Significado clínico y frecuencia de la alteración genético/molecular 11q23/MLL en lactantes con leucemia aguda en Chile

Background: Acute leukemia (AL) in infants generally shows distinctive biologic features and has a poor prognosis. Aim: To study the frequency of the cytogenetic alteration of11q23 chromosome or the recombination of MLL gene in infants less than 18 months old, with acute leukemia. Patients and metho...

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Autores principales:	Cabrera C,María Elena, Campbell B,Myriam, Quintana,Juan, Undurraga S,María Soledad, Ford,Anthony A, Greaves,Mel F
Lenguaje:	Spanish / Castilian
Publicado:	Sociedad Médica de Santiago 2001
Materias:	Genetic markers Genetic techniques Leukemia, acute lymphoblastic
Acceso en línea:	http://www.scielo.cl/scielo.php?script=sci_arttext&pid=S0034-98872001000600006
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spelling	oai:scielo:S0034-988720010006000062001-09-24Significado clínico y frecuencia de la alteración genético/molecular 11q23/MLL en lactantes con leucemia aguda en ChileCabrera C,María ElenaCampbell B,MyriamQuintana,JuanUndurraga S,María SoledadFord,Anthony AGreaves,Mel F Genetic markers Genetic techniques Leukemia, acute lymphoblastic Background: Acute leukemia (AL) in infants generally shows distinctive biologic features and has a poor prognosis. Aim: To study the frequency of the cytogenetic alteration of11q23 chromosome or the recombination of MLL gene in infants less than 18 months old, with acute leukemia. Patients and methods: We analyzed 37 cases of AL in infants less than 18 months of age diagnosed in Chile from 1989 to 1999. The clinical features and cytogenetic/molecular defects of 11q23MLL gene rearrangement and their influence in prognosis were determined. Results: There were 18 cases of acute Lymphoblastic leukemia (ALL) characterized by female sex (67%) high presenting leukocyte count (median 99 x109/L), blast cells with a CD10 negative phenotype (50%) and 11q23/MLL rearrangement (39%). Molecular abnormalities of 11q23 were significantly associated with adverse prognosis, with an event free survival (EFS) of only 14 ± 12%. Interestingly, infants with germ line 11q23 had a very good outcome with an EFS of 73 ± 11% (p<0.025). There were 19 cases of acute myeloblastic leukemia (AML) characterized by male sex (63%) high leukocyte count (median 93 x 109/L), FAB-MS morphology (53%) and 11q23/MLL rearrangement (53%). EFS was very poor, 20 ± 9% and 33±4% for rearranged and germinal group respectively (p=NS), due to a high mortality rate during the first month of diagnosis. Conclusions: These findings demonstrate that Chilean ALL infants with 11q23 abnormalities have a very poor prognosis. However those with germinal state can enjoy a prolonged disease free survival with the current treatment protocols (Rev Méd Chile2001; 129: 634-642)info:eu-repo/semantics/openAccessSociedad Médica de SantiagoRevista médica de Chile v.129 n.6 20012001-06-01text/htmlhttp://www.scielo.cl/scielo.php?script=sci_arttext&pid=S0034-98872001000600006es10.4067/S0034-98872001000600006
institution	Scielo Chile
collection	Scielo Chile
language	Spanish / Castilian
topic	Genetic markers Genetic techniques Leukemia, acute lymphoblastic
spellingShingle	Genetic markers Genetic techniques Leukemia, acute lymphoblastic Cabrera C,María Elena Campbell B,Myriam Quintana,Juan Undurraga S,María Soledad Ford,Anthony A Greaves,Mel F Significado clínico y frecuencia de la alteración genético/molecular 11q23/MLL en lactantes con leucemia aguda en Chile
description	Background: Acute leukemia (AL) in infants generally shows distinctive biologic features and has a poor prognosis. Aim: To study the frequency of the cytogenetic alteration of11q23 chromosome or the recombination of MLL gene in infants less than 18 months old, with acute leukemia. Patients and methods: We analyzed 37 cases of AL in infants less than 18 months of age diagnosed in Chile from 1989 to 1999. The clinical features and cytogenetic/molecular defects of 11q23MLL gene rearrangement and their influence in prognosis were determined. Results: There were 18 cases of acute Lymphoblastic leukemia (ALL) characterized by female sex (67%) high presenting leukocyte count (median 99 x109/L), blast cells with a CD10 negative phenotype (50%) and 11q23/MLL rearrangement (39%). Molecular abnormalities of 11q23 were significantly associated with adverse prognosis, with an event free survival (EFS) of only 14 ± 12%. Interestingly, infants with germ line 11q23 had a very good outcome with an EFS of 73 ± 11% (p<0.025). There were 19 cases of acute myeloblastic leukemia (AML) characterized by male sex (63%) high leukocyte count (median 93 x 109/L), FAB-MS morphology (53%) and 11q23/MLL rearrangement (53%). EFS was very poor, 20 ± 9% and 33±4% for rearranged and germinal group respectively (p=NS), due to a high mortality rate during the first month of diagnosis. Conclusions: These findings demonstrate that Chilean ALL infants with 11q23 abnormalities have a very poor prognosis. However those with germinal state can enjoy a prolonged disease free survival with the current treatment protocols (Rev Méd Chile2001; 129: 634-642)
author	Cabrera C,María Elena Campbell B,Myriam Quintana,Juan Undurraga S,María Soledad Ford,Anthony A Greaves,Mel F
author_facet	Cabrera C,María Elena Campbell B,Myriam Quintana,Juan Undurraga S,María Soledad Ford,Anthony A Greaves,Mel F
author_sort	Cabrera C,María Elena
title	Significado clínico y frecuencia de la alteración genético/molecular 11q23/MLL en lactantes con leucemia aguda en Chile
title_short	Significado clínico y frecuencia de la alteración genético/molecular 11q23/MLL en lactantes con leucemia aguda en Chile
title_full	Significado clínico y frecuencia de la alteración genético/molecular 11q23/MLL en lactantes con leucemia aguda en Chile
title_fullStr	Significado clínico y frecuencia de la alteración genético/molecular 11q23/MLL en lactantes con leucemia aguda en Chile
title_full_unstemmed	Significado clínico y frecuencia de la alteración genético/molecular 11q23/MLL en lactantes con leucemia aguda en Chile
title_sort	significado clínico y frecuencia de la alteración genético/molecular 11q23/mll en lactantes con leucemia aguda en chile
publisher	Sociedad Médica de Santiago
publishDate	2001
url	http://www.scielo.cl/scielo.php?script=sci_arttext&pid=S0034-98872001000600006
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Significado clínico y frecuencia de la alteración genético/molecular 11q23/MLL en lactantes con leucemia aguda en Chile

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