Essay Example on Malakoplakia of Gall Bladder Case report and review Introduction









Malakoplakia of Gall Bladder Case report and review Introduction Malakoplakia MP is a rare granulomatous disorder mostly involving urinary tract Few cases are reported about malakoplakia in gallbladder GB It morphologically mimics other lesions like xanthogranulomatous cholecystitis as well as malignancy 1 We report a case of gall bladder wall thickness clinically suspected to be carcinoma and then revealed as malakoplakia We will discuss the condition in the context of other causes of gallbladder wall thickness Case Report A 65 year old nondiabetic female patient presented with a complaint of upper abdominal discomfort Abdominal examination showed palpable non tender mass in the right hypochondrium Signs and symptoms of acute pancreatitis were negative Hemogram liver function tests renal function tests Serum amylase and serum lipase were within normal levels Ultrasonography whole abdomen revealed multiple stones contracted gall bladder with wall thickening and excluded presence of pancreatic and peripancreatic tissue edema Clinically Gall bladder carcinoma was suspected Open cholecystectomy was performed and gall bladder specimen was referred to histopathological examination HPE Grossly GB specimen was measured 10x6x4 cm with thickened wall Dissection showed greenish velvety mucosa with areas of ulceration obliteration of the cavity by diffuse nodules with yellowish discoloration and impacted three blackish stones Figure 1 HPE was done after staining the paraffin blocks with hematoxylin and eosin Sections examined from gall bladder wall revealed subtotally denuded mucosal lining with thickening of the wall 

Ulcerated area and lamina propria showed sheets of foamy macrophages with rounded concentrically layered intracytoplasmic inclusions Michaelis Gutmann bodies Figure 2 Rokitansky Aschoff sinus was also noted There was no evidence of granuloma polyps dysplasia or malignancy The characteristic Michaelis Gutmann bodies were stained positively for periodic acid Schiff stain Figure 3 The patient's postoperative period was uneventful She was given broad spectrum antibiotics She is symptom free and on regular follow up Discussion Malakoplakia from Greek Malako soft Plako plaque was first described in 1902 by Michaelis and Gutmann as a rare chronic granulomatous disorder most commonly affecting the urinary system1 However it was discovered also to affect a wide range of tissues extraurinary malakoplakia remains an occasional diagnosis2 4 There are few reported cases of malakoplakia in the gall bladder wherein it is usually presented as wall thickness as in our case or mass lesion and open cholecystectomy is indicated due to diagnostic suspicion of carcinoma as in our case5 

Grossly Malakoplakia specimens reveal a soft yellowish mass On routine staining microscopic examination shows aggregates of histiocytes with fine eosinophilic granular cytoplasm von Hansemann cells admixed with intracellular and extracellular basophilic discrete inclusions with concentric laminations known as Michaelis Gutmann bodies in a background of mixed inflammatory cells infiltrating stroma These bodies also demonstrate positive results using periodic acid Schiff stain They stain with von Kossa stain for calcium and Perls Prussian blue stain for iron1 HPE is necessary to differentiate MP from its mimickers Xanthogranulomatous cholecystitis and malakoplakia that can present in a similar pattern Both are thought to be part of spectrum of chronic inflammatory pathology with difference that malakoplakia is more aggressive and shows the presence of both intracellular and extracellular Michaelis Gutmann bodies as in our case Gallbladder involvement in autoimmune pancreatitis as a part of IgG4 associated systemic disease is also a common mimicker In our case this was excluded by lack of characteristic laboratory investigations and by the absence of characteristic histopathological findings of this disease Although more than a century has passed since Malakoplakia s original recognition the exact pathogenesis has not been fully recognized Malakoplakia is thought to result from insufficient lysis of bacteria by macrophages Experimental findings suggest that the defective phagolysosomal activity may be due to decreased intracellular concentration of cGMP which results in deficient fusion of lysosomes with phagosome6 Partially digested bacteria accumulate in monocytes or macrophages and lead to the deposition of calcium and iron on residual bacterial glycolipid resulting in accumulation of inclusion structures in cytoplasm of histiocytes the Michaelis Gutmann bodies which are considered to be pathognomic of malakoplakia3 7 

Another explanation suggested that the defect may exihibit the expression of a genetic disorder or an improper immune response like that observed in alcohol abuse malnutrition post organ transplant immunosuppressive drugs such as steroids or cytotoxic agents malignancy and chronic diseases such as diabetes mellitus and autoimmune disease2 Malacoplakia has been described also in association with several microorganisms E coliis usually seen in cases of urinary MP Rhodococcus aequi in patients with AIDS infection and pulmonary MP and Herpes simplex virus in young patients with cerebral MP Other bacteria i e Proteus Mycobacterium Shigella Klebsiella Staphylococcus Streptococcus Pseudomonas fungi i e Paracoccidiodes and parasites have also been reported6 Development of malakoplakia in chronic cholecystitis could be observed in the background of xanthogranulomatous cholecystitis which is thought to result from unsuccessful phagocytosis of the bile material8 In our case patient was nondiabetic female free of any of reported risk factors the matter which refers to the possibility of multifactorial pathogenesis of this disorder Although malakoplakia is considered as a benign pathological process that requires local excision it has been observed that many of these cases have aggressive course including high disease recurrence with fistulisation and poor response to antibiotics9 More specific therapy with antibiotics that concentrate in macrophages such as quinolone and trimethoprim sulfamethoxazole is associated with a high cure rate10 Bethanechol a choline agonist has been recommended in combination with antibiotics and surgery with the principle that Bethanechol may correct the decreased cGMP levels that are believed to interfere with successful phagolysosomal activity Ascorbic acid has been used also to increase the cGMP and cAMP levels in monocytes which may offer an effective strategy for therapy though it is still under trial11 Discontinuation of immunosuppressive drug therapy is usually needed to treat malakoplakia effectively12 Attempts of curative surgery would appear to be supported in all cases of gall bladder MP and its mimickers gall bladder carcinoma and xanthogranulomatous cholecystitis due to the difficulty of distinguishing them radiologically

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