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... 4 The most common symptom of malakoplakia is a recurring urinary tract infection (UTI), haematuria or renal failure occurring at presentation. 5 Typically, E. coli would be grown in urine cultures or evidence could be seen on cystoscopy. ...
... 13 The presence of Michaelis-Gutmann bodies, basophilic structures that exist within clusters of macrophages, on histological examination is pathognomonic for the diagnosis. 5 Although malakoplakia resembles carcinoma on imaging and gross pathology, its treatment can be very different. In the past, bilateral renal malakoplakia was treated with antibiotics, whereas unilateral was dependent on extension of the inflammation and the patient's condition. ...
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Renal malakoplakia, a seldom seen chronic inflammatory condition, continues to elude medical, surgical, radiological and pathological specialists due to its mimicry of other renal pathologies and low incidence. The variable clinical manifestations and non-specific radiological findings of malakoplakia can be misleading, and ultimately require a pathological diagnosis. A literature review reveals an extremely low prevalence of renal malakoplakia, a handful of invasive renal malakoplakia cases and no reports of liver and diaphragmatic invasion. We present a case of a renal mass with liver and diaphragmatic invasion in a 59-year-old woman that deceived clinicians and radiologists until a pathological diagnosis of renal malakoplakia was performed. This case highlights the need of awareness for malakoplakia in the differential diagnosis for renal invasive and non-invasive masses. The need to await a surgical biopsy and pathological diagnosis is critical to ensure a correct diagnosis and avoid unnecessary surgery of the kidney.
... For instance, patients with renal parenchymal malakoplakia commonly present with fever, loin pain and enlarged kidneys with inconstant renal dysfunction. [4][5][6] We report here [7] a new systemic manifestation of malakoplakia. In this patient, malakoplakia was indeed responsible for severe hypercalcemia through ectopic expression of 25-hydroxyvitamin D3 1-alphahydroxylase in the von Hansemann cells. ...
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Rationale: Malakoplakia is a rare disease characterized by the presence of nongranulomatous macrophage infiltration. In most cases, it affects the urinary tract. Malakoplakia can cause acute kidney injury when it is localized in the kidneys. Patient concerns: Here, we report the case of a 65-year-old female patient with renal malakoplakia responsible for hypercalcemia. During her initial assessment, she was also diagnosed 25-OH vitamin D insufficiency, for which she was prescribed oral cholecalciferol. Three months later, she developed severe hypercalcemia with normal 25-OH vitamin D and parathyroid hormone levels and high 1,25-dihydroxyvitamin D levels. Diagnoses: After a superimposed granulomatous disease was excluded, malakoplakia cells were suspected to be responsible for the abnormal 25-hydroxyvitamin D3 1-alpha-hydroxylase activity, which was confirmed by immunohistochemistry. Interventions: Cholecalciferol was stopped, the patient was rehydrated with intravenous physiological saline, and prednisone was initiated to decrease the enzyme activity. Outcomes: Six months later, she displayed normal serum calcium, 25-OH vitamin D and 1,25-dihydroxyvitamin D levels. Lessons: This case illustrates that malakoplakia may exhibit ectopic 25-hydroxyvitamin D3 1-alpha-hydroxylase activity and cause severe hypercalcemia upon vitamin D supplementation. Therefore, such supplementation should not be given in malakoplakia patients without an actual deficiency and requires careful monitoring of serum calcium.
... While it shows a predilection for the urinary bladder, Malakoplakia has a predilection for immunocompromised patients and is also frequently seen in middle-aged women with a history of chronic urinary tract infections. Renal failure is also common at presentation [ 35 ]. Common symptoms include fever, dysuria, and urinary frequency and urgency [ 36 , 37 ]. ...
Chapter
Nonneoplastic renal processes can present a diagnostic challenge for clinicians due to their ability to mimic malignant masses, both clinically and radiographically. This chapter will review benign renal diseases that can present clinically as a renal mass, including inflammatory conditions such as pyelonephritis, renal abscess, xanthogranulomatous pyelonephritis, and renal malakoplakia; cystic kidney disease including autosomal dominant and autosomal recessive polycystic disease; acquired cystic disease of the kidney; and other miscellaneous lesions. Radiographic images, gross pathology, and histological features will be discussed, as well as common management strategies for these entities.
... These findings require work up with renal biopsy. 1 While the gross presentation of the condition may resemble carcinoma, which indicates nephrectomy, malakoplakia calls for more conservative measuresmedicinal therapy is indicated. 6 The presence of Michaelis-Gutmann bodies on histological examination is pathognomonic for the diagnosis. These bodies are basophilic structures that exist within clusters of macrophages, termed von Hansemann cells, which are amalgamated with lymphocytes and plasma cells. ...
... These bodies are basophilic structures that exist within clusters of macrophages, termed von Hansemann cells, which are amalgamated with lymphocytes and plasma cells. 6 A Michaelis-Gutmann body can also be found extracellularly among bundles of collagen producing fibroblasts within the stroma, and its composition includes organic components as well as calcium, phosphorus and iron. 3 4 Treatment of malakoplakia is contingent on the extent of the disease and the underlying conditions of the patient. ...
