Abstract
The patient, a 66-year-old female suffering from chronic renal failure due to Sjögren syndrome, had been undergoing hemodialysis since January, 1992. In July, 1994, she developed new symptoms including swelling of both parotid glands and neck lymph nodes, fever, photophobia and an abnormally high. serum amylase value of 1, 195IU/l. Isozyme analysis of the amylase by electrophoresis showed an abnormal pattern which was difficult to classify. Further examination by Superose 12 gel permeation HPLC, immunoprecipitation reaction and counter immunoelectrophoresis revealed that this amylase was a macroamylase resulting from bonding with Ig-Aλ. This bonding accounted for the high amylase value.
This case experienced recurring gastrointestinal bleeding and, despite repeated extensive examinations, the exact cause could not be determined. Lymph node histodiagnosis during exploratory laparotomy revealed non-Hodgkin type of malignant lymphoma.
The patient died, due to the complication of disseminated intravascular coagulation postoperatively.
The average serum amylase value is higher for hemodialysis patients than for healthy subjects and pancreatitis Can raise this value even higher. However, Since there are very few reports of macroamylasemia in hemodialysis patients and no reports of Sjögren syndrome complicated by macroamylasemia, this case appears to represent a rare disorder.
We have presented a brief discussion, based on the literature, concerning the occurence of macroamylasemla in this case.
