Abstract Introduction Sickle cell disease (SCD) is an inherited hemoglobin disorder characterized by polymerization of deoxygenated hemoglobin, leading to sickle shaped red blood cells, multiorgan damage, and increased mortality. ¹ Hemorrhagic complications, particularly in the absence of trauma, are extremely rare and have been reported mostly as intracranial hemorrhages. 2, 3 We present a rare case of spontaneous perinephric hemorrhage in a patient with SCD. Case Presentation A 29-year-old African American male with SCD, not under regular hematology follow-up, presented with worsening spontaneous left abdominal and flank pain different from sickle cell pain unrelieved by over-the-counter analgesics for one day. He denied trauma, falls, anticoagulant use, or symptoms of infection. Laboratory evaluation showed hemoglobin 6. 8 g/dL, platelets 142 K/µL, WBC 22 K/µL, lactate 5. 1 mmol/L, CO2 14 mmol/L. CT abdomen and pelvis revealed a large left perinephric hemorrhage measuring 19. 5 × 11. 2 × 4. 9 cm with active extravasation. Interventional radiology was consulted. The patient was transfused packed red blood cells (PRBCs) with the goal to maintain hemoglobin above 8 g/dL. He remained hemodynamically stable, however, despite two units of irradiated PRBCs hemoglobin did not improve. Repeat CT demonstrated increased arterial bleeding from the left kidney mid cortex with hematoma expansion. He subsequently underwent successful left perinephric artery embolization with a liquid embolic agent. Post-procedure, his pain improved, hemoglobin stabilized at 8. 4 g/dL, and infectious workup was negative. Laboratory findings were consistent with sickle cell crisis with low haptoglobin and high LDH. He was managed with intravenous hydration, opioids, and hydroxyurea. Discussion SCD, complications typically arise from Vaso-occlusion rather than bleeding, hemorrhagic manifestations involve gastrointestinal, intracranial, or genitourinary sites, with renal complications such as papillary necrosis leading to hematuria. Perinephric hemorrhage is not recognized as a common or well-described complication of sickle cell disease. It most often results from renal tumors, vascular abnormalities, trauma, inflammatory disorders, or, rarely, anticoagulant use. 4 Spontaneous perinephric hematoma in SCD is therefore exceedingly rare and requires a high index of suspicion for prompt diagnosis and management. References: 1. Kavanagh PL, et al. Sickle Cell Disease: A Review. JAMA. 2022;328 (1): 57. doi: 10. 1001/jama. 2022. 102332. Chipongo H, et al. Massive Spontaneous Subdural Hemorrhage in a Sickle Cell Adolescent. Int J Surg Case Rep. 2024;115: 109255. doi: 10. 1016/j. ijscr. 2024. 1092553. Kamath SD, Pai MG. Hemorrhagic Neurological Complications of Sickle Cell Disease: A Case Series. Asian J Transfus Sci. 2021;15 (2): 241-246. doi: 10. 4103/ajts. AJTS₁01₁84. Zuckerman E, et al. Spontaneous Perinephric Hemorrhage in a Middle-Aged Diabetic Woman. J Urol. 1994;151 (4): 977-979. doi: 10. 1016/S0022-5347 (17) 35140-6 This abstract is funded by: none
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