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Malakoplakia, a medical, surgical, pathological and radiological enigma, is an infrequent chronic inflammatory condition that can affect many organ systems, including the gastrointestinal tract, integument, skeletal system and genitourinary tract. Review of the literature has shown that malakoplakia presents in paediatric as well as adult populations, and that it is associated with impaired immune function. Variable clinical manifestations as well as the sometimes non-specific radiological findings of malakoplakia can be misleading, making diagnosis quite difficult. We present a clinical case of renal malakoplakia mimicking a malignant renal carcinoma in a 62-year-old woman. This report highlights the importance of awareness of malakoplakia in the differential diagnosis for renal masses and renomegaly. This case can serve as a reminder that things are not always what they seem, and it reinforces the idea that unusual disease entities should be explored to aid in achieving a correct diagnosis and, thus, potentially avoid unnecessary treatment. 2015 BMJ Publishing Group Ltd.
... Malakoplakia is associated with urinary tract infections in the majority of cases. Renal failure is common at presentation with variable severity [4]. About 40% of patients have some form of immunosuppression, including solid organ transplants, autoimmune diseases requiring steroid use or chemotherapy, chronic systemic diseases, malignancy, alcohol abuse and poorly controlled diabetes mellitus [5]. ...
... The microscopic differential diagnoses are Whipple's lipodystrophy, xanthogranulomatous pyelonephritis, megalocytic interstitial nephritis and granular cell tumor [1]. The diagnosis of malakoplakia must be kept in mind for patients presenting with a renal mass and a history of long-term recurrent renal infections or renal failure [4]. Renal malakoplakia may mimic renal tumors and lead to unnecessary surgery. ...
... There are two reported cases of renal malakoplakia leading to renal failure. Diwakar et al. reported a case of renal malakoplakia causing acute renal failure [4]. Hegde and Coulthard reported another case of bilateral renal malakoplakia that caused end-stage renal failure in a five-year-old girl [5]. ...
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Introduction Malakoplakia is an uncommon chronic inflammatory condition that has a gross and microscopic appearance resembling that of xanthogranulomatous pyelonephritis. It is characterized by distinctive Michaelis-Gutmann bodies. Malakoplakia can affect any organ system but genitourinary tract involvement is the most common, particularly in immunocompromised individuals. Very rare cases have been reported to present as a unifocal lesion mimicking a renal tumor. Case presentation We report a case of renal malakoplakia in a 55-year-old Iranian man with a past history of recurrent urinary tract infections who presented with left flank pain. An ultrasound study showed a large solid left renal mass, and he underwent a left radical nephrectomy with a clinical diagnosis of a renal tumor. Pathology slides revealed the diffuse infiltration of sheets of Periodic Acid Schiff-positive histiocytes in his renal parenchyma; these cells showed strong immunoreactivity for CD 68. The final diagnosis was renal malakoplakia. Conclusion Renal malakoplakia must be kept in mind for patients presenting with a renal mass and a history of long-term recurrent renal infections or renal failure. The large, rapidly growing nodules of malakoplakia may mimic renal cell carcinoma in imaging studies. In these cases, a true cut needle biopsy may help the correct diagnosis and prevent unnecessary surgery.
... Le traitement de la malakoplakie repose sur une antibiothérapie adaptée au germe retrouvé et possédant une bonne pénétration intracellulaire. Des traitements par fluoroquinolone de plus de huit semaines sont généralement rapportés dans la littérature[2,5,[17][18][19][20]. Les traitements immunosuppresseurs devraient être interrompus dans la mesure du possible. ...
Article
Full-text available
Malakoplakia is an uncommon chronic granulomatous inflammatory disease which is associated with immunocompromised conditions such as malignancy, autoimmune disease, chronic alcohol intake, poorly controlled diabetes and long-term steroid use. Malakoplakia can occur at various sites, most commonly in the genitourinary tract including urinary bladder and the ureter. Renal parenchymal involvement is relatively uncommon, accounting for 15% of all malakoplakia. A few cases of renal malakoplakia have been reported in Korea, and only one case was accompanied by acute kidney injury. Here we report an 80-year-old female patient with renal parenchymal malakoplakia and acute interstitial nephritis presented as acute kidney injury with literature review.
Article
A 4-month-old female kitten presented with chronic lower urinary tract signs and Escherichia coli cystitis, and was diagnosed with urinary bladder malakoplakia based upon histopathology. The kitten was treated with a prolonged antibiotic course and the malakoplakia resolved. Malakoplakia is a chronic granulomatous reaction characterized by the formation of Michaelis-Gutman bodies within von Hansemann macrophages. It is well described in humans, but has never been documented in a living veterinary patient. This case report describes the first successful treatment of malakoplakia in veterinary medicine.