Malakoplakia of the bladder associated with advanced obstructive

CASE REPORT
Malakoplakia of the bladder associated
with advanced obstructive uropathy
Konstantinos Stamatiou1, Georgios Makris1, Dimitris Zavradinos1,
Eleutherios Geropappas1, Konstantinos Fokas1, Athanasios Papatsoris2.
1. Urology Department, Tzaneio General Hospital, Piraeus, Greece
nd
2. 2 Department of Urology, University of Athens, Athens, Greece
Corresponding Author:
Konstantinos Stamatiou
Urology Department, Tzaneio General Hospital, Piraeus, Greece
Salepoula 2, 18536, Piraeus - Tel: +30 2104592387
e-mail: [email protected]
Summary
Malakoplakia is an inflammatory condition that
rarely occurs in the urogenital tract. The most
frequently affected organ is the urinary bladder,
while involvement of the testis is extremely rare.
This condition has the features of a granulomatous
inflammation. In this article we present a case of
bladder malakoplakia associated with advanced
obstructive uropathy.
Key Words
Malakoplakia, inflammatory disease, urinary bladder, kidney failure.
Introduction
Malakoplakia is a granulomatous inflammation
which was originally described in 1902 by Michaelis
and Gutmann. Malakoplakia most often occurs in
patients above 40 years old, and usually in women.
Symptoms in the affected bladder are not specific
and are similar to cystitis1. The pathogenesis of
malakoplakia remains poorly understood, though it
is speculated to be caused by defective
macrophage elimination of bacteria.
Defective macrophage killing results in an
accumulation of bacterial degradation products
and a granulomatous reaction, which clinically
manifests as the formation of a papule, plaque or
ulceration. It is hypothesized that the disease
occurs in patients with history of E. coli infections,
immunosuppression (usually connected to kidney
transplantation and in rare cases due to lymphoma
or cancer), diabetes, and chronic steroid use,
although the relationship has not been clearly
described. Macroscopically, on the internal surface
of the affected organ, yellow- soft plaques are
detected, whilst microscopically the disease is
characterized by the presence of large
macrophages (foamy hystiocyotic cells-also
known as von Hansemann cells) containing
pathognomonic Michaelis-Gutmann bodies.
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Case report description
A male, 72 years old was brought to the Emergency
Department (E.R) reporting inability to urinate and
fever. He also reported weakness, fatigue and
dysuria all starting a month ago. The patient's
medical history was relevant for diabetes,
hypertension and peripheral vascular disease. His
past surgical history was relevant for a transvesical
prostatectomy 3 years ago, due to urinary
retention. This occurred after a significant history of
recurrent urinary tract infections. During their
evaluation a bladder biopsy of a membranous
mucosal lesion was performed indicating
nonspecific inflammation.
Upon admission the patient presented with
increased creatinine and glucose levels (21mg/dl,
300 mg/dl respectively). Anemia (Hct: 19%) with a
normal white cell count however was noted.
Ultrasound of the urinary system revealed bilateral
hydronephrosis due to obstructive uropathy
indicated by bladder wall thickening and
trabeculation. Nevertheless bladder capacity was
minimal with no residual urine observed (Image 1).
Image 1.Transabdominal ultrasonography: abnormally
thick bladder wall with the presence of exophytic lesion
Urine analysis indicated hematuria, pyuria and
microorganisms. Digital rectal examination
Malakoplakia of the bladder associated with advanced obstructive uropathy
revealed a small benign prostate. After immediate
antibiotic initiation and fluid replacement the
patient underwent initially hemodialysis due to the
presence of hyperkalemia, following nephrostomy
tube placement, which successfully stabilized renal
function.
Cystoscopic evaluation depicted atypical polypoid
lesions of the mucosa and scattered red-yellow
nodules sized 2 to 3.5cm in the trigone, left and
posterior bladder wall. A transurethral resection of
the lesions followed. Histology revealed malakoplakia of the urinary bladder (image 2-5). Despite
initial patient stabilization the patient died 8 months
later due to renal failure and complications of the
cardiovascular system since he continued to
present with urinary tract infections and chronic
hydronephrosis.
bioptic material with an absence of microscopic
pathognomonic findings such as acidophilic foamy
hystiocytic cells, or Michaelis-Gutmann bodies. In
these cases the emergence of positive results for
CD68 immunohistochemical staining confirms the
clinical hypothesis5. This disease is frequently
considered an auto-immune disorder or a type of
immunodeficiency5. Even though this inflammatory
disease is chronic, in certain cases progresses
acutely.
