Abstract: TH-PO098
Steroid-Responsive AKI in a Patient with Hemophagocytic Lymph Histiocytosis (HLH)
Session Information
- AKI: Clinical, Outcomes, and Trials - Epidemiology and Pathophysiology
October 24, 2024 | Location: Exhibit Hall, Convention Center
Abstract Time: 10:00 AM - 12:00 PM
Category: Acute Kidney Injury
- 102 AKI: Clinical, Outcomes, and Trials
Authors
- Amjad, Arfa, SUNY Downstate Health Sciences University, New York City, New York, United States
- Soe, Thin Thin, SUNY Downstate Health Sciences University, New York City, New York, United States
- Azhar, Muhammad, New York City Health and Hospitals Corporation, New York, New York, United States
Introduction
Acute kidney injury (AKI) in patients with hemophagocytic lymphohistiocytosis (HLH) is a frequent presentation that accounts for 30% to 50% of HLH cases. Most of these patients are managed with renal replacement therapy (RRT). We present a case that showed complete recovery of renal function to its baseline with steroid therapy in HLH patients without requiring RRT.
Case Description
64 y/o, African American Male with a history of hypertension, chronic kidney disease stage 3a who presented with 2 days of dizziness and malaise. He was febrile to 102.9 Fahrenheit and was found to be in multiorgan failure with Acute Kidney Injury, Acute liver injury & cytopenia. Labs revealed serum creatinine of 12.62 mg/dL from a baseline of ~1.28 mg/dL, thrombocytopenia, anemia, hyperferritinemia, elevated ESR & CRP with Soluble CD25: 2321.7, Urinalysis positive for cast cells of > 20. Renal ultrasound was unremarkable. His calculated HScore showed > 95% probability of HLH ( hypertriglyceridemia, hyperferritinemia, cytopenia, elevated AST with liver of 14.6 cm). His AKI was likely due to acute tubular necrosis related to HLH. He was started on dexamethasone for the treatment of HLH and was noticed to have a gradual recovery of renal function to its baseline without requiring renal replacement therapy.
Discussion
Hemophagocytic lymphohistiocytosis (HLH) is a rare heterogenous clinical syndrome involving massive activation and proliferation of natural killer cells and cytotoxic T lymphocytes, resulting in cytokine storm and hemophagocytosis.
Acute kidney injury in HLH results from ischemic or inflammatory lesions of the renal tubules. The most frequent presentation of renal involvement is acute tubular necrosis; an autopsy series found such lesions in up to 45 % of patients with HLH. AKI typically exhibits poor prognosis in affected HLH patients; a retrospective analysis of 600 patients showed 53.3% mortality in patients with AKI vs 17.5% without AKI. Treatment of HLH usually requires specific treatment of underlying infection or malignancy, chemoimmunotherapy to suppress inflammation, and in some cases hematopoietic stem cell transplantation. Most of these patients with AKI are managed with renal replacement therapy. Our case was interesting since the renal function normalizes with a shorter duration of steroids than what is usually prescribed in these cases.