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Diagnosis: Malakoplakia

Malakoplakia is the result of a defect in macrophage function causing impairment of bactericidal activity. The large macrophages that are present at sites of infection (von Hansemann cells) exhibit numerous secondary lysosomes containing partially digested organisms. Calcification of these lysosomes result in the formation of intracytoplasmic bodies called Michaelis–Gutmann bodies, considered pathognomonic of the disease. Renal parenchymal involvement may produce varying degrees of renal enlargement. Other relatively frequent described causes of massive bilateral non-cystic nephromegaly include renal infiltration by hematolymphoid diseases, nephroblastomatosis, megalocytic interstitial nephritis, xanthogranulomatous pyelonephritis and other infections. Six months after diagnosis, the patient is well, but nephromegaly continues and her serum creatinine is 0.7 mg/dL (creatinine clearance 33 mL/min). Prognosis of renal malakoplakia in children is variable: some patients recover completely, others develop end-stage renal disease and others may die.

This case was published (with other images) in Kidney International: Franco-Alzate C, Prada MC, Arias LF. Massive bilateral nephromegaly in an infant. Kidney International. 2012; 82(7): 828 [PubMed link].

See the chapter: Tubulointerstitial Diseases of our Tutorial.

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References

• Graves AL, Texler M, Manning L, Kulkarni H. Successful treatment of renal allograft and bladder malakoplakia with minimization of immunosuppression and prolonged antibiotic therapy. Nephrology (Carlton). 2014;19 Suppl 1:18-21. [PubMed link]
• Keitel E, Pêgas KL, do Nascimento Bittar AE, dos Santos AF, da Cas Porto F, Cambruzzi E. Diffuse parenchymal form of malakoplakia in renal transplant recipient: a case report. Clin Nephrol. 2014;81(6):435-9. [PubMed link]
• Leão CA, Duarte MI, Gamba C, Ramos JF, Rossi F, Galvão MM, David-Neto E, Nahas W, Shikanai-Yasuda MA, Pierrotti LC. Malakoplakia after renal transplantation in the current era of immunosuppressive therapy: case report and literature review. Transpl Infect Dis. 2012;14(6):E137-41. [PubMed link]
• Franco-Alzate C, Prada MC, Arias LF. Massive bilateral nephromegaly in an infant. Kidney Int. 2012;82(7):828. [PubMed link]

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