Congophilic fibrils in the glomeruli with polyclonal immunoglobulin gamma staining-another cause for diagnostic overlap:A case report  

作　　者：Maria Bernadette Che-Ying Chow Lucas Bushrow Irmeen Siddiqui April Chiu Mirza Hamirani Anjali A Satoskar 

机构地区：[1]Department of Pathology,The Ohio State University Medical Center,Columbus,OH 43210,United States [2]Department of Pathology,North District Hospital,Sheung Shui,Hong Kong,China [3]Department of Laboratory Medicine and Pathology,Mayo Clinic,Rochester,MN 55905,United States [4]Department of Internal Medicine,S.Joseph's Hospital,Parkersburg,WV 26101,United States

出　　处：《World Journal of Clinical Cases》2024年第17期3200-3205,共6页世界临床病例杂志

摘　　要：BACKGROUND Glomerulopathy with fibrillary deposits is not uncommon in routine nephropathology practice,with amyloidosis and fibrillary glomerulonephritis being the two most frequently encountered entities.Renal amyloid heavy and light chain(AHL)is relatively uncommon and its biopsy diagnosis is usually limited to cases that show strong equivalent staining for a single immunoglobulin(Ig)heavy chain and a single light chain,further supported by mass spectrometry(MS)and serum studies for monoclonal protein.But polyclonal light chain staining can pose a challenge.CASE SUMMARY Herein we present a challenging case of renal AHL with polyclonal and polytypic Ig gamma(IgG)staining pattern by immunofluorescence.The patient is a 62-yearold Caucasian male who presented to an outside institution with a serum creatinine of up to 8.1 mg/dL and nephrotic range proteinuria.Despite the finding of a polyclonal and polytypic staining pattern on immunofluorescence,ultrastructural study of the renal biopsy demonstrated the presence of fibrils with a mean diameter of 10 nm.Congo red was positive while DNAJB9 was negative.MS suggested a diagnosis of amyloid AHL type with IgG and lambda,but kappa light chains were also present supporting the immunofluorescence staining results.Serum immunofixation studies demonstrated IgG lambda monoclonal spike.The patient was started on chemotherapy.The chronic renal injury however was quite advanced and he ended up needing dialysis shortly after.CONCLUSION Tissue diagnosis of AHL amyloid can be tricky.Thorough confirmation using other available diagnostic techniques is recommended in such cases.

关 键 词：Heavy and light chain amyloid Fibrillary glomerulonephritis DNAJB9 Serum immunofixation Protein electrophoresis Mass spectrometry Congo red Case report 

分 类 号：R768.32[医药卫生—耳鼻咽喉科]