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Malakoplakia of liver diagnosed by a needle core biopsy; a case report and review of the literature. (Case Reports).

Reports of malakoplakia of the liver are rare. A computer search of the literature yielded only 4 reported cases to date. (1-4) Unlike previously reported cases of malakoplakia of the liver, to our knowledge, this is the first reported case in the English-language literature of malakoplakia of the liver that was diagnosed by a needle core biopsy. It was associated with small bowel obstruction due to ileus following Klebsiella pneumonia sepsis that occurred after aspiration.


This case occurred in a 19-year-old Hispanic man with a history of suspected mucopolysaccharide disorder with resulting neurodegenerative condition. He exhibited spastic paraparesis and profound mental retardation. He presented with nausea, vomiting, diarrhea, and abdominal distention due to ascites. He underwent paracentesis and removal of 5 L of fluid. Analysis of peritoneal fluid showed a white blood cell count of 6790, a red blood cell count of 254, 76% neutrophils, and 3% lymphocytes. Blood chemistry analysis showed the following values: blood urea nitrogen, 38; creatinine, 2.5; aspartate aminotransferase, 154; alanine aminotransferase, 69; alkaline phosphatase, 438; total bilirubin, 1.9; white blood cells, 15.7 with 68% neutrophils and 20% bands; hemoglobin, 10.4; and platelets, 216 000. A computed tomographic scan of the abdomen revealed cirrhosis and portal hypertension with dilated esophageal varices. The patient was given spironolactone and furosemide. The results of hepatic serologic tests for hepatitis C virus, hepatitis B virus, [[alpha].sub.1]-antitrypsin, antinuclear antibody, and ceruloplasmin were all negative. A needle core biopsy of the liver was performed. Despite supportive measures, the patient's abdominal distention persisted and he became increasingly lethargic. A chest x-ray film revealed left lobar pneumonia. He was immediately given ampicillin and sulbactam, but his respiratory status continued to worsen and he subsequently went into cardiorespiratory arrest. Permission for autopsy was denied.


Histologic sections of the liver needle core biopsy specimen showed cirrhotic hepatic parenchyma with bile ductular proliferation and portal inflammatory infiltrates composed of lymphocytes, neutrophils, and a few scattered eosinophils. Rare isolated bile ductules were associated with neutrophils consistent with cholangitis. Scattered within periportal and portal regions were small, round-to-oval targetoid structures consistent with Michaelis-Gutmann bodies (Figure, A). These stained positively with periodic acid-Schiff (with and without diastase) (Figure, B), Von Kossa calcium (Figure, C), and colloidal iron stain (with and without hyaluronidase) (Figure, D), but were negative for copper and [[alpha].sub.1]-antitrypsin. Immunohistochemistry with CD68 (Figure, E) demonstrated these targetoid bodies within scattered histiocytes and Kupffer cells. Electron microscopy confirmed the presence of Michaelis-Gutmann bodies (Figure, F) within histiocytes and failed to show any evidence of mucopolysaccharidosis. The hepatocyte cytoplasm was neither pale nor reticulated as would be expected in mucopolysaccharidosis.



Malakoplakia, a Greek term meaning soft (malako) plaque (plakia), is an unusual inflammatory process. It was originally described in the early 1900s. Although the original article was published by Michaelis and Gutmann in 1902, its discovery is best attributed to their senior colleague, Von Hanseman, even though his article was published a year later. (5)

Malakoplakia was first reported and is most commonly seen in the bladder. It has since been described in other locations, such as the colon, upper and lower genitourinary tract, gynecologic regions, upper and lower respiratory tract, spleen, joints, and brain. It has rarely been reported to occur in the liver This case is the fifth reported case of malakoplakia of the liver and the first to be diagnosed by a needle core biopsy (Table). Two other cases involved the liver through extension from the kidney and urinary tract.

Malakoplakia is usually associated with Klebsiella and Escherichia coli, although gram-negative and gram-positive cocci and acid-fast bacilli have also been identified. Recently, malakoplakia has been reported to occur in association with unusual organisms such as Rhodococcus equi and paracoccidioidomycosis infection. (6)

There is usually an underlying systemic disease in malakoplakia. Some of the reported associated systemic diseases include systemic lupus erythematosus, diabetes mellitus, myotonic dystrophy, and chronic active hepatitis. Immunosuppression is also common, but malakoplakia is not as common as one would expect in acquired immunodeficiency syndrome, which may be due to a relative or selective preservation of antimicrobial function of monocytes.

Malakoplakia has a distinct gross and microscopic appearance. In urothelial and colonic mucosa, where they are frequently seen, lesions of malakoplakia are characterized by variably sized yellow-to-brown plaques that are usually soft, often with central erosion and peripheral hyperemia. (7) The lesions of malakoplakia may be solitary, multifocal, or present as a large mass.

