Open AccessCombined light chain crystalline tubulopathy, podocytopathy, and histiocytosis associated with Bence–Jones κ protein diagnosed via immuno-electron microscopy
Author(s) -
Yoshihiro Nakamura,
Hiroshi Kitamura,
Hiroki Ikai,
Mari Yamamoto,
Yukari Murai,
Tsuyoshi Watanabe,
Naoho Takizawa,
Waka Yokoyama-Kokuryo,
Takashi Ehara,
Masaya Watarai,
Hideaki Shimizu,
Yoshiro Fujita
Publication year - 2021
Publication title -
cen case reports
Language(s) - English
Resource type - Journals
ISSN - 2192-4449
DOI - 10.1007/s13730-021-00588-9
Subject(s) - bence jones protein , medicine , immunoglobulin light chain , pathology , renal biopsy , renal function , biopsy , tubulopathy , immunoelectron microscopy , kidney , antibody , immunohistochemistry , immunology
We herein report a case of a combined crystalline light chain tubulopathy, podocytopathy, histiocytosis, and cast nephropathy in a patient with monoclonal gammopathy of renal significance (MGRS). A 66-year-old female with impaired renal function was referred to our department. Despite intravenous fluid resuscitation, the kidney function worsened progressively; thus, a kidney biopsy was performed. The kidney biopsy revealed light chain proximal tubulopathy (LCPT) with crystals, light chain crystal podocytopathy (LCCP), crystal-storing histiocytosis (CSH), and light chain cast nephropathy (LCCN). Of note, LCCP and CSH were diagnosed via electron microscopy. Serum and urine immunoelectrophoresis (IEP) revealed the presence of monoclonal Bence-Jones protein and free κ light chains. Bone marrow aspiration showed < 10% plasma cell proliferation. Thus, we had encountered a rare case in which a variety of kidney lesions were combined with MGRS. Most of the LCPT, LCCP, and CSH cases show monoclonal IgG κ, while our case showed Bence-Jones protein κ.