Malakoplakia of the urinary bladder: A review of the literature.

OBJECTIVE: The aim of the study is to make a review of the literature about bladder malakoplakia. MATERIAL AND METHODS: We searched articles on the PUBMED web-literature database with the following keywords: "vesical malakoplakia" and "bladder malakoplakia". In the literature we found 254 articles. At final we have excluded 219 articles, including in our study only 35 articles. RESULTS: The overall average age found was 50.85 years. The average age of men was 43.22 years, while that of women was 53.37 years. 75% of the patient cases were women and 25% were men. Regarding comorbidities, in 5.55% of the cases were missing whereas 47.22% of the patients suffered from recurrent urinary tract infection (UTI) and 19.44% from immune system disorders. Urine culture was positive in 69.44% with E.coli being isolated in 92% of cases. Hydroureteronephrosis was present in 44.44% of the cases: left in 6.25% of cases, right in 18.75% and bilateral in 75%. The mean serum creatinine of patients with hydroureteronephrosis was 5.11 (1-21) mg/dl. The most frequent site of the lesion was the vesicoureteral junction (VUJ) (42.31%), followed by the trigone (38.46%). 30.56% of patients were treated with antibiotic and surgery (transurethral resection of bladder, partial or radical cystectomy), less frequent options were antibiotics alone and surgery alone. The recurrence rate was 15%. CONCLUSIONS: Malakoplakia is a disorder usually related to other affections, like UTI and immunodepression, and it seem to be caused by an abnormal macrophage function. In almost half of the described cases of isolated bladder malakoplakia, hydroureteronephrosis and renal failure were present.Treatment is not standardized, but both medical and surgical therapies are effective to avoid recurrence.

Malacoplakia of the skin: overview of a rare clinical entity.

BACKGROUND: Malacoplakia is a rare acquired, infection-related granulomatous disorder, that may affect many systems, but typically occurs in the urinary tract. Cutaneous involvement is less prevalent, and most commonly presents with a perianal or genital region localization. Cutaneous malacoplakia is believed to be caused by an acquired bactericidal defect of macrophages in the setting of chronic infections and immunocompromised states. A diagnosis of cutaneous malacoplakia should be considered when encountering non-specific granulomatous lesions that are refractory to treatment. Histologic findings are marked by the presence of foamy macrophages containing the pathognomonic Michaelis-Gutman bodies. OBJECTIVES: The aim of this review is to discuss the current perspectives on the pathophysiology, clinical features, diagnosis, and treatment of this disease. We would also like to emphasize that the integration of clinical information, microscopic findings, and exclusion of other cutaneous granulomatous processes is necessary to accurately diagnose this exceedingly rare disease and provide opportunity for therapeutic intervention. PATIENTS/METHODS: Data for this work were collected from the published literature and textbooks. RESULTS: Combined surgical excision and protracted antibiotic courses appear to have the highest success rate. Antibiotics should be culture specific, but drugs that easily permeate the macrophages appear to be the best choice.

Diffuse parenchymal form of malakoplakia in renal transplant recipient: a case report.

Malakoplakia is an unusual chronic inflammatory disease related to prior urinary tract infection. It is characterized by the presence of macrophages with foamy cytoplasm exhibiting larger PAS positive inclusions that stain for calcium and iron. Malakoplakia affects renal allograft and is associated with severe morbidity. Herein, the authors report a new case of renal graft malakoplakia in a 23-year-old female patient. The patient received a living-related donor renal transplantation with a high immunological risk. Plasmapheresis and intravenous immunoglobulin (i.v. Ig) treatment, pre- and post-transplant, and induction with rabbit anti-thymocyte globulins were used due to presence of donor specific antibodies and positive B cross match by flow cytometry. The patient had an early urinary tract infection with a good outcome. On Day 36 post-transplant (PO), the patient returned to the clinic with fever, graft pain and acute renal dysfunction leading to hemodialysis. Escherichia coli (E. coli) was present in the blood and urine culture. At the time, the renal biopsy revealed numerous sheets of macrophages with foamy, eosinophilic cytoplasm showing several PAS positive granules and large inclusions that stained strongly with hematoxylin, calcium (von Kossa method) and iron (Prussian blue). The patient was diagnosed with malakoplakia related to a kidney transplant. Despite prolonged treatment with antibiotics, determined by a susceptibility test, the patient did not recover renal function and remained on dialysis.

