Membranous nephropathy: recurrence after kidney transplantation

BACKGROUND.: It is supposed that about 5% of dialysis patients had membranousnephropathy as a cause for their renal failure. Despite of thisprevalence, only 33 cases of recurrent membranous nephropathyafter kidney transplantation have been reported in the Englishliterature. METHODS.: Among 509 recipients of renal allografts, membranous glomerulonephritiswas the cause of renal failure in five patients, who receivedsix transplants. RESULTS.: Recurrence of the disease was observed in three allografts (50%)in three patients, all of them were on treatment with cyclosporinand low-dose prednisone. Proteinuria appeared at 2, 5 and 19months after grafting. One patient experienced a spontaneousremission after 12 months and he is free from proteinuria andwith good renal function after 5 years. The remaining two patientspresented progressive renal function deterioration and returnedto haemodialysis 24 and 17 months after the appearance of proteinuria.In these patients increasing the immunosuppression did not produceany beneficial effect. One of those patients underwent a secondtransplant; recurrence of the membranous nephropathy has notbeen observed after 3 years of follow-up. CONCLUSIONS.: In this study three new cases of recurrence of membranous nephropathyare reported. One patient experienced a spontaneous remissionof proteinuria. Recurrence of membranous nephropathy in renalallograft was very high in our series. Its appearance was associatedwith poor prognosis of the graft in most patients, althoughspontaneous remission of proteinuria is possible.     

An investigation of internal phase losses during the microencapsulation of fragrances.

Prototype fragrances, prepared from common fragrance components, were extracted with water, recovered, and characterized by gas chromatography before and after the water treatment, revealing a significant loss of the more water-soluble components. Unextracted prototype fragrances were also microencapsulated by a gelatin/gum arabic coacervation process. The microencapsulated fragrance oils were recovered from the microcapsules, using pepsin enzyme to open up the capsules. Comparison of GC results of microencapsulated fragrance oil versus unencapsulated oil showed many of the changes could be ascribed to solubility losses of the more water-soluble components to the process water. Deliberate inclusion of toluene as a fragrance component in one of the prototype fragrances showed that some losses of highly volatile fragrance components can be expected during microencapsulation; but because most fragrance components do not approach the volatility of toluene, such losses are expected to be minimal. Chromatograms taken before and after microencapsulation of two commercial fragrances are discussed.     

Cholesterol levels in HIV-HCV infected patients treated with lopinavir/r: results from the SCOLTA project.

BACKGROUND: It is not known whether antiretroviral therapy (ART) including lopinavir/r has a different effect on the lipid metabolism in HIV patients co-infected with HCV. This study investigated changes in lipid levels, comparing patients with HIV infection alone and those with HCV too, in the lopinavir/r cohort of the SCOLTA project. METHODS: We analyzed the data for the lopinavir/r nationwide cohort from 25 Italian infectious disease departments, which comprises 743 HIV-infected patients followed prospectively, comparing subjects with HIV-HCV co-infection and those with single-infection. RESULTS: At enrolment, co-infected patients had significantly lower mean cholesterol than HCV negative cases (162+/-43mg/dL vs. 185+/-52mg/dL, p=0.0009). Total and non-HDL cholesterol and triglycerides rose significantly from baseline in HIV single-infection patients, but not in those with co-infection. The patients with dual HIV-HCV infection, treated with an ART regimen including lopinavir/r, have only limited increases in total and non-HDL cholesterol and triglycerides. CONCLUSIONS: Changes in serum lipids in co-infected patients differed significantly from those in patients without HCV. It remains to be seen whether this is associated with a lower risk of progression of atherosclerotic disease.     

Systemic Lupus Erythematosus Appearing as an Urticarial Vasculitis

A patient with systemic lupus erythematosus presented with clinical and histologic feature of urticarial vasculitis, a systemic immune disorder of unknown origin. This could represent a subset of collagen vascular disease.     

