From the Mallinckrodt Institute of Radiology, Washington University School of Medicine, St Louis, Mo (M.I., V.M.M.); Department of Radiology, University of Louisville, Louisville, Ky (N.K.); Department of Radiology, VA Medical Center, Fayetteville, NC (A.R.); Department of Radiology, University of Wisconsin School of Medicine and Public Health, Madison, Wis (M.G.L.); Department of Radiology, Division of Abdominal Imaging, University of Pittsburgh Medical Center, Pittsburgh, Pa (A.K.D.); Department of Radiology, University of Texas Health at San Antonio, 7703 Floyd Curl Dr, San Antonio, TX 78229 (L.K., V.S.K.); and Department of Radiology, University of Texas MD Anderson Cancer Center, Houston, Tex (S.R.P.).
Radiographics. 2022 May-Jun;42(3):759-777. doi: 10.1148/rg.210169. Epub 2022 Apr 22.
There is a wide spectrum of hereditary and acquired immunodeficiency disorders that are characterized by specific abnormalities involving a plethora of humoral, cellular, and phagocytic immunologic pathways. These include distinctive primary immunodeficiency syndromes due to characteristic genetic defects and secondary immunodeficiency syndromes, such as AIDS from HIV infection and therapy-related immunosuppression in patients with cancers or a solid organ or stem cell transplant. The gut mucosa and gut-associated lymphoid tissue (the largest lymphoid organ in the body), along with diverse commensal microbiota, play complex and critical roles in development and modulation of the immune system. Thus, myriad gastrointestinal (GI) symptoms are common in immunocompromised patients and may be due to inflammatory conditions (graft versus host disease, neutropenic enterocolitis, or HIV-related proctocolitis), opportunistic infections (viral, bacterial, fungal, or protozoal), or malignancies (Kaposi sarcoma, lymphoma, posttransplant lymphoproliferative disorder, or anal cancer). GI tract involvement in immunodeficient patients contributes to significant morbidity and mortality. Along with endoscopy and histopathologic evaluation, imaging plays an integral role in detection, localization, characterization, and distinction of GI tract manifestations of various immunodeficiency syndromes and their complications. Select disorders demonstrate characteristic findings at fluoroscopy, CT, US, and MRI that permit timely and accurate diagnosis. While neutropenic enterocolitis affects the terminal ileum and right colon and occurs in patients receiving chemotherapy for hematologic malignancies, Kaposi sarcoma commonly manifests as bull's-eye lesions in the stomach and duodenum. Imaging is invaluable in treatment follow-up and long-term surveillance as well. RSNA, 2022.
遗传性和获得性免疫缺陷疾病谱广泛，其特征为涉及多种体液、细胞和吞噬免疫途径的特异性异常。这些疾病包括由于特征性遗传缺陷引起的独特原发性免疫缺陷综合征，以及继发性免疫缺陷综合征，如 HIV 感染引起的艾滋病和癌症或实体器官或干细胞移植患者的治疗相关免疫抑制。肠道黏膜和肠道相关淋巴组织（体内最大的淋巴器官）以及多样化的共生微生物群，在免疫系统的发育和调节中发挥着复杂而关键的作用。因此，免疫功能低下患者常出现多种胃肠道（GI）症状，这些症状可能是由于炎症性疾病（移植物抗宿主病、中性粒细胞减少性结肠炎或 HIV 相关性直肠结肠炎）、机会性感染（病毒、细菌、真菌或原生动物）或恶性肿瘤（卡波西肉瘤、淋巴瘤、移植后淋巴组织增生性疾病或肛门癌）引起的。免疫缺陷患者的胃肠道受累会导致严重的发病率和死亡率。除了内镜检查和组织病理学评估外，影像学在检测、定位、特征描述和鉴别各种免疫缺陷综合征及其并发症的胃肠道表现方面发挥着重要作用。某些疾病在透视、CT、US 和 MRI 上具有特征性表现，可实现及时准确的诊断。虽然中性粒细胞减少性结肠炎影响回肠末端和右结肠，发生于接受血液系统恶性肿瘤化疗的患者，但卡波西肉瘤通常表现为胃和十二指肠的靶心样病变。影像学在治疗随访和长期监测中也具有重要价值。RSNA，2022 年。
