Trautwein C, Hermann E, Rambow A, Löhr H, Klose P, Gabbert H, Poralla T
Medizinische Klinik und Poliklinik, Universität Mainz.
Dtsch Med Wochenschr. 1990 Feb 2;115(5):174-8. doi: 10.1055/s-2008-1064988.
An 18-year-old boy, who had severe abdominal pain for 18 months associated with marked weight loss, was found to have a stenosed ileal sling on double-contrast radiology of the small intestine. At operation a plate-like tumour was extending from the pancreas to the aortic bifurcation. Histological examination of removed tissue revealed retractile mesenteritis (mesenteric panniculitis; liposclerotic mesenteritis). The symptoms regressed and the patient again gained weight under immunotherapy with 1 mg/kg of prednisone and 2 mg/kg of cyclophosphamide daily. Subsequently, under prednisone alone, there was a recurrence, which responded within five weeks to daily 60 mg prednisone and 125 mg cyclophosphamide. The patient remains symptom-free on 125 mg cyclophosphamide and 10 mg prednisone daily.
一名18岁男孩，严重腹痛18个月并伴有明显体重减轻，小肠双重对比造影显示有一段狭窄的回肠襻。手术中发现一个板状肿瘤从胰腺延伸至主动脉分叉处。切除组织的组织学检查显示为回缩性肠系膜脂膜炎（肠系膜脂膜炎；脂肪硬化性肠系膜脂膜炎）。症状消退，患者在每日使用1mg/kg泼尼松和2mg/kg环磷酰胺进行免疫治疗后体重再次增加。随后，仅使用泼尼松时病情复发，每日使用60mg泼尼松和125mg环磷酰胺治疗五周后病情得到缓解。患者目前每日服用125mg环磷酰胺和10mg泼尼松，无症状。
