Abstract: TH-PO809
Histopathological Description of Sickle Cell Nephropathy of the Arab-Indian Haplotype
Session Information
- Pathology and Lab Medicine - I
November 02, 2023 | Location: Exhibit Hall, Pennsylvania Convention Center
Abstract Time: 10:00 AM - 12:00 PM
Category: Pathology and Lab Medicine
- 1800 Pathology and Lab Medicine
Authors
- Alnasrallah, Basil, Qatif Central Hospital, Qatif, Eastern, Saudi Arabia
- Alfaraj, Shatha Abdulrazaq, Qatif Central Hospital, Qatif, Eastern, Saudi Arabia
- Alqawain, Abdullah, Qatif Central Hospital, Qatif, Eastern, Saudi Arabia
- Al Khuraidah, Zainab Abdullah, Qatif Central Hospital, Qatif, Eastern, Saudi Arabia
- Alabbad, Eman, Qatif Central Hospital, Qatif, Eastern, Saudi Arabia
- Aljishi, Mohammed, Qatif Central Hospital, Qatif, Eastern, Saudi Arabia
- Alnasser, Ahmad Ali, Qatif Central Hospital, Qatif, Eastern, Saudi Arabia
- Al Rubh, Jafar Mohammed, Qatif Central Hospital, Qatif, Eastern, Saudi Arabia
- Aljishi, Manaf, Saudi German Hospital Dammam, Dammam, Eastern, Saudi Arabia
- Alzayer, Husam, Saudi Arabia Ministry of Health, Riyadh, Saudi Arabia
Background
Sickle cell disorders are a group of autosomal recessive disorders characterized by the presence of the hemoglobin S (HbS) variant, which leads to sickling and hemolysis of the red blood cells. The inheritance of one variant gene, sickle cell trait (SCT), leads to a carrier state and milder form of the disease while having 2 genes leads to the more aggressive state of sickle cell disease (SCD). The main variants of HbS genes are the haplotypes present in the African race and the Arab-Indian (AI) haplotype, found in Saudi Arabia and central India. Sickle Cell Nephropathy (SCN) has been well characterized in the population of African descent. However, the histopathological changes of SCN have not been described in the patients of AI haplotype.
Methods
This was a single-center retrospective analysis of all adult patients with sickle cell disorders (SCD/SCT) who underwent a kidney biopsy from January 2012 until May 2023. Histological specimens were retrieved and examined by a research histopathologist. Clinical and biochemical data were collected and analyzed at the time of biopsy and last follow-up.
Results
22 kidney biopsies were identified, 5 of which were excluded due to active lupus nephritis leaving 17 biopsies (12 SCD and 5 SCT). The mean age was 44 +/- 10 years, 8 were females (47%). The main indications for biopsy were unexplained raised creatinine (4/17) and proteinuria (13/17). The median hemoglobin S in SCD and SCT was 81% and 26%, respectively. Changes suggestive of SCN were only observed in SCD; the main findings were tubular hemosiderosis (92%), global sclerosis (83%), glomerular hypertrophy (75%), sickled RBCs (58%), FSGS lesions (42%), and duplication of the glomerular basement membrane (33%). None of the findings from SCT biopsies were consistent with SCN changes (2 diabetic glomerulosclerosis, 1 chronic glomerulonephritis, 1 normal glomerular architecture, and 1 cortical necrosis).
Conclusion
In patients with Sickle cell disorder in Saudi Arabia, where the AI haplotype is predominant, the SCN histopathological changes were largely similar to the ones previously reported with the haplotypes in the African race. These changes were only present in SCD and are likely to reflect the nature of the repetitive damage.