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Nesidioblastosis in sickle cell disease
D E Culberson1, E A Manci, A K Shah
1Centralized Pathology Unit for Sickle Cell Disease, University of South Alabama, Mobile, Alabama, USA.
Pediatric Pathology & Molecular Medicine
|April 4, 2003
Summary
Sickle cell disease causes significant microscopic changes in the endocrine pancreas, including vascular congestion, edema, and nesidioblastosis. These pancreatic alterations may stem from tissue hypoxia and increased metabolic demands in sickle cell patients.
Area of Science:
- Endocrinology
- Hematology
- Pathology
Background:
- The endocrine pancreas's role in sickle cell disease pathophysiology is understudied.
- Morphological changes in the sickle cell disease pancreas are not well-characterized.
Purpose of the Study:
- To investigate the microscopic features of the endocrine pancreas in sickle cell hemoglobinopathies.
- To correlate pancreatic changes with disease pathophysiology.
Main Methods:
- Reviewed 224 autopsy cases of sickle cell disease.
- Performed gross examination, histochemical, and immunohistochemical staining.
- Analyzed pancreas weights and microscopic findings including vascular congestion, edema, siderosis, and nesidioblastosis.
Main Results:
- Pancreas weights were significantly greater in childhood/adolescence but not in adulthood compared to controls.
- Microscopic findings included vascular congestion (48%), edema (65%), siderosis (31%), and nesidioblastosis (76%).
- Nesidioblastosis comprised islet cell dispersion (53%), hyperplasia (23%), and hypertrophy (25%), with dispersion/hyperplasia persisting and islet diameters increasing with age.
Conclusions:
- Sickle cell disease induces significant microscopic changes in the endocrine pancreas.
- These changes, including nesidioblastosis and altered islet morphology, may be linked to tissue hypoxia and increased metabolic demands.
- Further research is needed to fully understand the implications for endocrine function in sickle cell disease.
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