Abstract: FR-PO0438
Beyond Schistocytes: Dialysis-Dependent AKI and Nephrotic-Range Proteinuria as the Predominant Manifestation of Acquired Thrombotic Thrombocytopenic Purpura
Session Information
- AKI: Case Reports - TMA, Vasculitis, Immune-Mediated Injury, and Systemic Disease
October 23, 2026 | Location: Exhibit Hall A, Convention Center
Abstract Time: 10:00 AM - 12:00 PM
Category: Acute Kidney Injury
- 102 AKI: Clinical, Outcomes, and Trials
Authors
- Abro, Sheeraz, The University of Mississippi Medical Center, Jackson, Mississippi, United States
- Abro, Paras A., Liaquat University of Medical & Health Sciences, Jamshoro, Sindh, Pakistan
Introduction
Thrombotic thrombocytopenic purpura (TTP) classically presents with microangiopathic hemolytic anemia, thrombocytopenia, schistocytosis, neurologic dysfunction, and mild renal involvement. Severe dialysis dependent AKI is uncommon in TTP and more frequently associated with hemolytic uremic syndrome, making diagnosis difficult in atypical presentations.
Case Description
A 56 year old woman with steatotic liver disease who presented with encephalopathy, oliguria, nephrotic range proteinuria, and severe AKI requiring hemodialysis. Laboratory evaluation revealed elevated LDH, UPCR 4.58 g/g, severe ADAMTS13 deficiency with activity 0.04, and positive ADAMTS13 inhibitor. Peripheral smear lacked schistocytosis and haptoglobin normal. Extensive serologic evaluation was unrevealing. Kidney biopsy was deferred because of high bleeding risk with thrombocytopenia.
Despite minimal hematologic evidence of microangiopathic hemolysis, acquired TTP was diagnosed based on severe ADAMTS13 deficiency with inhibitor positivity, encephalopathy, thrombocytopenia, and dialysis dependent AKI. The patient was treated with plasma exchange, high dose corticosteroids, and rituximab 375 mg/m2 weekly for four doses. Following therapy, mentation, thrombocytopenia, urine output, and kidney function improved with discontinuation of hemodialysis. Proteinuria resolved with renal recovery. Repeat testing demonstrated normalization of ADAMTS13 activity with negative inhibitor levels.
Discussion
This case highlights an unusual presentation of acquired TTP manifesting predominantly as severe dialysis dependent AKI and nephrotic range proteinuria with minimal hematologic evidence of hemolysis. Absence of schistocytosis or overt hemolysis should not exclude TTP in patients with severe ADAMTS13 deficiency. Early recognition and prompt initiation of plasma exchange and immunosuppressive therapy may result in renal recovery and dialysis independence even in severe presentations.
Key Diagnostic Findings and Clinical Outcomes in Atypical Acquired TTP
| ADAMTS13 Activity | 0.04 |
| ADAMTS13 Inhibitor | Positive 1.6 |
| Peripheral Smear | No schistocytes |
| Peak Creatinine | 4.0 mg/dL requiring HD |
| Proteinuria | UPCR 4.58 g/g |
| Treatment | PLEX + steroids + rituximab |
| Outcome | Negative inhibitor with renal recovery and HD independence |