Ramón Enríquez J, Galván E, Uscanga L, Robles Díaz G
Dept de Gastroenterología Instituto Nacional de al Nutrición, Salvador Zubirán, México, D.F.
Rev Invest Clin. 1990 Oct-Dec;42(4):285-9.
Macroamylasemia (MA) is a rare condition characterized by an active macromolecular complex formed by normal amylase with abnormal proteins; to our knowledge, it has not been previously described in Mexico. The size of the macromolecular complex precludes its renal excretion; thus MA is characterized by high levels of amylase in serum with normal amylasuria. We report a 53-year-old male with abdominal pain and hyperamylasemia who was erroneously diagnosed as pancreatitis. Amylase in urine was normal and a protein electrophoresis demonstrated hyperglobulinemia. Several months after the initial work-up, the diagnosis of non-Hodgkin's lymphoma was established. Serum pancreatic amylase was again found elevated with normal urinary amylase. Precipitation of amylase with polyethylene-glycol was of 81% (normal: less than 70%). This established the diagnosis of MA associated to non-Hodgkin's lymphoma. After chemotherapy, the abnormal macroamylasemia and hyperglobulinemia disappeared.
巨淀粉酶血症(MA)是一种罕见病症,其特征是正常淀粉酶与异常蛋白质形成活性大分子复合物;据我们所知,此前墨西哥尚未有过相关描述。大分子复合物的大小使其无法经肾脏排泄;因此,MA的特点是血清淀粉酶水平升高而尿淀粉酶正常。我们报告一例53岁男性,有腹痛和高淀粉酶血症,曾被误诊为胰腺炎。尿淀粉酶正常,蛋白电泳显示高球蛋白血症。初始检查数月后,确诊为非霍奇金淋巴瘤。再次发现血清胰淀粉酶升高而尿淀粉酶正常。用聚乙二醇沉淀淀粉酶的比例为81%(正常:低于70%)。由此确诊为与非霍奇金淋巴瘤相关的MA。化疗后,异常的巨淀粉酶血症和高球蛋白血症消失。