Abstract: SA-PO825
A Tale of Two Findings: Thrombotic Microangiopathy and Podocytopathy Due to Graft vs. Host Disease
Session Information
- C3G, TMA, MGRS, Amyloidosis, and More
October 26, 2024 | Location: Exhibit Hall, Convention Center
Abstract Time: 10:00 AM - 12:00 PM
Category: Glomerular Diseases
- 1402 Glomerular Diseases: Clinical, Outcomes, and Therapeutics
Authors
- Hall, Jonathan, University of Nebraska Medical Center, Omaha, Nebraska, United States
- Mcmillan, David A., University of Nebraska Medical Center, Omaha, Nebraska, United States
- Leonardi, Nathaniel, University of Nebraska Medical Center, Omaha, Nebraska, United States
- Ravipati, Prasanth, University of Nebraska Medical Center, Omaha, Nebraska, United States
- Mullane, Ryan, University of Nebraska Medical Center, Omaha, Nebraska, United States
Introduction
Acute kidney injury (AKI) following hematopoietic stem cell transplantation (HSCT) is common, and typically multifactorial in etiology related to infections, medications, and graft versus host disease (GVHD). Thrombotic microangiopathy (TMA) following HSCT is not uncommon and can be associated with GVHD. When TMA is due to GVHD, it is often refractory to treatment with steroids. Nephrotic syndrome, however, is a rare presentation of kidney disease following HSCT.
Case Description
A 56-year-old female with myelodysplastic syndrome that progressed to acute myeloid leukemia for which she had undergone allogenic HSCT 8 months ago was found to have a serum creatinine of 2.9 mg/dL (prior serum creatinine of 1.0 mg/dL). She reported recent development of leg edema with weight gain. Her serum albumin was 2.5 g/dL, a urine protein to creatinine ratio was 14.7, and urine microscopy revealed microscopic hematuria. A kidney biopsy was performed, and the patient was begun on IV methylprednisolone.
Light microscopy revealed mesangiolysis, duplication of basement membranes, and intracapillary red cell fragmentation, consistent with TMA. Electron microscopy showed subendothelial widening with accumulation of flocculent material and podocyte effacement of at least 90% of the sampled capillary surface area.
Despite treatment with IV methylprednisolone, the patient had progression of kidney failure and required hemodialysis. Given the biopsy findings of TMA, treatment was started with eculizumab. The patient had subsequent improvement in her kidney function, and hemodialysis was stopped. Over the next 4 weeks, her serum creatinine decreased from a peak of 5.6 mg/dL to 2.2 mg/dL, and her serum albumin normalized.
Discussion
Co-presentation of TMA with diffuse podocytopathy is a rare cause of kidney dysfunction in patients post-HSCT. TMA due to GVHD is typically refractory to treatment with steroids and is thought to be due to alternative complement pathway dysfunction, necessitating treatment with complement blockade. Combination therapy with eculizumab and corticosteroids in this unusual case of concurrent TMA and podocytopathy led to improvement in the patient’s renal function and normalization of the patients’ serum albumin.