Chromosomal Translocation Involving the Immunoglobulin Kappa-Chain and Heavy-Chain Loci in a Child with Chronic Lymphocytic Leukemia

Chronic lymphocytic leukemia is usually a monoclonal B-cell neoplasm that occurs almost exclusively in middle-aged and elderly adults. We observed a rapidly progressive case of the disease in a 10-year-old girl. The leukemic cells bore surface IgM and IgD immunoglobulins of the kappa-light-chain typ...

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Veröffentlicht in:The New England journal of medicine 1983-09, Vol.309 (10), p.590-594
Hauptverfasser: Sonnier, Joseph A, Buchanan, George R, Howard-Peebles, Patricia N, Rutledge, Joe, Smith, R. Graham
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container_issue 10
container_start_page 590
container_title The New England journal of medicine
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creator Sonnier, Joseph A
Buchanan, George R
Howard-Peebles, Patricia N
Rutledge, Joe
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description Chronic lymphocytic leukemia is usually a monoclonal B-cell neoplasm that occurs almost exclusively in middle-aged and elderly adults. We observed a rapidly progressive case of the disease in a 10-year-old girl. The leukemic cells bore surface IgM and IgD immunoglobulins of the kappa-light-chain type. Karyotyping of the abnormal cells revealed an unusual translocation [t(2;14) (p13;q32)], with break points at or near the kappa-light-chain and heavy-chain gene loci. Translocations involving the immunoglobulin gene loci may be important in the pathogenesis of some cases of chronic lymphocytic leukemia, as they appear to be in Burkitt's lymphoma. (N Engl J Med 1983; 309:590–4.) CHRONIC lymphocytic leukemia is usually a monoclonal B-cell neoplasm 1 , 2 of middle-aged and elderly adults. Although rare cases have been reported in children, 3 4 5 none has been studied with modern immunologic and cytogenetic techniques. We have recently diagnosed chronic lymphocytic leukemia in a 10-year-old girl, whose leukemic cells expressed IgM and IgD kappa membrane immunoglobulins. Cytogenetic analysis showed a balanced 2p/14q translocation — an unexpected abnormality in this disease. 6 The exchange of genetic material between the short arm of chromosome 2 and the long arm of chromosome 14 occurred at bands p13 and q32, respectively. This finding is of great interest, since . . .
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Graham</creatorcontrib><title>Chromosomal Translocation Involving the Immunoglobulin Kappa-Chain and Heavy-Chain Loci in a Child with Chronic Lymphocytic Leukemia</title><title>The New England journal of medicine</title><addtitle>N Engl J Med</addtitle><description>Chronic lymphocytic leukemia is usually a monoclonal B-cell neoplasm that occurs almost exclusively in middle-aged and elderly adults. We observed a rapidly progressive case of the disease in a 10-year-old girl. The leukemic cells bore surface IgM and IgD immunoglobulins of the kappa-light-chain type. Karyotyping of the abnormal cells revealed an unusual translocation [t(2;14) (p13;q32)], with break points at or near the kappa-light-chain and heavy-chain gene loci. Translocations involving the immunoglobulin gene loci may be important in the pathogenesis of some cases of chronic lymphocytic leukemia, as they appear to be in Burkitt's lymphoma. (N Engl J Med 1983; 309:590–4.) 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Graham</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>Chromosomal Translocation Involving the Immunoglobulin Kappa-Chain and Heavy-Chain Loci in a Child with Chronic Lymphocytic Leukemia</atitle><jtitle>The New England journal of medicine</jtitle><addtitle>N Engl J Med</addtitle><date>1983-09-08</date><risdate>1983</risdate><volume>309</volume><issue>10</issue><spage>590</spage><epage>594</epage><pages>590-594</pages><issn>0028-4793</issn><eissn>1533-4406</eissn><abstract>Chronic lymphocytic leukemia is usually a monoclonal B-cell neoplasm that occurs almost exclusively in middle-aged and elderly adults. We observed a rapidly progressive case of the disease in a 10-year-old girl. The leukemic cells bore surface IgM and IgD immunoglobulins of the kappa-light-chain type. Karyotyping of the abnormal cells revealed an unusual translocation [t(2;14) (p13;q32)], with break points at or near the kappa-light-chain and heavy-chain gene loci. Translocations involving the immunoglobulin gene loci may be important in the pathogenesis of some cases of chronic lymphocytic leukemia, as they appear to be in Burkitt's lymphoma. (N Engl J Med 1983; 309:590–4.) CHRONIC lymphocytic leukemia is usually a monoclonal B-cell neoplasm 1 , 2 of middle-aged and elderly adults. Although rare cases have been reported in children, 3 4 5 none has been studied with modern immunologic and cytogenetic techniques. We have recently diagnosed chronic lymphocytic leukemia in a 10-year-old girl, whose leukemic cells expressed IgM and IgD kappa membrane immunoglobulins. Cytogenetic analysis showed a balanced 2p/14q translocation — an unexpected abnormality in this disease. 6 The exchange of genetic material between the short arm of chromosome 2 and the long arm of chromosome 14 occurred at bands p13 and q32, respectively. This finding is of great interest, since . . .</abstract><cop>United States</cop><pub>Massachusetts Medical Society</pub><pmid>6410236</pmid><doi>10.1056/NEJM198309083091005</doi><tpages>5</tpages></addata></record>
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subjects Abdomen
Blood platelets
Bone marrow
Burkitt's lymphoma
Child
Chromosome Mapping
Chromosome translocations
Chromosomes
Chronic lymphocytic leukemia
Cytoplasm
Female
Genes
Geriatrics
Heavy Chain Disease - history
History of medicine
Humans
Immunoglobulin D
Immunoglobulin D - analysis
Immunoglobulin D - genetics
Immunoglobulin kappa-Chains - analysis
Immunoglobulin kappa-Chains - genetics
Immunoglobulin Light Chains - genetics
Immunoglobulin M
Immunoglobulin M - analysis
Immunoglobulin M - genetics
Immunoglobulins
Karyotyping
Leukemia
Leukemia, Lymphoid - genetics
Leukemia, Lymphoid - immunology
Light
Lymphatic leukemia
Lymphatic system
Lymphocytes
Lymphocytes B
Lymphoma
Pediatrics
Translocation, Genetic
title Chromosomal Translocation Involving the Immunoglobulin Kappa-Chain and Heavy-Chain Loci in a Child with Chronic Lymphocytic Leukemia
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