Image 3. Malakoplakia with the use of a PAS stain in von
Hausemann Çystiocyotic cells.
Image 2. Pathognomonic histological image of malakoplakia of the urinary bladder. (HEX 100).
Discussion
Malakoplakia is a benign self-limiting disease that
usually has a benign course 3. Initial management
consists of treating aggressively associated urinary
tract infections and transurethral removal of the
lesion mainly for histologic diagnosis. Few
incidents of renal impairment, caused by multifocal
malakoplakia, are reported in the literature. Indeed,
when the symptoms are severe, the disease is
promptly diagnosed4. The reason for the delayed
diagnosis in the present case remains unknown.
As the symptoms were chronic, we hypothesize
that the bladder was already affected during the
first biopsy. Failure to diagnose the disease may
have occurred due to an insufficient amount of
In summary, malakoplakia of the bladder should be
carefully examined in patients with persistent infections of the urinary system and the presence of a
tumor-like mass in the cystoscopy. A numerous of
potential complications can be avoided with earlystage diagnosis and immediate antibiotic treatment.
Image 4. Malakoplakia of the Urinary Bladder
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55
K. Stamatiou, G. Makris, D. Zavradinos, E. Geropappas, K. Fokas, A. Papatsoris
Ðåñßëçøç
Ç ìáëáêïðëáêßá åßíáé ìéá öëåãìïíþäçò êáôÜóôáóç ðïõ óðÜíéá åìöáíßæåôáé óôïí ïõñïãåííç-
Ìáëáêïðëáêßá ôçò ïõñïäü÷ïõ êýóôçò
óõíäåüìåíç ìå ðñï÷ùñçìÝíç áðïöñáêôéêÞ
ïõñïðÜèåéá.
Kùíóôáíôßíïò Óôáìáôßïõ1, Ãåþñãéïò ÌáêñÞò1,
ÄçìÞôñçò Æáâñáäéíüò1, ÅëåõèÝñéïò Ãåñüðáððáò1,
Êùíóôáíôßíïò ÖùêÜò1, ÁèáíÜóéïò Ðáðáôóþñçò2
ôéêü óýóôçìá. Óõ÷íüôåñá ðñïóâáëëüìåíï üñãáíï åßíáé ç ïõñïäü÷ïò êýóôç, åíþ åßíáé åîáéñåôéêÜ
óðÜíéá ç åìðëïêÞ ôïõ üñ÷åïò. Ç ðÜèçóç Ý÷åé ôá
÷áñáêôçñéóôéêÜ ìéáò êïêêéùìáôþäïõò öëåãìïíÞò. Óôï ðáñüí Üñèñï ðáñïõóéÜæïõìå Ýíá ðåñéóôáôéêü ìáëáêïðëáêßáò ôçò ïõñïäü÷ïõ êýóôçò
1
ÏõñïëïãéêÞ KëéíéêÞ, ÔæÜíåéï Ãåíéêü Íïóïêïìåßï ÐåéñáéÜ
óõíäåüìåíçò ìå ðñï÷ùñçìÝíç áðïöñáêôéêÞ ïõ-
2
 ÐáíåðéóôçìéáêÞ ÏõñïëïãéêÞ ÊëéíéêÞ, Óéóìáíüãëåéï
ñïðÜèåéá.
Ãåíéêü Íïóïêïìåßï Áìáñïõóßïõ
Õðåýèõíïò åðéêïéíùíßáò: Kùíóôáíôßíïò Óôáìáôßïõ
ÏõñïëïãéêÞ êëéíéêÞ, Ôæáíåéï ãåíéêü Íïóïêïìåßï ÐåéñáéÜ
Óáëåðïýëá 2, 18536 ÐåéñáéÜò
ôçë: 2104592387 - e-mail: [email protected]
ËÝîåéò Åõñåôçñéáóìïý: Ìáëáêïðëáêßá, öëåãìïíþäçò íüóïò, ïõñïäü÷ïò êýóôç, íåöñéêÞ áíåðÜñêåéá.
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