Okumura Y, Tamba J, Shintani Y, Yoshioka U, Inoue H, Fujiyama Y, Bamba T
Second Department of Internal Medicine, Shiga University of Medical Science, Seta-Tukinowa, Otsu, Japan.
Pancreas. 1998 Mar;16(2):205-10. doi: 10.1097/00006676-199803000-00015.
A 38-year-old male patient who had been treated for Crohn's disease was found to have serum lipase activity that was persistently increased approximately 10-fold above the normal upper limit. He was diagnosed with chronic pancreatitis based on slightly elevated elastase-1 level and retrograde pancreatography showing slight dilatation of the main pancreatic duct. Therefore, the hyperlipasemia was thought to be due to pancreatitis. However, the serum amylase and trypsin was not increased at any time, and no serious findings suggestive of pancreatitis were detected on morphologic examination. Thus, there were discrepancies between the serum lipase activity and other laboratory and clinical findings. Exclusion chromatography of the patient's serum suggested macromolecular lipase, and further immunologic testing including affinity chromatography, enzyme-linked immunosorbent assay, and immunoprecipitation assay showed that serum lipase was bound to immunoglobulin Gkappa. Therefore, the hyperlipasemia was caused by immunoglobulin-linked lipase, termed "macrolipasemia." Macrolipasemia has rarely been reported, and this is the first reported case of macrolipasemia accompanied by Crohn's disease.
一名曾接受克罗恩病治疗的38岁男性患者,其血清脂肪酶活性持续升高,约为正常上限的10倍。基于弹性蛋白酶-1水平略有升高以及逆行胰胆管造影显示主胰管轻度扩张,他被诊断为慢性胰腺炎。因此,高脂血症被认为是由胰腺炎引起的。然而,血清淀粉酶和胰蛋白酶在任何时候均未升高,形态学检查也未发现提示胰腺炎的严重表现。因此,血清脂肪酶活性与其他实验室及临床检查结果之间存在差异。对患者血清进行排阻色谱分析提示存在大分子脂肪酶,进一步的免疫检测,包括亲和色谱分析、酶联免疫吸附测定和免疫沉淀测定显示血清脂肪酶与免疫球蛋白Gκ结合。因此,高脂血症是由免疫球蛋白相关脂肪酶引起的,称为“巨脂血症”。巨脂血症鲜有报道,这是首例报道的伴有克罗恩病的巨脂血症病例。