Abstract: SA-PO103
Renal Malakoplakia in a Young Patient
Session Information
- AKI: Epidemiology, Risk Factors, Prevention - II
November 04, 2023 | Location: Exhibit Hall, Pennsylvania Convention Center
Abstract Time: 10:00 AM - 12:00 PM
Category: Acute Kidney Injury
- 101 AKI: Epidemiology, Risk Factors, and Prevention
Authors
- Shrestha, Sanjivani, The Johns Hopkins University School of Medicine, Baltimore, Maryland, United States
- Le, Dustin, The Johns Hopkins University School of Medicine, Baltimore, Maryland, United States
- Atta, Mohamed G., The Johns Hopkins University School of Medicine, Baltimore, Maryland, United States
- Sperati, John, The Johns Hopkins University School of Medicine, Baltimore, Maryland, United States
Introduction
Malakoplakia is a rare inflammatory disorder which commonly affects the genitourinary tract, but may also involve the gastrointestinal tract, lungs and skin. It is usually described in patients above age 40 years and is associated with infections and immunosuppressed conditions. We present a case of renal malakoplakia in a young female in the setting of E. coli urinary tract infection (UTI).
Case Description
A 32-year-old female with history of recurrent UTI, Raynaud’s phenomenon, COVID-19, presented with 2 days of shortness of breath and lower extremity weakness. On examination, she was afebrile, tachycardic, hypotensive with diffuse petechial skin rash and hepatosplenomegaly. Labs showed leukocytosis, anemia, thrombocytopenia and acute kidney injury with creatinine 6.4 mg/dL (baseline 0.64mg/dL). Blood culture grew E. coli. CT abdomen pelvis showed marked bilateral kidney enlargement and continuous renal replacement therapy was started in the setting of worsening septic shock. The differential diagnosis of enlarged kidneys includes diabetes mellitus, cystic disease and infiltrative disease like amyloidosis, lymphoma, IgG4 related disease, hemophagocytic lymphohistiocytosis and malakoplakia. HIV and TB testing were negative. Kidney biopsy demonstrated pyelonephritis with focal micro-abscesses and diffuse CD163 positive inflammatory infiltrate consistent with macrophage/histiocytic cells. Von Kossa stain was positive with round inclusions in histiocytes, typical of Michaelis-Gutman bodies. Biopsy findings consistent with Malakoplakia. She was treated with antibiotics, bethanechol and ascorbic acid with recovery of kidney function sufficient to stop dialysis. Given intermittent fevers, persistent leukocytosis and enlarging left renal abscesses on CT, she underwent left nephrectomy 2 months into hospitalization. She was discharged home on oral minocycline.
Discussion
This case highlights the importance of considering malakoplakia in the differential diagnosis of nephromegaly, even in young patients. It is due to defective destruction of phagocytosed microorganisms by macrophages, although the underlying mechanisms remain unclear. Optimal therapy is uncertain, and this case emphasizes the importance of aggressive care.