Microscopically, malakoplakia is characterized by aggregates of histiocytes, the Von Hanseman cells, with Michaelis-Gutmann calcospherites. Surrounding inflammatory cells usually consist of lymphocytes and plasma cells. There are 3 phases in the histopathologic evolution of malakoplakia, as described by Smith. (8) The early phase consists of Von Hanseman histiocytes and plasma cells in an edematous stroma. The granulomatous phase displays many lymphocytes and plump histiocytes with typical Michaelis-Gutmann calcospherites. The fibrosing or healing phase is characterized by interwoven fibroblasts and strands of thickened collagen admixed with histiocytes. Michaelis-Gutmann bodies are sparse.

The most plausible pathogenetic explanation for the development of malakoplakia centers on the defective function of macrophages that phagocytose the bacteria but are unable to kill or digest them. Intracytoplasmic phagolysosomes continue to enlarge and coalesce with subsequent degenerative change of both matrix and limiting membrane. The deposition of crystals within this enlarged phagolysosome leads to the development of the characteristic, concentrically laminated Michaelis-Gutmann inclusions. (9)

Other authors propose that the defect in malakoplakia may be due to low levels of cyclic guanosine monophosphate in mononuclear cells, resulting in deficient lysosomal bacterial degradation and the inability of cells to release the lysosomal enzymes. Correction of this defect with cholinergic agonists would correct the lysosomal deficiency and prove therapeutically beneficial. (10)

In summary, this case occurred in a 19-year-old man with a cirrhotic liver of uncertain origin, who subsequently developed malakoplakia of the liver following sepsis that resulted from Klebsiella lobar pneumonia. It is the fifth reported case in the medical literature of malakoplakia that occurred primarily in the liver and, to our knowledge, the first to be diagnosed by a needle core biopsy. A surgical procedure, which comparatively has more potential for complications than a needle core biopsy, may be avoided in some cases. Complete workup and diagnosis of malakoplakia are possible even in specimens as small as those obtained with a needle core biopsy.
Reports of Malakoplakia of Liver

 Source, y Age/Sex Underlying Disease Material

Moldavski and 34/Female Miliary tuberculosis Autopsy
 Rustamov, 1984 with hepatic
De Saint-Maur and 68/Female Infected polycystic Segmentectomy
 Gallot, 1990 liver
Robertson 54/Female Liver abscess following Wedge biopsy
 et al, 1991 immunosuppression for
 systemic lupus
Boucher et al, 1994 43/Male Perforated colonic Segmentectomy
Present case, 2000 19/Male Small bowel ileus Needle core
 following Klebsiella biopsy

We acknowledge Raoul Fresco, MD, who interpreted the material that was submitted for electron microscopic studies and for his translation of a French text. We also acknowledge Elena Roukhadze, MD, for her translation of a Russian text.


(1.) Boucher L, Makoto A, Lee E, Cibull M. Malakoplakia of liver associated with perforated colonic diverticulum: a case report and review of the literature. J Clin Gastroenterol. 1994;19:318-320.

(2.) Robertson S, Higgins R, Powell C. Malakoplakia of liver: a case report. Hum Pathol. 1991;22:1294-1295.

(3.) Moldavski M, Rustamov I. Multichamber pseudocyst of the liver with malakoplakia [in Russian]. Kiln Khir. 1984;9:64-65.

(4.) De Saint-Maur P, Gallot D. Malakoplakie hepatique compliquant une polykystose [letter]. Ann Pathol. 1990;10:50-51.

(5.) Damjanov I, Moriber Katz S. Malakoplakia [review]. Pathol Ann. 1981;16(pt 2):103-126.

(6.) Rocha N, Suguiama EH, Maia D, et al. Intestinal malakoplakia associated with paracoccidioidomycosis: a new association. Histopathology. 1997;30:79-83.

(7.) Stanton M, Maxted W. Malakoplakia: a study of the literature and current concepts of pathogenesis, diagnosis, and treatment [review]. J Urol. 1981;125: 139-146.

(8.) Smith B. Malakoplakia of the urinary tract: a study of twenty-four cases. Am J Clin Pathol. 1965;43:409-417.

(9.) Lou T, Teplitz C. Malakoplakia: pathogenesis and ultrastructural morphogenesis: a problem of altered macrophage phagolysosomal response. Hum Pathol. 1974;5:191-207.

(10.) Abdou N, Napombiyare C, Sagawa A, et al. Malakoplakia: evidence for monocyte lysosomal abnormality correctable by cholinergic agonist in vitro and in vivo. N Engl J Med. 1977;297:1413-1419.

Accepted for publication August 3, 2001.

From the Department of Pathology, Loyola University Medical Center, Maywood, Ill. Dr Yong is now with Northwest Community Hospital, Arlington Heights, Ill. Dr Chejfec is now with the University of Illinois at Chicago.

Reprints: Grace Hartman, MD, Department of Pathology, Loyola University Hospital, 2160 S First Ave, Maywood, IL 60153 (e-mail:
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Author:Hartman, Grace; Yong, Sherri L.; Chejfec, Gregorio
Publication:Archives of Pathology & Laboratory Medicine
Geographic Code:1USA
Date:Mar 1, 2002
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