title: Nesidioblastosis in sickle cell disease.
abstract: Although the endocrine pancreas appears to play an important role in the pathophysiology of sickle cell disease, very little is known about the morphologic changes in this tissue. Our study was initiated to delineate the microscopic features of the endocrine pancreas in a large autopsy series of sickle cell hemoglobinopathies. From more than 650 cases archived at the Centralized Pathology Unit for Sickle Cell Disease (Mobile, AL), 224 autopsy cases were identified for review of clinical and gross autopsy findings and/or for microscopic studies, including histochemical stains (trichrome, reticulin, iron), and immunohistochemical stains (insulin, glucagon, somatostatin, and pancreatic polypeptide). The gross examinations were recorded as unremarkable in 65% of the autopsies. In childhood and adolescence (< or = 18 years), pancreas weights (50.76 +/- 5.16SE gm) were significantly greater (p < 0.0001) than age-matched controls (30.42 +/- 3.59SE gm). In adulthood, pancreas weights (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 findings may be related to local tissue hypoxia and/or increased metabolic energy needs in sickle cell disease.
authors.affiliation: Centralized Pathology Unit for Sickle Cell Disease, University of South Alabama, Mobile, Alabama, USA.
authors.forename: D E
authors.initials: DE
authors.lastname: Culberson
authors.forename: E A
authors.initials: EA
authors.lastname: Manci
authors.forename: A K
authors.initials: AK
authors.lastname: Shah
authors.forename: J
authors.initials: J
authors.lastname: Haynes
authors.forename: S K
authors.initials: SK
authors.lastname: Ballas
authors.forename: C
authors.initials: C
authors.lastname: Pegelow
authors.forename: E
authors.initials: E
authors.lastname: Vichinsky
mesh_terms: D000293:Adolescent; D000328:Adult; D000367:Age Factors; D000368:Aged; D000755:Anemia, Sickle Cell / Q000150:complications* / Q000503:physiopathology; D015415:Biomarkers; D002452:Cell Count; D015687:Cell Hypoxia; D002648:Child; D002675:Child, Preschool; D005260:Female; D005355:Fibrosis; D005934:Glucagon / Q000032:analysis; D006801:Humans; D006965:Hyperplasia; D007223:Infant; D007328:Insulin / Q000032:analysis; D007501:Iron / Q000032:analysis; D008297:Male; D008875:Middle Aged; D009929:Organ Size; D010179:Pancreas / Q000737:chemistry / Q000473:pathology; D010182:Pancreatic Diseases / Q000209:etiology* / Q000378:metabolism / Q000473:pathology; D010191:Pancreatic Polypeptide / Q000032:analysis; D013004:Somatostatin / Q000032:analysis
chemical_list: D015415:Biomarkers; D007328:Insulin; D013004:Somatostatin; D010191:Pancreatic Polypeptide; D005934:Glucagon; D007501:Iron