[Renal hemosiderosis following mechanical hemolysis: An original case report]. / Hémosidérose rénale consécutive à une hémolyse mécanique : un cas original.

Renal hemosiderosis is a rare cause of acute kidney injury, but it can also lead to chronic kidney failure. We report here the case of a 73-year-old patient with acute kidney caused by a massive hemosiderosis following the proximal disinsertion of a prosthesis of the ascendant aorta with chronic aortic dissection. The kidney biopsy revealed the diagnosis, showing massive iron deposits inside the proximal tubules, especially with Perls staining and also diffuse hematic casts in the lumen of the tubules. Pathophysiology of hemosiderosis is well described, as well as protective mechanisms. This case report is one of the numerous different causes of renal hemosiderosis that can be related to genetic, infectious or mechanical hemolysis.

[Management of idiopathic membranous nephropathy: evaluation of a standardized strategy in a Lorraine health network]. / Évaluation d'une stratégie standardisée de prise en charge de la glomérulonéphrite extramembraneuse idiopathique au sein d'un réseau de santé en Lorraine (Néphrolor).

INTRODUCTION: The evolution of idiopathic membranous membranous (MNi) is variable, treatment is discussed. Our work's main objective was to evaluate the results of a standardized management strategy proposed in 2006 in Lorraine (Nephrolor health network). The secondary objective was to evaluate the professional practices. PATIENTS ET METHODS: This study compared the evolution of incident MNi between 2002 and 2005 (Before) and between 2007 and 2010 (After), the respective dates point to 31/12/2011 and 31/12/2006. In nephrotics patients without renal failure, the risk assessment of end stage renal disease based on urinary ß2-microglobulin (uß2 m, threshold to 0,5 µg/min). Patients at high risk should receive immunosuppressive therapy (IS). RESULTS: Seventy-four biopsy-proven MNi were diagnosed with 50 nephrotic (22 Before and 28 After) of which 20 had normal renal function. In these nephrotic, there was no significant difference in the probability and the average time of remission between the two groups (P=0.26). Dosage of uß2m was performed in 35% of cases. Fifty percent were treated with IS respecting strategy. CONCLUSION: Despite differences between the strategy and practices, which may explain these non-significant results, we observed changes in thinking and therapeutic attitude of the clinician. The implementation of this strategy tends to standardize and improve practices about MNi in Lorraine, which seems to improve results in remission. We encourage to generalize the application of this strategy and to continue this cohort follow.

[Renal infiltrate by a plasmocytoïd chronic B lymphocytic leukaemia and renal failure: a rare occurrence in nephropathology. A case report and review of the literature]. / Infiltration du parenchyme rénal par une leucémie lymphoïde chronique B à différenciation plasmocytaire et insuffisance rénale: une éventualité rare en néphropathologie. Revue de la littérature à propos d'un cas.

We report the case of a 55-year-old male with renal failure as the initial manifestation of interstitial and focal infiltration of the kidneys by a small B-cell lymphoma. Since three years, this patient had a history of CLL with plasmocytic differentiation and was left untreated owing to stade A Binet classification. After chemotherapy, the lymphocytosis and the adenopathies disappear and the renal function improve. Infiltration of the kidneys by non-Hodgkin small B-cell lymphoma, including chronic lymphocytic leukaemia (CLL), is usually asymptomatic, fortuitously discovered at the time of an X-ray examination or at autopsy. Association with renal failure is extremely rare. We review the reported cases of renal failure associated with lymphomatous infiltration (13 cases of CLL and five cases of lymphoplasmocytic lymphoma kappa or lambda IgM), with the following conclusions: in most cases, renal insufficiency appears in a few months and significantly disappears after chemotherapy; the renal infiltrate is usually focal in lymphoplasmocytic lymphoma and rather massive and diffuse in CLL; the neoplastic feature of a small B-cell lymphoïd infiltrate may be difficult to determine: a poorly limited, monomorphous, CD20+ CD5+ lymphoid infiltrate is lymphomatous. In case of plasmocytic differentiation, it must be looked for kappa or lambda monotypy; the type of the lymphomatous infiltrate according to the WHO 2008 classification may be difficult to determine in a small sampling of renal tissue: the renal infiltrate must be compared, if possible, with a lymph node infiltrate. Owing to its bad prognosis, mantle cell lymphoma must be distinguished from other small B-cell lymphoma like CLL/small lymphocytic lymphoma, marginal zone lymphoma and lymphoplasmocytic lymphoma.

Immunoglobulin deposition disease with a membranous pattern and a circulating monoclonal immunoglobulin G with charge-dependent aggregation properties.

A 62-year-old woman presented with nephrotic syndrome, monoclonal gammopathy, and membranous-like nephropathy with nonorganized deposits composed of monoisotypic immunoglobulin G1 lambda protein. Nephrotic syndrome remitted after a brief course of treatment with melphalan despite ongoing production of the monoclonal protein. The circulating monoclonal immunoglobulin G1 lambda showed unusual in vitro aggregation properties, including dependence on low ionic strength and neutral pH, suggesting that electrostatic interactions had a role in the precipitation process. This case illustrates the importance of looking for monoclonal immunoglobulin deposits when kidney biopsy findings are suggestive of membranous nephropathy. In addition, our in vitro demonstrations of the role of physicochemical factors in immunoglobulin precipitation help elucidate the pathogenesis of immunoglobulin deposition disorders. Although binding to podocyte antigens is a well-recognized determinant of subepithelial immunoglobulin deposition, proneness to aggregation as described in this case also might be nephritogenic.