|Year : 2022 | Volume
| Issue : 4 | Page : 596-598
A rare case of malakoplakia of the urinary bladder
Pranav Raja Yadav, Piyush Singhania, Piyush Sahu
Department of Urology, MGM Medical College and Hospital, Navi Mumbai, Maharashtra, India
|Date of Submission||25-Feb-2022|
|Date of Acceptance||07-Dec-2022|
|Date of Web Publication||29-Dec-2022|
Prof. Piyush Singhania
Department of Urology, MGM Medical College and Hospital, Kamothe, Navi Mumbai 410209, Maharashtra
Source of Support: None, Conflict of Interest: None
Malakoplakia (from Greek Malako “soft” + Plako “plaque”) is a rare inflammatory condition that presents as a papule, plaque, or ulceration that usually affects the genitourinary tract. The most frequently affected organ is the urinary bladder. This condition has features of granulomatous inflammation. The pathogenesis of the disease is not completely understood. It is often misdiagnosed as malignancy. In this case report, we present a case of urinary bladder malakoplakia which presented with obstructive uropathy and acute kidney injury (AKI).
Keywords: Malakoplakia, rare inflammatory condition, uropathy
|How to cite this article:|
Yadav PR, Singhania P, Sahu P. A rare case of malakoplakia of the urinary bladder. MGM J Med Sci 2022;9:596-8
| Introduction|| |
Malakoplakia was first described by Michaelis and Gutmann in 1902 and later named by Von Hansseman. It is more common in immunocompromised (HIV, renal transplant recipients, long-term therapy with systemic corticosteroids) patients and women. The mean age at diagnosis is the fifth decade. It has been proposed that it is caused by defects in phagocytic or degradative functions of histiocytes in response to gram-negative coliforms (E. coli or Proteus) that results in a chronic inflammatory state, followed by intracellular deposition of iron and calcium (known as Michaelis-Gutmann bodies). Clinically, malakoplakia can mimic bladder tumors. However, the typical cystoscopy appearance of the affected bladder is characterized by yellow soft tiny plaques.
| Case presentation|| |
A 73-year-old Indian man came to the emergency department with complaints of fever, weakness, and irritative voiding symptoms for the last month. His routine investigations showed raised serum creatinine value of 6.0 mg/dl and Hb of 8g/dl, a total leucocyte count of 13,000/cumm, and a Potassium of 7.0meq/l. Urinalysis revealed hematuria, pyuria, and bacteriuria He was diabetic and taking oral medication. His Ultrasound of the abdomen showed growth in his urinary bladder (5.4 x 3.0 cm) with focal thickening in the right lateral wall and increased vascularity. CT urography suggested an abnormal heterogeneously enhancing soft tissue lesion in the urinary bladder with associated diffuse irregular mural thickening of the rest of the urinary bladder involving bilateral vesicouretric junctions and intravesical segments of bilateral ureters with resultant bilateral hydronephrosis (left>right), hydroureter and left perirenal and periuretric inflammatory changes [Figure 1].
In view of hyperkalemia at presentation, the patient was dialyzed and also underwent a Left Per cutaneous nephrostomy. The patient was put on broad-spectrum antibiotics.
After the S. Creatinine normalized, the patient was taken up for check cystoscopy and transurethral resection of bladder tumor (TURBT). The bladder was full of turbid urine and the mucosa was thickened with exaggerated folds. There were multiple scattered yellow nodules seen in the bladder.[Figure 2] TUR biopsy was taken and B/L DJ stenting was done.
Histopathology showed tissue comprising stroma and fibro collagenous tissue lined by thin urothelial lining. The stroma is comprised of loosely arranged plump and spindle myofibroblasts in edematous myxoid background. Also seen at places were dens of mononuclear infiltrate of plasma cells, lymphocytes, and histiocytes. Immunohistochemistry showed CD68 was positive. Classical signs of malakoplakia showing abundant Michaelis Gutman bodies and foamy granular histiocytes with PAS-positive granular eosinophilic cytoplasm were present in lamina propria along with lymphocytes [Figure 3] and [Figure 4].
