Background: Light chain cast nephropathy (LCCN) is the most common kidney lesion in multiple myeloma patients. LCCN may exhibit a crystalline appearance. The frequency and clinical significance of crystalline LCCN are not well understood. Here, we report the first retrospective study of crystalline LCCN.
Methods: Twenty-six patients with LCCN were enrolled. We studied the clinicopathological features and outcomes of LCCN patients and compared ordinary LCCN patients (n = 18) with crystalline LCCN patients (n = 8).
Results: Crystalline LCCN was not rare (8/26, 30.8%) in our study. The median age of LCCN patients was 57.5 (range, 41-75) years. No patients presented with nephrotic syndrome. No significant differences in clinical features were observed between the two groups. All crystalline LCCN patients suffered from advanced multiple myeloma and acute kidney injury. There was a dominance of the λ isotype (7/8, 87.5%) in patients with crystalline LCCN. Patients with ordinary LCCN had significantly higher scores of tubular atrophy and acute tubular injury than those with crystalline LCCN. The crystalline casts of 5 crystalline LCCN patients stained negative with antihuman Tamm-Horsfall glycoprotein. There were no significant differences in the median overall survival between the crystalline LCCN group and the ordinary LCCN group (6.0 months vs. 35.0 months, p = 0.173). However, crystalline LCCN patients had higher early mortality than ordinary LCCN patients (50.0% vs 11.1%, p = 0.03).
Conclusion: Crystalline LCCN patients had higher early mortality than ordinary LCCN patients. Thus, for patients with LCCN, crystalline appearance should be screened carefully.
Download full-text PDF |
Source |
---|---|
http://dx.doi.org/10.1016/j.intimp.2021.107875 | DOI Listing |
J Chromatogr B Analyt Technol Biomed Life Sci
December 2024
Pulmonary Circulation Centre, Department of Cardiac and Vascular Diseases, Jagiellonian University Medical College, Krakow, Poland; Department of Cardiac and Vascular Diseases, St. John Paul II Hospital, Krakow, Poland.
A considerable percentage of ineffective treatment in pulmonary arterial hypertension (PAH) may be related to subtherapeutic dosage or non-adherence. The aim of the study was to develop a simple analytical method suitable for plasma determination of selected drugs: riociguat (RIO), bosentan (BOS) and macitentan (MAC) administered to PAH patients. An isocratic HPLC-UV system (Spectra Physics - Shimadzu) with a manual injector (50 μL loop) was applied.
View Article and Find Full Text PDFFASEB J
January 2025
Department of Hematology, Nephrology, and Rheumatology, Graduate School of Medicine, Akita University, Akita, Japan.
Various tubular diseases in patients with multiple myeloma (MM) are caused by monoclonal immunoglobulin light chains (LCs). However, the physicochemical characteristics of the disease-causing LCs contributing to the onset of MM-associated tubular diseases remain unclear. We herein report a rare case of MM-associated combined tubulopathies: non-crystalline light chain proximal tubulopathy (LCPT) and crystalline light chain cast nephropathy (LCCN).
View Article and Find Full Text PDFPresse Med
December 2024
Division of Pathology, Hôpital Maisonneuve-Rosemont, Université de Montréal, Montréal, Canada.
Front Nephrol
November 2024
Division of Nephrology and Hypertension, Department of Medicine, University of Minnesota, Minneapolis, MN, United States.
A toxic monoclonal protein typically results in a single kidney pathology due to the specific biophysical characteristics of monoclonal proteins. Multiple monoclonal protein lesions are rarely reported and often portend a poor prognosis. We present a 57-year-old male who developed rapidly progressive glomerulonephritis after concealed ruptured diverticulitis.
View Article and Find Full Text PDFBMC Nephrol
September 2024
Departments of Pathology, Xijing Hospital, School of Basic Medicine, Fourth Military Medical University, 169 Changle Western Street, Xi'an, 710032, Shaanxi, China.
Background: Multiple myeloma (MM) often causes renal tubular damage, such as the light chain cast nephropathy (LCCN) and the light chain proximal tubulopathy (LCPT). The excessive light chains deposited in the proximal and distal tubules usually manifest with different characteristics, leading to a rare coexistence of the two pathological conditions. Here we report a unique case of a patient with multiple myeloma (MM) who presented with acute kidney injury (AKI) due to dual conditions of λ light chain-restricted non-crystalline LCPT and LCCN.
View Article and Find Full Text PDFEnter search terms and have AI summaries delivered each week - change queries or unsubscribe any time!