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Light Chain Proximal Tubulopathy: Clinical and Pathologic Characteristics in the Modern Treatment Era.

ABSTRACT: Light chain proximal tubulopathy (LCPT) is characterized by cytoplasmic inclusions of monoclonal LC within proximal tubular cells. The significance of crystalline versus noncrystalline LCPT and the effect of modern therapies are unknown. We reported the clinical-pathologic features of 40 crystalline and six noncrystalline LCPT patients diagnosed between 2000 and 2014. All crystalline LCPTs were ?-restricted and displayed acute tubular injury. One-third of noncrystalline LCPT patients displayed ?-restriction or acute tubular injury. Only crystalline LCPT frequently required antigen retrieval to demonstrate monoclonal LC by immunofluorescence. In five of 38 patients, crystals were not detectable by light microscopy, but they were visible by electron microscopy. Hematolymphoid neoplasms, known before biopsy in only 15% of patients, included 21 monoclonal gammopathies of renal significance; 15 multiple myelomas; seven smoldering multiple myelomas; and three other neoplasms. Biopsy indications included Fanconi syndrome (38%; all with crystalline LCPT), renal insufficiency (83%), and proteinuria (98%). Follow-up was available for 30 (75%) patients with crystalline LCPT and all six patients with noncrystalline LCPT, of whom 11 underwent stem cell transplant, 16 received chemotherapy only, and nine were untreated. Complete or very good partial hematologic remissions occurred in six of 22 treated crystalline LCPT patients. By multivariable analysis, the only independent predictor of final eGFR was initial eGFR, highlighting the importance of early detection. All patients with crystalline LCPT treated with stem cell transplant had stable or improved kidney function, indicating the effectiveness of aggressive therapy in selected patients.

SUBMITTER: Stokes MB

PROVIDER: S-EPMC4849818 | biostudies-other | 2016 May

REPOSITORIES: biostudies-other

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Light Chain Proximal Tubulopathy: Clinical and Pathologic Characteristics in the Modern Treatment Era.

Stokes Michael B MB Valeri Anthony M AM Herlitz Leal L Khan Abdullah M AM Siegel David S DS Markowitz Glen S GS D'Agati Vivette D VD

Journal of the American Society of Nephrology : JASN 20150915 5

Light chain proximal tubulopathy (LCPT) is characterized by cytoplasmic inclusions of monoclonal LC within proximal tubular cells. The significance of crystalline versus noncrystalline LCPT and the effect of modern therapies are unknown. We reported the clinical-pathologic features of 40 crystalline and six noncrystalline LCPT patients diagnosed between 2000 and 2014. All crystalline LCPTs were κ-restricted and displayed acute tubular injury. One-third of noncrystalline LCPT patients displayed λ ...[more]

PMID: 26374607

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Project description:BackgroundSeveral strategies for the treatment of pathologic proximal femur fractures are practiced but treatment outcomes have not been rigorously compared.Questions/purposesMajor variations in the use of intramedullary fixation, extramedullary/plate-screw fixation, and endoprosthetic reconstruction techniques for pathologic proximal femur fractures in patients with skeletal metastases are reported. The clinical and surgical variables that influence this choice differ among treating surgeons. To characterize the technique preferences and to identify areas of consensus regarding specific clinical presentations, we administered an online survey to the Musculoskeletal Tumor Society (MSTS) membership. We also tested whether responses correlated with the respondents' years in practice and asked about the indications for wide tumor resection and the role of tumor debulking and adjuvant cementation.MethodsA 10-minute, web-based survey was sent via email to 244 MSTS members. The survey queried participants' musculoskeletal oncology training and experience and presented case scenarios illustrating different combinations of four variables that influence decision-making: cancer type, estimated patient survival, fracture displacement, and anatomic region of involvement.ResultsForty-one percent (n = 98) of MSTS members completed the survey. Intramedullary nail fixation (IMN; 45%) and proximal femur resection and reconstruction (34%) were the most commonly recommended techniques followed by long-stem cemented hemiarthroplasty/cemented hemiarthroplasty (15%) and open reduction and internal fixation (7%). Most respondents (56%) recommended use of cementation with IMN. Differences of opinion on recommended treatment were associated with variations in cancer type, fracture displacement, and anatomic region of involvement.ConclusionsOur online survey showed a trend among MSTS members for selecting IMN and arthroplasty-related techniques to treat pathologic fractures of the proximal femur, but major differences in preferred operative technique exist. Prospective studies are needed to develop consistent, evidence-based treatment recommendations.

Dataset Information

Light Chain Proximal Tubulopathy: Clinical and Pathologic Characteristics in the Modern Treatment Era.

Publications

Light Chain Proximal Tubulopathy: Clinical and Pathologic Characteristics in the Modern Treatment Era.

Similar Datasets

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