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[Malacoplakia of the kidney]
Lj Dokić1, D Cvetković, N Svirtlih
1Institute of Infectious and Tropical Diseases, University Clinical Centre, Belgrade.
Abstract:
Malakoplakia is a rare disease expressed as a special type of inflammatory reaction to infection with various bacteria and fungi. This unusual response is based on defective function of the macrophage system. In malakoplakia infiltrates, most cells are macrophages mixed with lymphocytes, plasma cells and fibroblasts. The macrophages (von Hansemann's histiocytes) with intracytoplasmatic inclusions defined as Micaelis-Guttmann bodies are pathognomonic histological findings. The clinical manifestation of the disease depends on the localisation of lesions, most often in the urinary bladder and the kidney. The authors present a case of renal malakoplakia of in a 32-year-old female patient. The symptoms were not enough characteristic for making the true diagnosis. The diagnosis of malakoplakia was established during autopsy. The histopathological examination revealed von Hansemann's histiocytes and Michaelis-Guttmann bodies in malakoplakia infiltrates.
Insights
Malakoplakia is a rare inflammatory condition resulting from defective macrophage function. This case highlights renal malakoplakia, diagnosed post-mortem via characteristic histopathology, including von Hansemann
Area of Science:
- Pathology
- Immunology
- Rare Diseases
Background:
- Malakoplakia is an uncommon inflammatory disorder characterized by a unique host response to bacterial and fungal infections.
- The condition stems from impaired macrophage (a type of white blood cell) function.
- Histologically, malakoplakia infiltrates contain macrophages with characteristic intracytoplasmic inclusions known as Michaelis-Guttmann bodies.
Observation:
- The authors report a case of renal malakoplakia in a 32-year-old female patient.
- Clinical symptoms were nonspecific, hindering a definitive diagnosis during the patient's lifetime.
- The diagnosis was confirmed post-mortem through autopsy and detailed histopathological examination.
Findings:
- Histopathological analysis revealed the presence of von Hansemann's histiocytes (macrophages) and Michaelis-Guttmann bodies within the renal malakoplakia infiltrates.
- These findings are pathognomonic for malakoplakia, confirming the diagnosis.
Implications:
- This case underscores the diagnostic challenges posed by renal malakoplakia due to its often subtle and non-specific clinical presentation.
- Autopsy and histopathology remain crucial for diagnosing rare conditions like malakoplakia, especially when clinical suspicion is low.
- Understanding the pathognomonic histological features is vital for accurate diagnosis and contributes to the literature on rare inflammatory diseases.