Background
Epstein–Barr virus‐associated smooth muscle tumors (EBV SMT) in adult kidney transplant recipients (KTR) are rare. The aims of this study are to document the clinical features, types of ...treatment given, and outcomes of KTR with EBV SMT in our institution.
Methods
Sixteen patients were identified from our institution's databases. Patients' survival, tumor outcome, and graft survival were compared between patients who remained on cyclosporine‐based immunosuppressant and those who converted to sirolimus‐based therapy.
Results
The median time of diagnosis was 9.4 yr after kidney transplantation, and majority of the patients had multifocal disease at the time of diagnosis. Overall, the patient survival rate was 75% over a mean follow‐up period of five yr. Two patients with non‐functioning allograft at the time of diagnosis of EBV SMT were excluded from the treatment outcome analysis. Comparing the sirolimus (n = 7) vs. cyclosporine groups (n = 7), patient survival rate was 100% vs. 42.9% (p = 0.08), graft survival 71.4% vs. 28.7% (p = 0.53), and disease‐free status 42.9% vs. 14.3% (p = 0.73), respectively.
Conclusion
Surgical resection in combination with decreasing immunosuppression or conversion to sirolimus appears to be effective in the treatment of EBV SMT in KTR.
Perivascular epithelioid cell tumours (PEComa) are rare tumours of mesenchymal origin. We report a 39-year-old patient who presented with painful defecation secondary to an anorectal mass. The ...diagnosis of anorectal PEComa was confirmed following excision and histopathological examination. We review the literature and discuss the management of this uncommon entity.
Light chain proximal tubulopathy (LCPT) is an uncommon renal disease characterized by the accumulation of monoclonal light chains within proximal tubular epithelial cells, with or without crystal ...formation. We report a rare case of lambda LCPT with crystals. Renal biopsy showed substantial acute tubular injury with unusual cytoplasmic changes affecting proximal tubules. In addition, abnormal tubular casts suggested concomitant light chain cast nephropathy. A clonal plasma cell infiltrate was present in the tubulointerstitial compartment. Immunofluorescence demonstrated strong staining for lambda light chain in tubular epithelial cells. Despite the absence of discernible crystals on light microscopy (LM), they were readily identified when ultrastructural evaluation was undertaken. Crystalline inclusions demonstrated positive immunogold labelling for lambda.
A lack of consensus exists as to the timing of kidney biopsy in children with steroid-dependent nephrotic syndrome (SDNS) where minimal change disease (MCD) predominates. This study aimed at ...examining the applicability of a biomarker-assisted risk score model to select SDNS patients at high risk of focal segmental glomerulosclerosis (FSGS) for biopsy.
Fifty-five patients with SDNS and biopsy-proven MCD (n = 40) or FSGS (n = 15) were studied. A risk score model was developed with variables consisting of age, sex, eGFR, suPAR levels and percentage of CD8
memory T cells. Following multivariate regression analysis, total risk score was calculated as sum of the products of odds ratios and corresponding variables. Predictive cut-off point was determined using receiver operator characteristics (ROC) curve analysis.
Plasma suPAR levels in FSGS patients were significantly higher, while percentage of CD45RO
CD8
CD3
was significantly lower than in MCD patients and controls. ROC analysis suggests the risk score model with threshold score of 16.7 (AUC 0.84, 95% CI 0.72-0.96) was a good predictor of FSGS on biopsy. The 100% PPV cut-off was >24.0, while the 100% NPV was <13.3.
A suPAR and CD8
memory T cell percentage-based risk score model was developed to stratify SDNS patients for biopsy and for predicting FSGS.
Rosai‐Dorfman disease (RDD) is a rare, idiopathic histiocytic proliferative disorder. We report two cases of RDD related to the breast which showed common distinctive imaging characteristics which ...can help facilitate accurate diagnosis and appropriate management.
The Banff Conference diagnostic categories and their criteria for renal biopsy interpretation were created in 1991 by a group comprising nephrologists, pathologists, transplant surgeons and ...immunologists. These guidelines are widely used in many countries. Every two years, participants at these meetings present and discuss research findings that have added to our knowledge of allograft pathology. From the start, features of T-cell mediated rejection were established. This was followed by discovery of C4d staining in biopsy tissue and better characterisation of antibody mediated rejection. The formation of working groups to look into problematic areas has allowed better refinements to be made to the classification scheme. The latest Banff 2013 report is significant for the inclusion of a C4d-negative category under humoral rejection. Together with the realisation that endarteritis may be antibody mediated, this latest report will greatly impact how pathologists interpret the allograft biopsy.
PDF
