Abstract: TH-PO0066
Between a Rock and a Hard Place!
Session Information
- AKI: Pathogenesis and Disease Mechanisms
November 06, 2025 | Location: Exhibit Hall, Convention Center
Abstract Time: 10:00 AM - 12:00 PM
Category: Acute Kidney Injury
- 102 AKI: Clinical, Outcomes, and Trials
Authors
- Baigam, Nahida, LSU Health New Orleans, New Orleans, Louisiana, United States
- Mohandas, Rajesh, LSU Health New Orleans, New Orleans, Louisiana, United States
- Bajracharya, Siddhartha Darshan, LSU Health New Orleans, New Orleans, Louisiana, United States
Introduction
Hemophagocytic lymphohistiocytosis (HLH) and thrombotic thrombocytopenic purpura (TTP) are rare disorders with high mortality, and rarely occur concurrently.
Case Description
43-year-old male with a history of asthma, depression, migraines, and seizures presented with general malaise and chest pain, along with bruising and skin mottling. He had elevated INR, D-dimers, lactic acid, liver enzymes, ferritin, troponin, creatinine, and thrombocytopenia. Blood cultures showed pneumococcal bacteremia. EBV serology was Negative. An H-score of 235 indicated a high probability of HLH, and was started on empiric steroids. ADAMTS13 activity was 1%, suggesting TTP. He was started on therapeutic plasma exchange (TPE) which improved his platelet count and ADAMTS13 level; also started renal replacement therapy (RRT) for oliguric AKI presumed due to septic acute tubular injury, IL-2 receptor levels were >4000, suggesting HLH. He developed bullous rash w/subcutaneous edema. He continues to receive TPE, steroids, and antibiotics with a bone marrow biopsy planned.
Discussion
TTP and HLH are both life-threatening syndromes requiring immediate attention. TTP is diagnosed using a clinical pentad and confirmed with ADAMTS13 assay, and treated with TPE. Accurate diagnosis of HLH relies on meeting five of eight clinical criteria and H-score, with elevated ferritin levels above 7,500-10,000 ng/ml enhancing specificity. Neurological symptoms and thrombocytopenia in both conditions can complicate diagnosis. Both conditions demand aggressive treatment strategies— for TTP, plasma exchange, high-dose corticosteroids, and adjunct therapies like Rituximab; HLH is treated with steroids, IVIG, and addressing underlying etiology such as infection or malignancy. There are case reports of pneumococcal infections separately causing TTP and HLH; and also reports of concurrent HLH and TTP, due to EBV infection or malignancy. This is the first reported case of pneumococcal infection causing concurrent TTP and HLH.