Hemophagocytic syndrome in kidney transplant recipients: report of four cases from a single center.

BACKGROUND: The prognosis of hemophagocytic syndrome (HPS) in kidney transplant recipients is reported to be poor, however the optimal therapeutic approach is still unclear. PATIENTS AND METHODS: The clinical and follow-up data of the 4 patients with HPS (3 male, 1 female; age 39.7 +/- 11.3 years) among 368 kidney transplant recipients during a 5-year period were retrospectively analyzed. RESULTS: HPS developed 35-61 days in the post-transplant period. All 4 patients presented with fever. Hepatosplenomegaly and lymphadenopathy were observed only in the first patient. Laboratory tests revealed pancytopenia and hyperferritinemia in all patients, but elevated liver enzymes were observed in 3. Two patients had cytomegalovirus infection, and 1 had Epstein-Barr virus infection. Three patients died despite aggressive supportive therapy, however the fourth case survived after graft nephrectomy. CONCLUSION: HPS pathogenesis in kidney transplants appears to be related with the graft itself. Graft nephrectomy may be the preferable therapeutic approach for kidney transplant recipients with HPS resistant to standard supportive therapy.

Calcium oxalate deposition in renal cell carcinoma associated with acquired cystic kidney disease: a comprehensive study.

The main complication of acquired cystic kidney disease (ACKD) is frequent development of renal tumors, including renal cell carcinoma (RCC). Intratumoral deposition of calcium oxalate (CaOx) is a distinct feature of ACKD-associated RCCs, but several features of this type of RCC are not known. Features of the 30 end-stage renal disease (ESRD)-associated RCCs identified within a 13-year period, including eight with CaOx deposition, were analyzed. Pathologic and clinical features of CaOx positive (+) and negative (-) RCCs were evaluated and compared. The CaOx+ RCCs showed higher tendency for bilaterality and multifocality. Seven tumors displayed distinctive morphologic features characterized by tumor cells with ill-defined cell membrane, abundant granular eosinophilic cytoplasm, large nuclei, and prominent nucleoli. One tumor was of clear cell type. Regardless of histologic type, all tumors displayed a proximal tubular differentiation. No significant difference was noted for tumors' stage, proliferation, and apoptosis rate between the CaOx+ and CaOx- RCCs. CaOx+ RCCs account for a significant portion of all ESRD-associated RCCs. The majority of these RCCs display a distinctive morphologic profile. Proximal tubular cell differentiation in conjunction with ESRD-mediated high serum level may be pathogenetically important for intratumoral CaOx deposition. These RCCs seems to have a relatively good prognosis.

Effects of hemodialysis on myocardial performance index.

The myocardial performance index (MPI) reflects global ventricular function. Chronic hypervolemia and uremia may negatively affect the myocardium of both ventricles. The aims of this study were to investigate how chronic renal failure (CRF) affects biventricular MPI and to determine whether preload reduction by hemodialysis (HD) affects left ventricular MPI (LVMPI) and right ventricular MPI (RVMPI) in CRF. Twenty-one patients with CRF (group 1) were examined 1 hour before and 1 hour after an HD session and 17 healthy control patients (group 2) were examined once by echocardiography. The MPI for each ventricle was calculated as the sum of isovolumic time intervals divided by the ejection time. Before HD, the LVMPI of group 1 was similar to that in group 2 (P>.05), but the RVMPI of group 1 was significantly higher (P=.007). After the HD session, LVMPI and RVMPI remained unchanged (P>.05 for both). The LVMPI and RVMPI were not correlated either before or after HD in group 1 (P>.05 for both), whereas they were correlated in group 2 (r=0.671, P=.003). Chronic renal failure causes isolated RV dysfunction, as reflected by increased RVMPI values. Preload reduction by HD does not affect LVMPI or RVMPI. Patients with CRF also do not exhibit the correlation of LVMPI and RVMPI that is observed in healthy individuals.

Calcium oxalate deposition in renal allografts: morphologic spectrum and clinical implications.

Many aspects of calcium oxalate (CaOx) deposition in renal transplant biopsies are not known. Review of all renal transplant biopsies performed in a 7-year period showed that CaOx deposition could be classified into three groups. Group I: Seven biopsies within a month post-transplant displayed rare CaOx foci against a background of acute tubular necrosis or acute cell-mediated rejection. At follow-up, five grafts functioned well and two failed due to chronic allograft nephropathy. CaOx in this context was an incidental finding secondary to a sudden excretion of an end-stage renal disease-induced increased body burden of CaOx. Group II: Two biopsies performed 2 and 10 months post-transplant showed rare CaOx foci against a background of chronic allograft nephropathy, leading to graft loss. CaOx in this context reflected nonspecific parenchymal deposition due to chronic renal failure regardless of causes. Group III: One biopsy with recurrent PH1 characterized by marked CaOx deposition associated with severe tubulointerstitial injury and graft loss 6 months post-transplant. There were two previously reported cases in which CaOx deposition in the renal allografts was due the antihypertensive drug naftidrofuryl oxalate or increased intestinal absorption of CaOx. CaOx deposition in renal allografts can be classified in different categories with distinctive morphologic features and clinical implications.

Post-transplant nephrotic syndrome: A comprehensive clinicopathologic study.

BACKGROUND: Post-transplant (Tx) nephrotic syndrome (NS) is not well defined. METHODS: Seventy-four renal transplant recipients with NS were studied. RESULTS: Biopsies showed chronic allograft nephropathy (CAN) in 31 patients; recurrent glomerular disease (GN) in 15, de novo GN in 18, and undetermined GN in 9. NS developed 0.25 to 384 months post-Tx and was treated with angiotensin-converting enzyme inhibitors (ACEI) or angiotensin receptor blockers (ARB) in 18 patients; calcium channel blockers in 25; or both drugs in 31. NS remitted in 24% of cases 2 to 28 months after onset, and this persisted in all except 3 patients. The remission rate was lowest (9%) for CAN and highest (47%) for de novo GN. Compared with persistent NS, those with remission showed higher prevalence of de novo GN (53% vs. 17%), lower prevalence of CAN (18% vs. 50%), earlier onset of NS (39 vs. 59 months), lower serum SCr at onset (2.3 vs. 2.9 mg/dL), and higher incidence of treatment with ACE or ARB. The 5-year graft loss rates for CAN, recurrent and de novo GN were 57%, 36%, and 23%, respectively. Compared with the functioning grafts, the failed grafts showed higher prevalence of CAN (60% vs. 16%), lower prevalence of de novo GN (12% vs. 46%), earlier onset of NS (47 vs 65 months post-Tx), higher serum SCr at onset (3.3 vs. 2.0 mg/dL), lower prevalence of remission of NS (5% vs. 48%), and higher proteinuria at follow-up (5.1 vs. 2.5 g/day). Graft survival improved with NS remission (88% vs. 18%). CONCLUSION: Post-Tx NS displays distinctive clinicopathologic features with pathogenetic and therapeutic implications.