The macrophages appear large, polygonal with foamy eosinophillic cytoplasm PS-341 nmr – the so-called von Hansemann cell. Attempts to correct the abnormal ratio of cGMP to cyclic adenosine monophosphate (cAMP) with the cholinergic agonist bethanechol chloride and ascorbic acid have had mixed results. Due to the protean nature of presentation and histopathological findings, it is likely the disease is under-recognized. A positive result from renal biopsy may yield the correct diagnosis in only 30% of cases. The disorder
most commonly associates with recurrent E. coli infection (80% of cases), with the exception of those cases related to human immunodeficiency virus (HIV), wherein infection with Rhodococcus equi is the rule. In some cases, inciting organisms have been cultured from biopsy tissue, just as we were able to demonstrate K. pneumoniae in the bladder biopsies in our patient, despite sterile urine. This suggests that the local environment may be permissive for bacterial survival and provide a viable reservoir for the ongoing aberrant inflammatory process. Malakoplakia can present with SCH772984 infection at multiple sites but expresses particular affinity for the genitourinary tract, especially
in females, with 58% cases involving this organ system. The kidney is the predominant site of involvement in 15% of cases, but has only been reported in renal allografts on fewer than 20 occasions. In the kidney, the enlarging parenchymal nodules can sometimes be mistaken for malignancy, with the diagnosis only made following transplant nephrectomy. The gastrointestinal tract is the second most common site with a spectrum of presentations possible, from an incidental 3-oxoacyl-(acyl-carrier-protein) reductase finding to haemorrhage or obstruction. Historically, malakoplakia was associated with poor outcomes, with a 6-month mortality rate above 50%. The development of quinolone antibiotics in the 1990s, agents with high bioavailability within macrophages, has improved the outlook. Sulphonamides are similarly active against malakoplakia. However, despite the success of these agents, malakoplakia has resulted in permanent
loss of renal function through graft failure, transplant nephrectomy and salt losing nephropathy over time.[2, 5] Patients with bilateral disease tend to fare especially poorly. These cases pose a difficult question as to whether treating nephrologists should pursue repeat transplantation, given the risk of recurrence on long-term immunosuppression is unknown. However, successful outcomes with preserved renal function have been documented. In our case, and in a few recent case reports, a strategy of minimization of immunosuppressive medications and prolonged antibiotic therapy has resulted in patient and allograft survival. In particular, the use of purine synthesis inhibitors such as azathioprine or mycophenolate mofetil might relate to poor outcomes through suppression of monocyte function.