Amiot Aurelien, Cazals-Hatem Dominique, Joly Francisca, Lavergne-Slove Anne, Peuchmaur Michel, Bouhnik Yoram, Bedossa Pierre, Messing Bernard
Pôle des Maladies de l'appareil Digestif, Unité de Gastroentérologie et d'assistance Nutritive, Hôpital Beaujon, Clichy, France.
Am J Surg Pathol. 2009 May;33(5):749-58. doi: 10.1097/PAS.0b013e31819b381a.
Chronic intestinal pseudoobstruction (CIPO) is classified into enteric visceral myopathies, neuropathies, and/or mesenchymopathies. Although the histology usually permits to highlight pathologic abnormalities of CIPO, it fails in almost a third of cases. The yield of a systematic immunohistochemistry needs to be evaluating.
Twenty-one adult patients with idiopathic CIPO [11 females/10 males, median age 23.1 (0.3 to 57) y] were included and compared with 27 control and 10 with mechanical obstruction patients. Comparison between standard histology (hematoxylin and eosin-stained sections) and systematic immunohistochemistry using muscular (smooth muscle alpha-actin, desmin, and smoothelin-A/B), nervous (Hu C/D, Bcl-2, and S100 protein), and mesenchymal (CD117) markers was carried out.
Histology showed neuromuscular abnormalities in 13 out of 21 (62%) patients, consisting of enteric visceral myopathy in 9 (43%) patients, enteric visceral neuropathy in 2 (9.5%), and mixed neuromyopathy in 2 (9.5%). Among the 8 patients who had no histologic structural abnormality, 6 patients (75%) had underlying abnormalities detected with immunohistochemistry: immunostain with Hu C/D detected a hypoganglionosis (<50 ganglion cells/cm) in 6 out of 21 (29%) patients, 4 of them undiagnosed on standard histology; CD117 (c-kit) detected a interstitial cells of Cajal defect in 10 out of 21 (48%) patients, 2 of them with no histologic structural abnormality. Smoothelin-A/B and desmin were useless as normally expressed in all patients with no myopathy; although it was not relevant in ileal samples (86% of abnormal expression in control patients), smooth muscle alpha-actin showed an abnormal expression in 2 CIPO patients (2/21).
Immunohistochemistry using Hu C/D and CD117 antibodies combined to the standard histology increased the yield of detection of neuromuscular abnormalities in idiopathic CIPO patients.
慢性肠道假性梗阻（CIPO）分为肠道内脏肌病、神经病和/或间充质病。虽然组织学检查通常能凸显CIPO的病理异常，但在近三分之一的病例中却无法做到。系统性免疫组化的检出率有待评估。
纳入21例成年特发性CIPO患者[11例女性/10例男性，中位年龄23.1（0.3至57）岁]，并与27例对照患者和10例机械性肠梗阻患者进行比较。对标准组织学（苏木精和伊红染色切片）与使用肌肉标志物（平滑肌α-肌动蛋白、结蛋白和平滑肌肌动蛋白A/B）、神经标志物（Hu C/D、Bcl-2和S100蛋白）以及间充质标志物（CD117）的系统性免疫组化进行比较。
组织学检查显示，21例患者中有13例（62%）存在神经肌肉异常，其中9例（43%）为肠道内脏肌病，2例（9.5%）为肠道内脏神经病，2例（9.5%）为混合性神经肌病。在8例无组织学结构异常的患者中，6例（75%）通过免疫组化检测出潜在异常：Hu C/D免疫染色在21例患者中的6例（29%）检测到神经节减少（<50个神经节细胞/cm），其中4例在标准组织学检查中未被诊断出来；CD117（c-kit）在21例患者中的10例（48%）检测到 Cajal间质细胞缺陷，其中2例无组织学结构异常。平滑肌肌动蛋白A/B和结蛋白在所有无肌病的患者中均正常表达，因此无用；虽然在回肠样本中不相关（对照患者中86%表达异常），但平滑肌α-肌动蛋白在2例CIPO患者（2/21）中显示异常表达。
将使用Hu C/D和CD117抗体的免疫组化与标准组织学相结合，提高了特发性CIPO患者神经肌肉异常的检出率。
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