Nesidioblastosis in sickle cell disease

Although the endocrine pancreas appears to play an important role in the pa thophysiology of sickle cell disease, very little is known about the morpho logic changes in this tissue. Our study was initiated to delineate the micr oscopic features of the endocrine pancreas in a large autopsy series of sic kle cell hemoglobinopathies. From more than 650 cases archived at the Centr alized Pathology Unit for Sickle findings and/or for microscopic studies, i ncluding histochemical stains (trichrome, reticulin, iron), and immunohisto chemical stains (insulin, glucagon, somatostatin, and pancreatic polypeptid e). The gross examinations were recorded as unremarkable in 65% of the auto psies. In childhood and adolescence (less than or equal to 18 years)- pancr eas weights (50.76 +/- 5.16SE gm) were significantly greater (p < 0.0001) t han age-matched controls (30.42 +/- 3.59SE gm). In adulthood, pancreas weig hts (108.34 +/- 5.29SE gm) were not significantly different from controls ( 110 gm). Microscopic findings included vascular congestion (48%), edema (65 %), siderosis (31%), and nesidioblastosis (76%), which included islet cell dispersion (53%), hyperplasia (23%), and hypertrophy (25%). Analysis by age groups suggested that islet cell dispersion/hyperplasia persists unchanged , whereas diameters of compact islets tend to increase with age. These find ings may be related to local tissue hypoxia and/or increased metabolic ener gy needs in sickle cell disease.