细胞减少症:两例报告及文献综述

Cyclic Neutropenia Mimicking Crohn's Disease: Two Case Reports and a Narrative Review.

作者信息

Guarino Alessia Dalila, Luglio Gaetano, Imperatore Nicola, Cerciello Giuseppe, Pugliese Novella, Castiglione Fabiana, Tropeano Francesca Paola, Testa Anna, Olmo Oriana, Rispo Antonio

机构信息

Gastroenterology, Department of Clinical Medicine and Surgery, "Federico II" University, 80131 Naples, Italy.

Endoscopic Surgery, Department of Clinical Medicine and Surgery, "Federico II" University, 80131 Naples, Italy.

出版信息

J Clin Med. 2023 Sep 30;12(19):6323. doi: 10.3390/jcm12196323.

Abstract

Cyclic neutropenia is a rare hematological condition characterized by periodic fluctuations in neutrophil counts, with a 21-day periodicity. Clinical presentation varies from mild to severe forms of the disease, with the onset of recurrent fever, painful oral ulcers, recurrent bacterial infections, peritonitis, and septic shock. The availability of granulocyte colony-stimulating factor (G-CSF) has revolutionized the management and natural history of this disease, regulating the proliferation, differentiation, and maturation of the progenitor cells, and reducing the duration of neutropenia. Inflammatory bowel disease (IBD), including Crohn's disease (CD) and ulcerative colitis (UC), is a group of chronic pathologies that affect the gastrointestinal tract. The onset of both diseases may be at a young age (even during childhood or adolescence), and clinical manifestations may lead to misdiagnosis, due to similar characteristics such as recurrent infections, oral ulcers, perianal abscesses, and infertility. Moreover, the two pathologies are rarely associated, with different management and therapeutic options. Here, we describe two case reports of patients who underwent surgery because of diagnosis of complicated CD. After surgery, due to persistent neutropenia, the hematologist consultant confirmed suspicions of cyclic neutropenia, and G-CSF therapy was started with benefits, underlining the crucial importance of proper differential diagnosis.

摘要

周期性中性粒细胞减少症是一种罕见的血液系统疾病,其特征为中性粒细胞计数呈周期性波动,周期为21天。临床表现从疾病的轻度形式到重度形式不等,包括反复发热、疼痛性口腔溃疡、反复细菌感染、腹膜炎和感染性休克。粒细胞集落刺激因子(G-CSF)的出现彻底改变了这种疾病的管理和自然病程,它调节祖细胞的增殖、分化和成熟,并缩短中性粒细胞减少的持续时间。炎症性肠病(IBD),包括克罗恩病(CD)和溃疡性结肠炎(UC),是一组影响胃肠道的慢性疾病。这两种疾病都可能在年轻时发病(甚至在儿童期或青春期),并且由于反复感染、口腔溃疡、肛周脓肿和不孕等相似特征,其临床表现可能导致误诊。此外,这两种疾病很少相关,治疗方法和治疗选择也不同。在此,我们描述两例因诊断为复杂性CD而接受手术的患者病例报告。手术后,由于持续中性粒细胞减少,血液科会诊医生证实怀疑为周期性中性粒细胞减少症,并开始使用G-CSF治疗,效果良好,强调了正确鉴别诊断的至关重要性。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/a87e/10573598/c8df7d2debf1/jcm-12-06323-g001.jpg

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