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[Diclonal gammopathy (IgG kappa, IgA lambda) with nephrotic syndrome terminating into IgA lambda myeloma after three years--report of a case]. 1990

H Shigemi, and S Murakami, and N Oku, and K Itoh, and N Fujita, and S Nakanishi, and C Shimazaki, and D Inoue, and H Takahashi, and M Nakagawa
Second Department of Medicine, Kyoto Prefectural University of Medicine.

A 43-year-old man was admitted to our hospital because of legs edema and periorbital edema in Dec. 1983. Laboratory findings showed massive proteinuria (3.7 g/day), Bence Jones protein (BJP) in urine, and hypoproteinemia. Peripheral blood examinations were normal and a bone marrow aspiration showed hypocellularity with slight increase of monocytes and plasma cells. Serum immunoelectrophoresis showed two M-components (IgG kappa, IgA lambda). Serum IgG was 1,690 mg/dl, IgA 379 mg/dl and IgM 160 mg/dl. No remarkable findings were obtained in bone survey, Ga-scintigraphy and rectal biopsy, and a diagnosis of diclonal gammopathy with nephrotic syndrome was made. In Aug. 1986, serum IgA started to increase rapidly with concomitant decrease IgG. He died of pneumonia due to pancytopenia in Dec. 1986, when serum IgG was 450 mg/dl, IgA 1,014 mg/dl, and IgM less than 39 mg/dl. Immunoelectrophoresis showed two M-components (IgG kappa, IgA lambda) in serum and BEP (kappa, lambda), IgG (kappa) and IgA (lambda) in urine. An autopsy showed massive infiltration of myeloma cells which were positive for lambda light chain in bone marrow, suggesting a development of myeloma from a diclonal gammopathy in about 3 years.

UI MeSH Term Description Entries
D007070 Immunoglobulin A Represents 15-20% of the human serum immunoglobulins, mostly as the 4-chain polymer in humans or dimer in other mammals. Secretory IgA (IMMUNOGLOBULIN A, SECRETORY) is the main immunoglobulin in secretions. IgA,IgA Antibody,IgA1,IgA2,Antibody, IgA
D007145 Immunoglobulin kappa-Chains One of the types of light chains of the immunoglobulins with a molecular weight of approximately 22 kDa. Ig kappa Chains,Immunoglobulins, kappa-Chain,kappa-Immunoglobulin Light Chains,Immunoglobulin kappa-Chain,kappa-Chain Immunoglobulins,kappa-Immunoglobulin Light Chain,kappa-Immunoglobulin Subgroup VK-12,kappa-Immunoglobulin Subgroup VK-21,Chains, Ig kappa,Immunoglobulin kappa Chain,Immunoglobulin kappa Chains,Immunoglobulins, kappa Chain,Light Chain, kappa-Immunoglobulin,Light Chains, kappa-Immunoglobulin,kappa Chain Immunoglobulins,kappa Chains, Ig,kappa Immunoglobulin Light Chain,kappa Immunoglobulin Light Chains,kappa Immunoglobulin Subgroup VK 12,kappa Immunoglobulin Subgroup VK 21,kappa-Chain, Immunoglobulin,kappa-Chains, Immunoglobulin
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
D008297 Male Males
D009101 Multiple Myeloma A malignancy of mature PLASMA CELLS engaging in monoclonal immunoglobulin production. It is characterized by hyperglobulinemia, excess Bence-Jones proteins (free monoclonal IMMUNOGLOBULIN LIGHT CHAINS) in the urine, skeletal destruction, bone pain, and fractures. Other features include ANEMIA; HYPERCALCEMIA; and RENAL INSUFFICIENCY. Myeloma, Plasma-Cell,Kahler Disease,Myeloma, Multiple,Myeloma-Multiple,Myelomatosis,Plasma Cell Myeloma,Cell Myeloma, Plasma,Cell Myelomas, Plasma,Disease, Kahler,Multiple Myelomas,Myeloma Multiple,Myeloma, Plasma Cell,Myeloma-Multiples,Myelomas, Multiple,Myelomas, Plasma Cell,Myelomas, Plasma-Cell,Myelomatoses,Plasma Cell Myelomas,Plasma-Cell Myeloma,Plasma-Cell Myelomas
D009194 Myeloma Proteins Abnormal immunoglobulins characteristic of MULTIPLE MYELOMA. M Components,Proteins, Myeloma
D009404 Nephrotic Syndrome A condition characterized by severe PROTEINURIA, greater than 3.5 g/day in an average adult. The substantial loss of protein in the urine results in complications such as HYPOPROTEINEMIA; generalized EDEMA; HYPERTENSION; and HYPERLIPIDEMIAS. Diseases associated with nephrotic syndrome generally cause chronic kidney dysfunction. Childhood Idiopathic Nephrotic Syndrome,Frequently Relapsing Nephrotic Syndrome,Multi-Drug Resistant Nephrotic Syndrome,Pediatric Idiopathic Nephrotic Syndrome,Steroid-Dependent Nephrotic Syndrome,Steroid-Resistant Nephrotic Syndrome,Steroid-Sensitive Nephrotic Syndrome,Multi Drug Resistant Nephrotic Syndrome,Nephrotic Syndrome, Steroid-Dependent,Nephrotic Syndrome, Steroid-Resistant,Nephrotic Syndrome, Steroid-Sensitive,Nephrotic Syndromes,Steroid Dependent Nephrotic Syndrome,Steroid Resistant Nephrotic Syndrome,Steroid Sensitive Nephrotic Syndrome,Steroid-Dependent Nephrotic Syndromes,Steroid-Resistant Nephrotic Syndromes,Steroid-Sensitive Nephrotic Syndromes,Syndrome, Nephrotic,Syndrome, Steroid-Sensitive Nephrotic
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D006942 Hypergammaglobulinemia An excess of GAMMA-GLOBULINS in the serum due to chronic infections or PARAPROTEINEMIAS. Hyperimmunoglobulinemia,Hypergammaglobulinemias,Hyperimmunoglobulinemias
D000328 Adult A person having attained full growth or maturity. Adults are of 19 through 44 years of age. For a person between 19 and 24 years of age, YOUNG ADULT is available. Adults

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