Malakoplakia in a healthy young female patient.

Malakoplakia is a rare granulomatous disease that occurs commonly in the urinary tract and secondarily in the gastrointestinal tract. Most reported cases of malakoplakia are associated with immunosuppressive diseases or chronic prolonged illness. Here, we report a rare case of malakoplakia in a young healthy adolescent without any underlying disease. A 19-year-old female was referred to our hospital following the discovery of multiple rectal polyps with sigmoidoscopy. She had no specific past medical history but complained of recurrent abdominal pain and diarrhea for 3 months. A colonoscopy revealed diverse mucosal lesions including plaques, polyps, nodules, and mass-like lesions. Histological examination revealed a sheet of histiocytes with pathognomonic Michaelis-Gutmann bodies. We treated the patient with ciprofloxacin, the cholinergic agonist bethanechol, and a multivitamin for 6 months. A follow-up colonoscopy revealed that her condition was resolved with this course of treatment.

Renal malakolpkia followed by chronic myelogenous leukaemia: treatment challenges case report and literature review.

Malakoplakia is a rare granulomatous disorder of unknown aetiology and usually affects patients with underlying immunosuppression. This disorder usually involves the genitourinary tract but has been reported in a wide array of anatomical sites. We are presenting, what is to our knowledge, the first case in the literature in which a patient was diagnosed with malakoplakia and after his successful management; he was diagnosed with chronic meylomoncytic leukaemia. All cases of malakoplakia reported in the literature were either preceded or accompanied by an immunosuppressive state.

Successful treatment of malakoplakia of the bladder in a kitten.

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.

[Malakoplakia involving kidney, both ureters and bladder: a case report and review of the literature]. / Malakoplakie rénale, urétérale bilatérale et vésicale : à propos d'un cas et revue de la littérature.

Malakoplakia is an unusual chronic inflammation characterized macroscopically by pseudotumoral lesions and histologically by the presence of "Michaelis-Gutman" bodies in macrophages. It affects principally the urinary tract and is probably secondary to an unusual macrophagic reaction to recurrent urinary tract infections. If the bladder is the most frequent site of occurrence, the ureters can very rarely be affected resulting in urinary tract obstruction. We presented here a unique presentation of renal, bilateral ureteral and bladder malakoplakia.

Malacoplakia of the vallecula masquerading as a malignancy.

Malacoplakia is an unusual chronic granulomatous inflammation that commonly occurs in the urinary tract. It is rarely encountered in the head and neck region, and only a handful of such cases have been described. We report a case of malacoplakia in a 50-year-old woman who presented with a mass in the vallecula that was clinically suspected of being malignant. This case illustrates the importance of recognizing this unusual benign lesion, which closely mimics a malignancy on clinical examination.

[Renal malakoplakia: an underestimate cause of renal failure]. / Malacoplakie renale: une cause méconnue d'insuffisance rénale.

Malakoplakia is an inflammatory granulomatous disease induced by defective phagocytic activity of macrophage. Malakoplakia is histologically characterized by the presence of Michaelis-Gutmann bodies in macrophages. Although not uncommon in the genito-urinary tract, isolated malakoplakia of the kidney is rarely found. Its main clinical presentation associates acute renal failure and acute pyelonephritis. The clue for diagnosis of renal malakoplakia is based on renal biopsy showing Michaelis-Gutmann bodies. Establishing the diagnosis of renal malakoplakia is essential as it determines the choice of antibiotics and duration of treatment. Prognosis remains poor, leading frequently to chronic renal failure. In this paper, we report four cases of renal malakoplakia and discuss clinical presentation, biological and pathological features, treatment and prognosis of this disease.

