Monoclonal immunoglobulin crystalline nephropathies. 2024

Samih H Nasr, and Christophe Sirac, and Nelson Leung, and Frank Bridoux
Department of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, Minnesota, USA. Electronic address: Nasr.samih@mayo.edu.

Monoclonal Ig crystalline nephropathies are rare lesions resulting from precipitation of monoclonal Igs in the kidney as crystalline inclusions. They can be categorized into lesions with predominant intracellular crystals (light chain [LC] proximal tubulopathy, LC crystal-storing histiocytosis, and LC crystalline podocytopathy) and lesions with predominant extracellular crystals (crystalglobulin-induced nephropathy and crystalline variant of LC cast nephropathy). The majority of these lesions are associated with low tumor burden lymphoproliferative disorders, with the exception of crystalline variant of LC cast nephropathy. Extrarenal involvement (e.g., skin and cornea) is frequent. Kidney biopsy is the cornerstone for diagnosis, which often requires electron microscopy and antigen retrieval. A thorough hematologic workup and evaluation of extrarenal involvement is mandatory for management. Treatment of these lesions is with clone-directed therapy, with the goal of achieving hematologic very good partial response or complete response, which preserves or improves kidney function. In vitro and in vivo studies, animal models, and novel sequencing techniques have been invaluable tools to understand the pathogenesis of LC proximal tubulopathy and can be used to increase our limited knowledge of the pathogenesis of the other monoclonal Ig crystalline nephropathies. This review provides an update on the pathology, renal and hematologic characteristics, extrarenal manifestations, prognosis, treatment, and pathogenesis of monoclonal Ig crystalline nephropathies.

UI MeSH Term Description Entries

Related Publications

Samih H Nasr, and Christophe Sirac, and Nelson Leung, and Frank Bridoux
July 2012, Archives of pathology & laboratory medicine,
Samih H Nasr, and Christophe Sirac, and Nelson Leung, and Frank Bridoux
December 2021, Kidney international reports,
Samih H Nasr, and Christophe Sirac, and Nelson Leung, and Frank Bridoux
May 1998, Australian and New Zealand journal of ophthalmology,
Samih H Nasr, and Christophe Sirac, and Nelson Leung, and Frank Bridoux
January 1987, Pathology annual,
Samih H Nasr, and Christophe Sirac, and Nelson Leung, and Frank Bridoux
October 2023, Clinical journal of the American Society of Nephrology : CJASN,
Samih H Nasr, and Christophe Sirac, and Nelson Leung, and Frank Bridoux
May 2020, Seminars in diagnostic pathology,
Samih H Nasr, and Christophe Sirac, and Nelson Leung, and Frank Bridoux
January 1984, Presse medicale (Paris, France : 1983),
Samih H Nasr, and Christophe Sirac, and Nelson Leung, and Frank Bridoux
January 1982, Biochemistry,
Samih H Nasr, and Christophe Sirac, and Nelson Leung, and Frank Bridoux
June 1986, The Journal of clinical investigation,
Samih H Nasr, and Christophe Sirac, and Nelson Leung, and Frank Bridoux
May 1978, Nihon rinsho. Japanese journal of clinical medicine,
Copied contents to your clipboard!