Belghiti Hicham, Cazals-Hatem Dominique, Couvelard Anne, Guedj Nathalie, Bedossa Pierre
Service d'anatomie-pathologique, hôpital Beaujon, AP-HP, 92118 Clichy cedex, France.
Ann Pathol. 2009 Dec;29(6):468-74. doi: 10.1016/j.annpat.2009.09.013.
Mesenteric panniculitis (MP), sclerotic mesenteritis (SM) and mesenteric lipodystrophy (ML) are the three histopathological forms of a rare inflammatory mesenteric disease. The pathogenesis is unknown until now. Hypothesis of a dysimmune disorder is advocated. Our purpose was to find histological basis to assert this hypothesis.
All patients with a diagnosis of inflammatory mesenteric disease made in Beaujon hospital were selected. A histological study and a systematic immunostaining with IgG4, CD3 and CD8 antibodies were performed for each patient with paraffin block available. Dysimmune features were defined by: a plasma cells component >30 %, an eosinophilic polynuclears component >10 %, a rate TCD8/CD3 >30 % and IgG4+ plasma cells >30/high power field (x400). Cases were classified in 3 forms on the basis of the predominant component: MP (lymphoid/plasma cell infiltrate), SM (fibrosis), ML (fat necrosis).
Thirteen patients (7 men, 6 women) were selected, with a mean age of 57 years (21-80 years); a paraffin block was available for 10 patients. The clinical and histological presentations were heterogeneous: a mesenteric mass was the most frequent mode of revelation (6/13 patients) and all histological forms were present (3 MP, 5 SM and 5 ML). Nine patients had surgical biopsy, 2 had a trephine biopsy and 2 a resection. Standard histological analysis showed numerous eosinophils (> or =10 %) and plasma cells (>30 %) in respectively 3 (23 %) and 6 (46 %) of the 13 cases. The immunohistochemistry (performed in 10 patients) showed numerous TCD8+ in 80 % of the cases and numerous IgG4+ plasma cells in only 31 % of the cases (4 cases) (IgG4+ >30/x400).
Our results demonstrate that inflammatory mesenteric diseases are heterogeneous: high components of plasma cells, eosinophils and TCD8+ lymphocytes were found respectively in 46, 23 and 80 % of cases; eosinophils were observed exclusively in sclerotic forms. Only 31 % of cases encompass numerous IgG4+ plasma cells, suggesting a pathogenesis different than hyper-IgG4 diseases.
肠系膜脂膜炎（MP）、硬化性肠系膜炎（SM）和肠系膜脂肪营养不良（ML）是一种罕见的炎症性肠系膜疾病的三种组织病理学形式。其发病机制至今尚不明确。有人提出免疫失调的假说。我们的目的是寻找组织学依据来证实这一假说。
选取在博戎医院诊断为炎症性肠系膜疾病的所有患者。对每例有石蜡块的患者进行组织学研究以及用IgG4、CD3和CD8抗体进行系统免疫染色。免疫失调特征定义为：浆细胞成分>30%，嗜酸性多形核细胞成分>10%，TCD8/CD3比率>30%，且IgG4 +浆细胞>30/高倍视野（×400）。根据主要成分将病例分为3种形式：MP（淋巴样/浆细胞浸润）、SM（纤维化）、ML（脂肪坏死）。
选取了13例患者（7例男性，6例女性），平均年龄57岁（21 - 80岁）；10例患者有石蜡块。临床和组织学表现具有异质性：肠系膜肿块是最常见的表现形式（6/13例患者），且所有组织学形式均有出现（3例MP，5例SM和5例ML）。9例患者进行了手术活检，2例进行了环钻活检，2例进行了切除术。标准组织学分析显示，13例病例中分别有3例（23%）和6例（46%）有大量嗜酸性粒细胞（≥10%）和浆细胞（>30%）。免疫组织化学（在10例患者中进行）显示，80%的病例有大量TCD8 +，仅31%的病例（4例）有大量IgG4 +浆细胞（IgG4 +>30/×400）。
我们的结果表明，炎症性肠系膜疾病具有异质性：分别在46%、23%和80%的病例中发现了高比例的浆细胞、嗜酸性粒细胞和TCD8 +淋巴细胞；嗜酸性粒细胞仅在硬化形式中观察到。仅31%的病例有大量IgG4 +浆细胞，提示其发病机制与IgG4相关疾病不同。
