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Journal of Clinical Pathology.... 1975
Review
Topics: Heavy Chain Disease; Humans; Immunoglobulin alpha-Chains
PubMed: 830057
DOI: 10.1136/jcp.s1-6.1.72 -
Clinical and Experimental Nephrology Dec 2013Heavy chain deposition disease (HCDD) is one of three entities of monoclonal immunoglobulin deposition disease, characterized histopathologically by the presence of... (Review)
Review
Heavy chain deposition disease (HCDD) is one of three entities of monoclonal immunoglobulin deposition disease, characterized histopathologically by the presence of nodular glomerulosclerosis and glomerular and tubular deposition of monoclonal heavy chains without associated light chains. Although HCDD is an extremely rare disease, >30 cases have been reported to date in the literature. Of these cases, only three cases have been reported in Japan. The majority of the patients presents with nephrotic syndrome, hematuria, and hypertension, and develop progressive renal failure with or without the complication of multiple myeloma. Some cases have been treated successfully using chemotherapy. Because of its rarity, a thorough understanding of HCDD is essential for both accurate diagnosis and adequate subsequent treatment.
Topics: Female; Heavy Chain Disease; Humans; Immunoglobulin Heavy Chains; Kidney; Male; Middle Aged; Nephrotic Syndrome
PubMed: 23652830
DOI: 10.1007/s10157-013-0812-x -
Blood Mar 2019
Topics: Aged; Autoimmune Diseases; B-Lymphocytes; Fatal Outcome; Female; Heavy Chain Disease; Humans; Immunoglobulin gamma-Chains
PubMed: 30898778
DOI: 10.1182/blood-2018-11-887133 -
Haematologica Jul 2021
Topics: Aged; Female; Heavy Chain Disease; Humans; Male; Proteinuria; Waldenstrom Macroglobulinemia
PubMed: 33596645
DOI: 10.3324/haematol.2020.277137 -
British Medical Journal Nov 1968
Topics: Heavy Chain Disease; Humans; Immunoglobulin G; Male; Middle Aged
PubMed: 5687601
DOI: No ID Found -
Blood Apr 1974
Topics: Blood Protein Electrophoresis; Blood Viscosity; Chlorambucil; Chromatography, Gel; Chromium Radioisotopes; Cote d'Ivoire; Electrophoresis, Polyacrylamide Gel; Fluorescent Antibody Technique; Heavy Chain Disease; Humans; Immunodiffusion; Immunoelectrophoresis; Immunoglobulin Fab Fragments; Immunoglobulin Fc Fragments; Immunoglobulin Fragments; Male; Middle Aged; Papain; Plasmapheresis; Sodium Dodecyl Sulfate; Spleen; Splenomegaly; Trypsin
PubMed: 4361394
DOI: No ID Found -
Lancet (London, England) Jul 1979
Topics: Antibodies, Anti-Idiotypic; Heavy Chain Disease; Humans; Immunoelectrophoresis; Immunoglobulin Fab Fragments; Immunoglobulin Heavy Chains; Immunoglobulin alpha-Chains; Lymphoma
PubMed: 87892
DOI: No ID Found -
Molecular Immunology Jul 1986A patient (BW) was studied with Mu heavy chain disease (mu HCD) in whom a leukemic B-cell clone secreted a shortened monoclonal mu chain without associated light chain....
A patient (BW) was studied with Mu heavy chain disease (mu HCD) in whom a leukemic B-cell clone secreted a shortened monoclonal mu chain without associated light chain. The cells did, however, produce a normal-sized kappa light chain that was detected as urinary Bence-Jones protein. The cytoplasmic and secreted monomeric mu chain had an approximate mol. wt of 58,000. Radiochemical sequence analysis of the biosynthetically labelled mu chain revealed a protein that lacked the entire variable region. The sequence initiated at amino acid position 5 within the first constant region domain (CH1) of C mu. The primary in vitro translation product, the cytoplasmic and secreted proteins were all similarly truncated, thereby excluding extensive postsynthetic degradation. The mu RNA, that directed the synthesis of the truncated mu protein, was about 350 bp smaller than the normal mu RNA. Furthermore, by primer extension analysis it was possible to localize this deletion in the mu RNA to a region 5' of CH1. Thus, a defect at the level of Ig gene structure/assembly that deletes coding information or results in aberrant RNA processing must be responsible for the truncated mu HCD protein BW.
Topics: Amino Acid Sequence; Bence Jones Protein; Electrophoresis, Polyacrylamide Gel; Female; Heavy Chain Disease; Humans; Immunoelectrophoresis; Immunoglobulin mu-Chains; Protein Biosynthesis; RNA, Messenger
PubMed: 3099178
DOI: 10.1016/0161-5890(86)90083-0 -
Clinical Chemistry and Laboratory... Nov 2017
Topics: Aged; Heavy Chain Disease; Humans; Male; Middle Aged
PubMed: 28665788
DOI: 10.1515/cclm-2017-0254 -
Maryland State Medical Journal Dec 1964
Topics: Blood Protein Disorders; Heavy Chain Disease; Humans; Immunoglobulin G; gamma-Globulins
PubMed: 14225385
DOI: No ID Found