Intragraft vascular occlusive sickle crisis with early renal allograft loss in occult sickle cell trait

• Published in: Human pathology 2011-07, Vol.42 (7), p.1027-1033
• Main Authors: Kim, Lisa, MD, Garfinkel, Marc R., MD, Chang, Anthony, MD, Kadambi, Pradeep V., MD, Meehan, Shane M., MD
• Format: Article
• Language: English
• Subjects: Adult; African Americans; Anemias. Hemoglobinopathies; Biological and medical sciences; Diseases of red blood cells; Female; Graft Rejection - pathology; Hematologic and hematopoietic diseases; Humans; Investigative techniques, diagnostic techniques (general aspects); Kidney Transplantation - pathology; Medical sciences; Middle Aged; Mortality; Nephrectomy; Pathology; Pathology. Cytology. Biochemistry. Spectrometry. Miscellaneous investigative techniques; Renal allograft loss; Renal Artery - pathology; Sickle cell anemia; Sickle Cell Trait - complications; Sickle Cell Trait - pathology; Sickle trait; Transplants & implants; Vascular Diseases - complications; Vascular Diseases - pathology; Vascular occlusion; Veins & arteries
• ISSN: 0046-8177; 1532-8392
• DOI: 10.1016/j.humpath.2010.09.013

Summary Early renal allograft failure due to sickle cell trait is rare. We present clinical and pathologic findings in 2 cases of early renal allograft failure associated with renal vein thrombosis and extensive erythrocyte sickling. Hemoglobin AS was identified in retrospect. In case 1, a 41-year-old female recipient of a deceased donor renal transplant developed abdominal pain and acute allograft failure on day 16, necessitating immediate nephrectomy. In case 2, the transplanted kidney in a 58-year-old female recipient was noted to be mottled blue within minutes of reperfusion. At 24 hours, the patient was oliguric; and the graft was removed. Transplant nephrectomies had diffuse enlargement with diffuse, nonhemorrhagic, cortical, and medullary necrosis. Extensive sickle vascular occlusion was evident in renal vein branches; interlobar, interlobular, and arcuate veins; vasa recta; and peritubular capillaries. The renal arteries had sickle vascular occlusion in case 1. Glomeruli had only focal sickle vascular occlusion. The erythrocytes in sickle vascular occlusion had abundant cytoplasmic filaments by electron microscopy. Acute rejection was not identified in either case. Protein C and S levels, factor V Leiden, and lupus anticoagulant assays were within normal limits. Hemoglobin analysis revealed hemoglobin S of 21.8% and 25.6%, respectively. Renal allograft necrosis with intragraft sickle crisis, characterized by extensive vascular occlusive erythrocyte sickling and prominent renal vein thrombosis, was observed in 2 patients with sickle cell trait. Occult sickle cell trait may be a risk factor for early renal allograft loss.