Renal cell carcinoma presenting as AA amyloidosis: a case report and review of the literature.

A 47-year-old Caucasian man developed mild diarrhoea associated with more than 10 kg weight loss, severe fatigue and anaemia. Endoscopy demonstrated deposits of AA amyloid within the gastrointestinal tract. He had heavy proteinuria with a serum albumin of 15 g/L consistent with systemic AA amyloidosis. He had no symptoms to suggest an underlying chronic inflammatory condition but had CRP 130 mg/L and SAA 474 mg/L. In an attempt to identify the source of his inflammatory response, he underwent a contrast-enhanced whole-body computed tomography scan, which revealed a necrotising mass lesion in the right kidney consistent with a renal cell carcinoma. It also showed non-mechanical obstruction of the small bowel and, immediately post-imaging, the patient developed intractable vomiting followed by oliguric renal failure requiring haemodialysis. Despite his renal and gut failure, he underwent right radical nephrectomy without further complications. Histology showed complete resection of a clear cell renal cell carcinoma and renal amyloid deposits. Post-surgery, his acute-phase response decreased to normal, consistent with the renal cell carcinoma acting as the inflammatory stimulus. Although he remains dialysis dependent, his gut function improved and he has regained both normal weight and serum albumin. Our case demonstrates partial resolution of AA amyloidosis with removal of the inflammatory source.

Hypogammaglobulinemia and bronchiectasis in mycophenolate mofetil-treated renal transplant recipients: an emerging clinical phenomenon?

BACKGROUND: Mycophenolate mofetil (MMF) inhibits T- and B-cell proliferation and can cause acquired or secondary hypogammaglobulinemia. This finding and the subsequent development of opportunistic infection, including pneumonia, have been reported in patients receiving MMF. Chronic pulmonary infection and hypogammaglobulinemia predispose to bronchiectasis, and we aimed to establish the incidence and clinical pattern of this condition within our MMF-treated renal transplant population. METHODS: We performed a retrospective analysis of MMF-treated transplant recipients. Two hundred and eighty-nine patients were identified and for each, demographic, clinical, radiological and laboratory data from case notes and electronic records were collected. RESULTS: Twenty-three of 289 patients had recurrent severe chest infections (>2 episodes) between 12 and 95 months after the introduction of MMF. The mean age was 53 ± 17yr. Pulmonary lesions fulfilled clinical, radiographic and computerized tomography criteria for bronchiectasis in 7/289 (2.4%). All seven patients with bronchiectasis had low serum IgG levels. Three patients had sufficient samples available for B-cell phenotype analysis but no conclusive results emerged. No cases of post-transplant bronchiectasis were identified in our transplant population not receiving MMF. DISCUSSION: We report seven cases of bronchiectasis in renal transplant patients receiving MMF. We speculate that low immunoglobulin levels may contribute to the development of this significant pulmonary disease.

UK Renal Registry 11th Annual Report (December 2008): Chapter 14 UK Renal Registry and international comparisons.

BACKGROUND: The aim of this study is to report Renal Replacement Therapy (RRT) incidence and prevalence rates, the percentage of incident patients with diabetes mellitus as cause of renal disease, the RRT modality mix and the transplant rate in different countries. The number of national or regional registries collecting and reporting data pertaining to traditional cardiovascular (CV) risk factors in prevalent dialysis patients is also examined. METHODS: Data on numbers of incident and prevalent RRT patients in England, Wales, Scotland and Northern Ireland for the year 2007 were collected from the UK Renal Registry (UKRR) database and collated to meet the specifications on the US Renal Data System (USRDS) international data collection form. RESULTS: In 2007, the incidence and prevalence of RRT in the UK were 110 and 759 per million of the population (pmp) respectively. Incidence of RRT placed the UK 34th out of the 43 countries reporting to the USRDS in 2006. In the majority of reporting countries, 20-44% have diabetes as the primary cause of end stage renal disease. Only the Finnish, Malaysian and US Renal Registries were found to routinely report attainment of cardiovascular risk standards. CONCLUSIONS: A comparison among international renal registries about RRT epidemiology and reporting cardiovascular risk factors in prevalent RRT patients forms an important part of the quality improvement process and often allows for improving standards and performances between reporting countries. Despite the high CV morbidity associated with RRT, few renal registries routinely report data on CV risk management; where data are reported there is little agreement in what represents quality of care, making direct comparison difficult.