An 18-year-old Indian male presented with history of low-grade fever and bilateral pedal edema for about one and half months in 1995. He had no history of urinary symptoms including oliguria or burning micturition. There was no notable finding in physical examination except bilateral pedal edema and facial puffiness. He was a non-smoker and teetotaler. He was not diabetic. He was normotensive. There was no significant past history. Tests for retrovirus; hepatitis B and C were negative. Routine investigation revealed normal hematocrit (Hemoglobin-14 gm/dl) and slightly elevated renal parameters (Serum creatinine-1.6 mg/dl, blood urea-51 mg/dl), apart from albuminuria and pyuria on urine analysis. On further evaluation his 24-hour urine protein was found to be 959 mg/day. Urine culture was sterile. Ultrasound of abdomen and pelvis revealed cholelithiasis and bilateral hydroureteronephrosis and polypoidal mixed echogenic lesions mostly at the base of the bladder. IVU (Intra venous urogram) was not done due to altered renal parameters and patient was subjected to diuretic Renogram to assess functional status and drainage pattern of the kidneys. Renogram revealed decreased tracer uptake and delayed excretion of tracer on both sides. Cortical margin was very much thinned out in left kidney when compared to right kidney. Relative contribution of left kidney was 32% with GFR of 24 ml/min. Micturating cystourethrogram has not revealed any evidence of reflux into upper tracts. His urodynamic evaluation was normal with evidence of normal compliance and normal voiding pressure. Cystoscopy done for the evaluation of polypoidal lesions of the bladder revealed pinkish-yellow nodules of 2 to 3.5 cm size over trigonal area, on left ureteric orifice and at bladder neck. Ureteroscopy was performed on both sides in view of the bilateral hydroureteronephrosis, which revealed multiple white nodules in the lower segment of left ureter. Biopsy of the trigonal and lower ureteric lesions were performed using flexible biopsy forceps.
Microscopy has revealed the presence of sheets of large granular macrophages (von Hansemann cells) with tiny basophilic periodic acid-Schiff and von Kossa positive calcific inclusions (Michaelis Gutmann bodies), admixed with lymphocytes and plasma cells (). The macrophages were immunohistochemically positive for CD68 antibodies ().
Macrophages (von Hansemann cells) with tiny basophilic inclusions (Michaelis Gutmann bodies) (H&E × 200).
Macrophages showing positivity for CD68.
Higher magnification showing intra and extra cellular Michaelis Gutmann bodies (H&E × 400).
Michaelis Gutmann bodies (Von kossa).
Patient was put on antibiotic treatment and left ureterocystotomy was done to prevent deterioration of existing function of the left kidney. Histopathological examination of excised distal segment of left lower ureter also revealed malakoplakia (). He was put on suppressive antibiotics and advised regular follow-up but was lost for follow-up after six months. He turned up again after a gap of ten years with feature of end stage renal disease. According to the patient, he was alright for almost nine years. Then he was evaluated for chronic renal failure and detected to have hypertension in 2004. He was advised to undergo renal transplantation but the patient could not afford it. He was admitted again in 2005 with complaint of low grade intermittent fever with chills and rigor, oliguria, urgency and difficulty in micturition of one month duration. There was history of facial puffiness and rapidly worsening breathlessness of 10days duration. There was also one episode of epistaxis. He had been in altered sensorium for one day prior to the day of admission. At the time of presentation, clinical examination revealed anemia, edema, hypertension, bilateral basal rales and altered sensorium. Investigations revealed severe renal insufficiency (serum creatinine was 19.9 mg/dl and serum urea was 340 mg/dl), hyperphosphatemia, and anemia (Hemoglobin was 5.1 gm/dl), leucocytosis with left shift, thrombocytopenia, pyuria and pulmonary edema. Ultrasound revealed normal sized right kidney with echogenic cortex and loss of corticomedullary distinction, left kidney contracted with increased echoes. He underwent 3 sessions of hemodialysis. During his hospital stay he developed one episode of generalized tonic clonic seizures for which phenytoin was started. Prognosis of patients condition and need for long term regular therapy was explained to the relatives. But they requested to take the patient home. Hence, he was discharged and advised treatment with Cefodoxime, Pantoprazole, Calcium, Multivitamin and Phenytoin. The patient succumbed to the illness soon after.
Malakoplakia of left ureter (H&E × 200).