Malacoplakia of the tongue: a case report and clinicopathologic review of 6 cases.

BACKGROUND: Extra-urogenital tract malacoplakia is uncommon, with tongue malacoplakia being exceptionally rare. The nonspecific clinical presentation and variable histologic patterns can make recognition of this lesion and separation from other lesions challenging. There are only a few reported cases in the English literature. MATERIALS AND METHODS: Five case reports of tongue malacoplakia were compiled from the literature (MedLine 1960-2008) and integrated with this case report. RESULTS: The patients included 4 males and 2 females, ranging in age from 9 to 98 years (mean, 64 years). Patients presented with difficulty swallowing, foreign body sensation, a mass lesion, or referred pain (neck or ear). Symptoms were present from a few days up to 18 months. The base of the tongue was the most frequent site, although midline tongue and half of the tongue were also affected. Radiographic studies demonstrated a mass, with a single lesion showing positron emission tomography positivity. Two patients had previous cancers (prostate and colorectal; larynx). This case report was a farm hand for horses, with gram-negative rods, suggestive of Rhodococcus equi identified. The lesions were 1 to 2 cm in greatest dimension. Histologically, there is pseudoepitheliomatous hyperplasia or ulceration with a heavy acute and chronic inflammatory infiltrate. The subepithelial spaces are completely filled with eosinophilic histiocytes, most of which contain granular material in their cytoplasm. Well-formed, blue, calcific bodies are noted, a few showing a "targetoid appearance" and concentric lamination. These Michaelis-Gutmann bodies are positive with von Kossa, iron, and periodic acid-Schiff stains. These findings support a diagnosis of malacoplakia. The differential diagnosis includes granular cell tumor, poorly differentiated carcinoma, and Langerhans histiocytosis. Patients are managed with antibiotic therapy and excision. CONCLUSIONS: Tongue malacoplakia is rare, often presenting as a mass lesion. Histologic recognition of this abnormal phagocytic disorder will prevent potentially disfiguring surgery.

Cutaneous malakoplakia.

Malakoplakia is an acquired granulomatous disorder first described by Michaelis and Gutmann in 1902. The pathogenesis of malakoplakia is poorly understood, but it is thought to be secondary to an acquired bacteriocidal defect in macrophages occurring mostly in immunosuppressed patients or in the setting of autoimmune disease. Malakoplakia has been described in numerous anatomic locations, most commonly in the genitourinary tract. Microscopically, malakoplakia consists predominantly of sheets of macrophages known as von Hansemann cells with scattered targetoid intracytoplasmic inclusions known as Michaelis-Gutmann bodies. Cutaneous malakoplakia is a rare entity with less than 50 cases reported in the literature. In this article, we review cutaneous malakoplakia including the clinical, gross, and microscopic features as well as the treatment and prognosis of 40 cases of cutaneous malakoplakia identified in the literature.

[Pulmonary malakoplakia and Rhodococcus equi infection. A report of two cases and review]. / Malakoplakia pluca i zakazenie Rhodococcus equi. Opis dwóch przypadków i przeglad pismiennictwa.

Malakoplakia is a rare, chronic, granulomatous disease seen in immunocompromised patients, caused by multiple infectious agents, most often bacteria. Microscopically, accumulations of macrophages with granular cytoplasm and intra- or extracellular structures, called Michaelis-Gutmann bodies are characteristic. Main cause of pulmonary malakoplakia is Rhodococcus equi infection. We report two cases of pulmonary malakoplakia, in one case R. equi infection was confirmed. We also present a summary of recent knowledge about the pathogenesis, morphology and clinical course of the disease.