Kidney transplantation in systemic lupus erythematosus nephritis: a one-center experience

Eight patients with end-stage renal disease secondary to systemic lupus erythematosus (SLE) received 8 cadaveric renal allograft. Patient and graft survival was 100 and 87%, respectively. None of them showed extrarenal manifestations of SLE or recurrence of lupus nephritis after grafting. One graft was lost because of chronic rejection. In another patient, an episode of graft function deterioration due to bad control of arterial hypertension was observed. Three patients were transplanted during their first year on hemodialysis. Two women became pregnant after successful kidney transplantation; one suffered a spontaneous abortion and the other had a successful delivery. In neither of them, was SLE observed during or after pregnancy. Morbidity was low in this series, and infections were the most frequent complication. In summary, our experience with renal transplantation in SLE patients compares, favorably with the general nodiabetic transplanted population.     

Perirenal subcapsular fluid collection in a patient with membranous nephropathy and renal vein thrombosis

We report a case of perirenal fluid collection associated with membranous nephropathy and right renal vein thrombosis. Surgical removal of the fluid and instillation of povidone iodine resolved the problem.     

Low incidence of hepatotoxicity in a cohort of HIV patients treated with lopinavir/ritonavir

We describe the hepatotoxicity encountered in a cohort of HIV-positive patients treated with lopinavir/ritonavir. We used the database from the SCOLTA project, an on-line pharmacovigilance programme involving 25 Italian infectious disease centres. A total of 755 patients were followed, over a mean observation period of 16 months. The incidence of severe events was low despite the high prevalence of patients co-infected with hepatitis virus at enrollment.     

Anagrelide and Mutational Status in Essential Thrombocythemia

Background

Anagrelide is an orally active, quinazolone-derived, platelet-lowering agent that acts by blocking megakaryocyte maturation and polyploidization as well as proplatelet formation, and is currently indicated for second-line treatment of high-risk patients with essential thrombocythemia (ET) in Europe. In recent years various clinical trials have confirmed the safety and efficacy of this drug in ET, with some also considering Janus kinase 2 (JAK2) mutational status, but have not confirmed the impact that the other driver mutations, i.e., calreticulin (CALR) and myeloproliferative leukemia virus (MPL), may have on the response to this therapy.

Objective

To assess the impact of JAK2, MPL, CALR gene mutational status on response to anagrelide therapy in patients with ET treated at the Oncohematology Division, IRCCS Ca’ Granda–Maggiore Policlinico Hospital Foundation, Milan between 2004 and 2015.

Methods

Among 213 ET patients who were diagnosed between January 1983 to November 2014, 21 consecutive cases who were started on anagrelide as a second-line therapy and received at least 1-year of treatment were included. Inclusion criteria were the availability of demographic, clinical, histological, and hematologic data at diagnosis, and at least one granulocyte DNA sample to assess the mutational status of the JAK2, MPL, and CALR genes.

Results

The JAK2V617F mutation was detected in seven patients (33.3 %), CALR mutations were identified in another seven cases, and the remaining seven patients were defined as “triple-negative” (i.e., no JAK2, CALR, or MPL mutation). After a median anagrelide treatment duration of 4.6 years, 16 of 21 patients (76.2 %) achieved at least a partial platelet response: in particular, the hematological response rate was substantially comparable between JAK2-positive and “triple-negative” patients, whereas the five patients who did not achieve any platelet response all had CALR mutations.

Conclusion

Although it needs to be confirmed with a larger number of ET patients treated with anagrelide, we suggest that mutational status should be considered carefully when deciding on the most appropriate therapy for each patient, mainly because anagrelide alone was not able to achieve an appropriate hematological response in CALR-mutated ET cases.

     

Biological and molecular characterization of a rare case of cutaneous Richter syndrome

Richter syndrome (RS) is the transformation of chronic lymphocytic leukemia in a high‐grade lymphoma usually presenting nodal and bone marrow involvement. Richter syndrome can be localized at extranodal sites including the gastrointestinal tract, lungs, and skin. Cutaneous RS is an extremely rare disease apparently showing a less aggressive course than common presentations. While nodal RS has been extensively investigated in literature, pathogenesis and prognosis of cutaneous RS are still partially unknown, even if a role of Epstein‐Barr virus infection and p53 disruption has been suggested. Herein, we characterized the histopathological, immunohistochemical features and cytogenetics and molecular alterations of a case of cutaneous RS developed after 8 years chronic lymphocytic leukemia history. Moreover, we reviewed the literature reports concerning cutaneous RS and made a focus on biological patterns and prognostic implications.