[A case report of light and heavy chain deposition disease (IgG2 lambda)]. 1994

Y Akashi, and N Yoshizawa, and S Oshima, and A Takeuchi, and T Kubota, and S Kondo, and Y Oshikawa, and T Oda, and Y Suzuki, and J Shimizu
Second Department of Internal Medicine, National defense Medical College, Saitama, Japan.

A 73-year-old male was admitted to the renal division of our hospital because of hypertension, proteinuria and bilateral pretibial edema. Eight years previously, he was diagnosed as being afflicted with interstitial pneumonia on the basis of a chest X-ray examination. Laboratory tests conducted during the current admission showed normocytic normochromic anemia, renal dysfunction and mild proteinuria. Total IgG was normal, but a high proportion of IgG2 was observed. M-protein in the serum was positive for both IgG lambda and Bence Jones protein (lambda type). A bone marrow biopsy showed the proportion of plasma cells to be 10.6%, but atypical cells were not found. We diagnosed the patient's condition as plasma cell dyscrasia. Light microscopy examination of a renal biopsy specimen showed moderate mesangial proliferation with a deposition of PAS-positive and Congo red negative materials in the mesangial area: nodular gomerulonephritis was seen in some glomeruli. Immunofluorescence revealed IgG and lambda light chains, strong linear staining along the glomerular basement membrane and tubular basement membrane and positivity in the mesangial area. Results of staining for IgA, IgM, fibrinogen and C3 were weakly positive in the mesangium area, while those for C4, Clq and free kappa were negative. Positive staining of IgG2 was seen by immunoperoxidase study, but the tissue was negative for IgG1, IgG3, IgG4. Electron microscopy demonstrated a dense granular deposition in the mesangial, subendothelial and peritubular area and a microfibrillar structure in the mesangial area. The diameter of the microfibrillar structure was 14 nm on the average.(ABSTRACT TRUNCATED AT 250 WORDS)

UI MeSH Term Description Entries
D007146 Immunoglobulin lambda-Chains One of the types of light chain subunits of the immunoglobulins with a molecular weight of approximately 22 kDa. Ig lambda Chains,Immunoglobulins, lambda-Chain,Immunoglobulin lambda-Chain,lambda-1-Immunoglobulin,lambda-2-Immunoglobulin,lambda-Chain Immunoglobulins,lambda-Immunoglobulin Light Chain,lambda-Immunoglobulin Light Chains,lambda-x Immunoglobulin,Chains, Ig lambda,Chains, lambda-Immunoglobulin Light,Immunoglobulin lambda Chain,Immunoglobulin lambda Chains,Immunoglobulin, lambda-x,Immunoglobulins, lambda Chain,Light Chain, lambda-Immunoglobulin,Light Chains, lambda-Immunoglobulin,lambda 1 Immunoglobulin,lambda 2 Immunoglobulin,lambda Chain Immunoglobulins,lambda Chains, Ig,lambda Immunoglobulin Light Chain,lambda Immunoglobulin Light Chains,lambda x Immunoglobulin,lambda-Chain, Immunoglobulin,lambda-Chains, Immunoglobulin
D007147 Immunoglobulin Light Chains Polypeptide chains, consisting of 211 to 217 amino acid residues and having a molecular weight of approximately 22 kDa. There are two major types of light chains, kappa and lambda. Two Ig light chains and two Ig heavy chains (IMMUNOGLOBULIN HEAVY CHAINS) make one immunoglobulin molecule. Ig Light Chains,Immunoglobulins, Light-Chain,Immunoglobulin Light Chain,Immunoglobulin Light-Chain,Light-Chain Immunoglobulins,Chains, Ig Light,Chains, Immunoglobulin Light,Immunoglobulins, Light Chain,Light Chain Immunoglobulins,Light Chain, Immunoglobulin,Light Chains, Ig,Light Chains, Immunoglobulin,Light-Chain, Immunoglobulin
D008297 Male Males
D006362 Heavy Chain Disease A disorder of immunoglobulin synthesis in which large quantities of abnormal heavy chains are excreted in the urine. The amino acid sequences of the N-(amino-) terminal regions of these chains are normal, but they have a deletion extending from part of the variable domain through the first domain of the constant region, so that they cannot form cross-links to the light chains. The defect arises through faulty coupling of the variable (V) and constant (C) region genes. Franklin Disease,gamma-Chain Disease,mu-Chain Disease,Franklin's Disease,Franklins Disease,Heavy Chain Diseases,gamma Chain Disease,gamma-Chain Diseases,mu Chain Disease,mu-Chain Diseases
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000368 Aged A person 65 years of age or older. For a person older than 79 years, AGED, 80 AND OVER is available. Elderly
D015432 Glomerulonephritis, Membranoproliferative Chronic glomerulonephritis characterized histologically by proliferation of MESANGIAL CELLS, increase in the MESANGIAL EXTRACELLULAR MATRIX, and a thickening of the glomerular capillary walls. This may appear as a primary disorder or secondary to other diseases including infections and autoimmune disease SYSTEMIC LUPUS ERYTHEMATOSUS. Various subtypes are classified by their abnormal ultrastructures and immune deposits. Hypocomplementemia is a characteristic feature of all types of MPGN. C3G Complement 3 Glomerulopathy,Complement 3 Glomerulopathies,Complement 3 Glomerulopathy,Glomerulonephritis, Mesangiocapillary,MPGN Membranoproliferative Glomerulonephritis,Membranoproliferative Glomerulonephritis,Mesangiocapillary Glomerulonephritis,DDD MPGNII,Dense Deposit Disease,Glomerulonephritis, Hypocomplementemic,MPGNII,Membranoproliferative Glomerulonephritis Type II,Membranoproliferative Glomerulonephritis, Type I,Membranoproliferative Glomerulonephritis, Type II,Membranoproliferative Glomerulonephritis, Type III,Mesangiocapillary Glomerulonephritis, Type I,Mesangiocapillary Glomerulonephritis, Type II,Subendothelial Membranoproliferative Glomerulonephritis,Type II MPGN,DDD MPGNIIs,Glomerulonephritides, MPGN Membranoproliferative,Glomerulonephritides, Membranoproliferative,Glomerulonephritis, MPGN Membranoproliferative,Glomerulopathies, Complement 3,Glomerulopathy, Complement 3,Hypocomplementemic Glomerulonephritides,Hypocomplementemic Glomerulonephritis,MPGN Membranoproliferative Glomerulonephritides,MPGN, Type II,MPGNII, DDD,MPGNIIs,Membranoproliferative Glomerulonephritides,Membranoproliferative Glomerulonephritides, MPGN,Membranoproliferative Glomerulonephritis, MPGN,Membranoproliferative Glomerulonephritis, Subendothelial,Mesangiocapillary Glomerulonephritides,Type II MPGNs
D017809 Fatal Outcome Death resulting from the presence of a disease in an individual, as shown by a single case report or a limited number of patients. This should be differentiated from DEATH, the physiological cessation of life and from MORTALITY, an epidemiological or statistical concept. Fatal Outcomes,Outcome, Fatal,Outcomes, Fatal

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