|Figure 3: Collections of large histiocytes with granular eosinophilic cytoplasm: Von Hansemann cells (PAS stain)|
Click here to view
|Figure 4: Michaelis–Gutmann bodies, (M-G bodies) which are concentrically layered basophilic inclusions found in Hansemann cells|
Click here to view
The patient was started on low-dose ciprofloxacin 250 mg once a day. After 3 weeks, the patient’s pyuria was not settling. So repeat urine culture was done and the antibiotic was changed to trimethoprim accordingly. The patient’s pyuria cleared but his creatinine levels increased again and he was put on routine maintenance hemodialysis. The patient was free of urinary symptoms but was having oliguria. Repeat USG showed B/L hydroureteronephrosis. There was a significant weight loss of 20kgs over 4 weeks. Because of these findings, the patient has advised B/L percutaneous nephrostomy but the patient refused any further treatment.
| Discussion|| |
Malakoplakia is an Inflammatory disease that affects the Genitourinary tract with a special affinity for the bladder. Given that malakoplakia is rare, large prospective trials are lacking and evidence regarding successful treatment is largely anecdotal. There are no standardized guidelines. Since symptoms are usually non-specific, diagnosis can be missed by many.
Failure in diagnosis might be due to insufficient biopsy material and concomitant absence of pathognomonic findings (such as microscopic acidophilic foamy histiocytes and Michaelis-Gutmann bodies). In these cases especially immunohistochemistry is useful.
Lesions can Undergo spontaneous regression in immunocompetent individuals for a period of 4 to 6 months. Long-term low-dose antibiotic treatment can result in regression of lesions in 3 months. After initial treatment with antibiotics if there are persistent plaques they can be surgically resected
Surgical excision of malakoplakia lesion should be considered based on on-site affected. Upper tract malakoplakia is usually aggressive. If renal parenchyma is involved nephrectomy is advised. For bladder involvement, TURBT, and subtotal/partial cystectomy may be required.
In patients treated with surgery with or without antibiotics, a cure rate of 81% is seen. However, the prognosis of bilateral involvement is poor with 100% mortality in six months irrespective of treatment choice.
| Conclusion|| |
We conclude by saying, malakoplakia should be suspected in patients presenting with recurrent UTIs, who have not responded to antibiotics and have suspicious features in imaging as well as cystoscopy. An early diagnosis and early treatment with antibiotics may be useful in preventing the development of possible complications.
Institutional Ethics Committee (IEC) of MGM Medical College and Hospital, Navi Mumbai, Maharashtra, India reviewed and approved the Case Study entitled: A rare case of malakoplakia of the urinary bladder in the IEC meeting held on 12 October 2022. Approval for undertaking the proposed Case Study has been communicated vide letter no. DHR-EC/2022/10/52 dated 10 November 2022.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
| References|| |
Michealis L, Gutmann C Ueber Einschlusse in Blasentumoren. Ztschr Kin Med 1902;47:208.
Darvishian F, Teichberg S, Meyersfield S, Urmacher CD Concurrent malakoplakia and papillary urothelial carcinoma of the urinary bladder. Ann Clin Lab Sci 2001;31:147-50.
Gupta R, Mahajan A, Atri S, Gupta CL Recurrent painless hematuria secondary to malacoplakia of the urinary bladder: A case report and review of literature. Urol J 2013;10:821-3.
Dhabalia JV, Nelivigi GG, Jain NK, Suryavanshi M, Kakkattil S Malakoplakia of the ureter: An unusual case. Indian J Urol 2008;24:261-2.
Kwan E, Riley CA, Robinson CA Malakoplakia. [Updated 2021 Nov 5]. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2022. Available from: https://www.ncbi.nlm.nih.gov/books/NBK564348/
Stanton MJ, Maxted W Malacoplakia: A study of the literature and current concepts of pathogenesis, diagnosis and treatment. J Urol 1981;125:139-46.
Stamatiou K, Chelioti E, Tsavari A, Koulia K, Papalexandrou A, Efthymiou E, et al
. Renal failure caused by malakoplakia lesions of the urinary bladder. Nephrourol Mon 2014;6:e18522.
Partin AW, Peters CA, Kavoussi LR, Dmochowski RR, Wein AJ Campbell Walsh Wein Urology. 12th ed, vol 1 (3 vols set). Philadelphia, PA: Elsevier; 2020. p. 1181.
Dong H, Dawes S, Philip J, Chaudhri S, Subramonian K Malakoplakia of the urogenital tract. Urol Case Rep 2015;3:6-8.
[Figure 1], [Figure 2], [Figure 3], [Figure 4]