Description, Causes and Risk Factors:
Malakoplakia is a rare inflammatory condition most often affecting the genitourinary system. Malakoplakia has been reported in various sites, but in majority of cases it involves the urinary tract. In the genitourinary form of the disease, the bladder is most commonly affected, followed by isolated upper urinary tract lesions and combined upper and lower tract lesions. It is more common in females with a peak of incidence around 50 years of age. Malakoplakia is very rare in children and its incidence is probably underestimated in adults.
Malakoplakia seems to be due to an impaired response to bacterial infection. Macrophages and monocytes show a defective phagolysosomal activity: they phagocytose bacteria but are unable to digest them completely. Partially digested bacteria accumulate in the cytoplasm and lead to a granulomatous reaction of the immune cells. It has been suggested that the defective phagolysosomal activity could be due to a decrease in the concentration of intracellular cyclic guanosine monophosphate (cGMP).
It has been suggested that malakoplakia may be an expression of microtubular/microfilamental dysfunction. The intracellular system of microtubules and microfilaments is thought to be concerned with several cellular functions, including active movement. In the cases reported herein, malakoplakic cells were noted in drainage lymph and there had also been extension into the retroperitoneum. One wonders if active movement is a component of these extension of the malakoplakic process, and, if it is, then that aspect of microtubule/microfilament function wound not be disordered.
People whose immune systems are weakened, such as those suffering from leukemia, diabetes, or those who have undergone organ transplants, are most likely to experience this inflammatory condition.
Symptoms of malakoplakia, especially in the genitourinary lining, can be hard to detect, as the disease develops internally. Aside from the yellow sores, a person can experience an inflammation in the colon, manifested by diarrhea or some stomachache. Inflammation in the kidney also creates a similar stomach pain on the side, while the rarer malakoplakia on the neck and tongue is more apparent with pink or yellow bumps on the skin and on the tongue.
There is no imaging technique that can diagnose malakoplakia with certainty. Ultrasound may demonstrate renal dilatation or hypereechoic lesions, while the bladder may be small and contracted with soft tissue masses. CT scan and magnetic resonance imaging (MRI) findings are also nonspecific. Malignant bladder tumors represent the main differential diagnosis.
The diagnosis is exclusively histological after biopsy or TURB. Malakoplakia is characterized by aggregates of large macrophages with foamy eosinophilic cytoplasm that are present at the sties of infection and exhibit numerous secondary lysosomes. The fusion and calcification of these lysosomes result in the formation of intracytoplasmic crystalline bodies with a central hydroxyapatite core that give macrophages a concentric target-like appearance.
Usually, the sores associated with malakoplakia are chronic, meaning they may remain in the body for a long time. The good thing is that it does not largely affect the patient or weaken him in any way, but an immediate treatment may be recommended to avoid further complications.
Treatment consists in antibiotherapy that uses intracellular molecules (fluoroquinolones, trimethoprim and sulfamethoxazole). Currently, the duration of therapy is still not standardized. In pseudotumoral cases, surgical removal of the lesions is required.
Prognosis is usually good. However, recurrences and complications may occur over the years such as renal failure in case of urinary tract involvement.
NOTE: The above information is educational purpose. The information provided herein should not be used during any medical emergency or for the diagnosis or treatment of any medical condition.
DISCLAIMER: This information should not substitute for seeking responsible, professional medical care.
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