Malakoplakia of the Bladder- Causes, Symptoms, and Treatment

Introduction:

Malakoplakia is a rare inflammatory condition that occurs in certain immunocompromised people and is secondary to a defective function of the white blood cells. It is a granulomatous disease- a disorder in which the white blood cells are not able to kill certain microbes. The name derives from the Greek words malakos (soft) and plakos (plaque). It was first described by Von Hansemann in 1901 and 1902 by Michaelis and Gutmann. Malakoplakia usually affects the genitourinary tract; however, nearly every other organ seems to be involved with the disorder.

The first case of malakoplakia that occurred outside the genitourinary system was in 1958.

What Is Bladder Malakoplakia?

The urinary bladder is a sac-like organ that stores the urine prepared by the kidneys. Bladder malakoplakia is a rare granulomatous (inflammatory) disease. It was first reported by Michaelis and Gutmann in 1902. The cause of the disease is difficult to comprehend, so the diagnosis becomes a little erroneous sometimes. The urinary tract is the most common site of malakoplakia. Wielenberg et al. found eighty-nine cases involving the urinary tract among one hundred and three patients. Next to the bladder, other standard sites are the kidneys, ureters, and renal pelvis.

What Is the Epidemiology Of Bladder Malakoplakia?

Malakoplakia of the bladder is more prevalent in females than males, with a ratio of approximately four to one. It is also common in older patients over fifty years of age.

What Causes Bladder Malakoplakia?

The exact cause of bladder malakoplakia is not clear. However, several theories have been suggested to explain the reason for malakoplakia:

• Bacterial Infection: Bacteria such as E. coli, Proteus, and Mycobacterium tuberculosis cause infection. The localized environment of the urinary bladder is suitable for the growth of bacteria and leads to an inflammatory response within the bladder.

• Immunocompromised Patients: An altered immune response and immunocompromised individuals are prone to the disease. Long-term chronic diseases such as HIV infection, tuberculosis, sarcoma, diabetes, and ulcerative colitis make the individual susceptible.

• Defective White Blood Cells (Macrophages): The phagocytosis (bacteria-killing) and digestion functions of the macrophages are abnormal. They can engulf the bacteria but cannot digest it fully. Partially digested bacteria accumulate within the macrophages, leading to the deposition of calcium and iron on the cell wall of the bacteria, forming calcified intracellular bodies called Michaelis-Gutmann bodies.

What Are the Symptoms Of Bladder Malakoplakia?

Malakoplakia can be asymptomatic. The different symptoms include:

• Hematuria: This is the presence of blood in the urine.

• Urinary Tract Infection: Infections in any region of the urinary system- the kidney, ureters, urinary bladder, or the urethra.

• Septicemia: This is the poisoning of blood by bacteria.

• Hydronephrosis: This is the swelling of the kidneys due to urine accumulation.

• Acute Renal Failure: This is a sudden failure of the kidneys that happens within a few hours to a few days.

• End-stage Kidney Disease: In this condition, the kidneys gradually lose their function, reaching an advanced stage.

How Is Bladder Malakoplakia Diagnosed?

Urine Culture- A urine culture is a test to check for the presence of microbes like bacteria or other germs in urine. It is generally used to diagnose urinary tract infections in adults and kids.

1. Imaging Techniques:

• Positron Emission Tomography (PET) Scanning- A positron emission tomography scan is an imaging test that reveals the metabolic or biochemical (chemical process occurring inside a living organism) function of the tissues and organs. It uses a radioactive drug called a tracer to show normal as well as abnormal metabolic activity. For example, increased uptake of the tracer was found in the bladder during the scan, diagnosing malakoplakia of the bladder.

• Magnetic Resonance Imaging (MRI)- This technique uses a combination of magnetic resonance, sound waves, and computer technology to produce images of the organs. MRI of bladder malakoplakia has shown several nodules.

2. Cystoscopy:A cystoscopy is a procedure to look inside the bladder with the help of a thin camera called cystoscopy. The cystoscopy is inserted into the urethra (a tube that carries urine from the bladder to outside the body) and the bladder for the doctor to see. The bladder appears small and contracted with several masses of yellow or reddish areas.

3. Histological Examination: Histological findings of Michaelis-Gutmann bodies.

How Is Bladder Malakoplakia Treated?

Malakoplakia is rare, and there are no standard treatment guidelines. Treatment guidelines include antibiotics, surgical removal, and restricting immunosuppression.

• Antibiotics: Antibiotics that enhance the killing of bacteria by white blood cells successfully treat malakoplakia. Antibiotics such as fluoroquinolones, Trimethoprim, and Rifampin. The duration of the treatment is unknown, but long-term antibiotic therapy is generally recommended.

• Surgery: If medications fail, surgical removal of malakoplakia should be considered. The combination of surgery and quinolone antibiotic therapy might be most effective.

• Restricting Immunosuppression: Malakoplakia is associated with immunosuppression in case of organ transplantation or connective tissue diseases. Prednisone and Azathioprine have been associated with decreased bacterial killing by white blood cells. Malakoplakia has been found to improve with the discontinuation of immunosuppression.

What Are the Differential Diagnosis of Bladder Malakoplakia?

The differential diagnosis includes:

• Tuberculosis (an infectious condition that typically affects the lungs).

• Lepromatous leprosy (a type of leprosy marked by light-colored skin lesions).

• Leishmaniasis (a parasitic disease).

• Condyloma lata (skin ailment that causes wart-like sores on the genitalia).

• Sarcoidosis (a disorder where the immune system reacts inappropriately and forms granulomas, which are lumps or nodules).

• Xanthomas (specific lipid accumulations inside an organ system).

• Lymphoma (cancer that starts in lymphocytes, which are immune system cells that fight infections).

What Are the Complications of Bladder Malakoplakia?

The complications of malakoplakia depend on the site. For example, malakoplakia of the urinary tract may lead to acute kidney injury, recurrent urinary tract infections, or renal failure.

What Is the Prognosis of Bladder Malakoplakia?

The prognosis of bladder malakoplakia varies from person to person. The condition, in some instances, has resolved after antibiotic therapy with no recurrence. While in other cases, disease recurrence has been found to cause life-threatening complications. Regular follow-up is essential to keep a check on any possible recurrence of the condition.

Conclusion:

Bladder malakoplakia is a rare chronic inflammatory disease with ambiguous clinical features and high rates of imprecise diagnosis. The causes are very vague and not understood fully. Malakoplakia was thought to be a correctable and relatively benign or non-cancerous disease, but in certain cases, it might result in renal failure. A definite diagnosis depends on identifying the Michaelis-Gutmann bodies. The treatment involves long-term antibiotic therapy. Bladder tumor removal can be combined with medical management to achieve a better therapeutic effect. Early diagnosis and appropriate antibiotic therapy are important in patients with Malakoplakia. A regular follow-up is a must to avoid any future advancement to severe kidney disease.