Hemoglobinúria paroxística noturna e gravidez Paroxysmal nocturnal hemoglobinuria in pregnancy

A hemoglobinúria paroxística noturna é doença rara, causada por mutação adquirida de um gene no sistema hematopoético com 16-18% dos casos diagnosticados durante a gravidez. Descrevemos dois casos de gestantes portadoras de hemoglobinúria paroxística noturna com diagnóstico anterior à gestação. A mo...
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Autor*in:	Marcelo Luís Nomura [verfasserIn] Fernanda Garanhani de Castro Surita [verfasserIn] Mary Angela Parpinelli [verfasserIn] Egle Cristina Couto Carvalho [verfasserIn] Renato Passini Júnior [verfasserIn]

Format:	E-Artikel
Sprache:	Englisch ; Portugiesisch

Erschienen:	2004

Schlagwörter:	Hemoglobinúria Aplasia medular Gestação Hemoglobinuria Medullary aplasia Pregnancy complications

Übergeordnetes Werk:	In: Revista Brasileira de Ginecologia e Obstetrícia - Federação Brasileira das Sociedades de Ginecologia e Obstetrícia, 2004, 26(2004), 7, Seite 579-582
Übergeordnetes Werk:	volume:26 ; year:2004 ; number:7 ; pages:579-582

Links:	Link aufrufen Link aufrufen Link aufrufen Journal toc

DOI / URN:	10.1590/S0100-72032004000700011

Katalog-ID:	DOAJ024048488

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520			\|a A hemoglobinúria paroxística noturna é doença rara, causada por mutação adquirida de um gene no sistema hematopoético com 16-18% dos casos diagnosticados durante a gravidez. Descrevemos dois casos de gestantes portadoras de hemoglobinúria paroxística noturna com diagnóstico anterior à gestação. A mortalidade materna é de 8-10%, devido principalmente a tromboembolismo e, em menor escala, transformação leucêmica. As perdas fetais chegam a 30% das gestações. Estes dois casos ilustram situação grave e extremamente complexa, que é a conduta obstétrica em mulheres portadoras de doença hematológica muito rara, grave e potencialmente fatal. Com uma abordagem multidisciplinar em serviços terciários é possível obter bons resultados maternos e perinatais.<br<Paroxysmal nocturnal hemoglobinuria is a rare disease caused by an acquired gene mutation of the hematopoietic system, with 16-18% of the cases diagnosed during pregnancy. We describe two cases of pregnancy in women with paroxysmal nocturnal hemoglobinuria. Maternal mortality reaches 8-10%, mainly due to thromboembolism and, less frequently, to leukemic transformation. Fetal losses may reach 30%. These two cases illustrate a serious and extremely complex situation, which is the obstetrical management of a patient with a very rare, serious and potentially fatal hematological condition. Using a multidisciplinary approach in tertiary care centers, it is possible to attain good maternal and perinatal outcomes.
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allfields	10.1590/S0100-72032004000700011 doi (DE-627)DOAJ024048488 (DE-599)DOAJ982d8dc07312412bbed24f1e25609e9e DE-627 ger DE-627 rakwb eng por RG1-991 Marcelo Luís Nomura verfasserin aut Hemoglobinúria paroxística noturna e gravidez Paroxysmal nocturnal hemoglobinuria in pregnancy 2004 Text txt rdacontent Computermedien c rdamedia Online-Ressource cr rdacarrier A hemoglobinúria paroxística noturna é doença rara, causada por mutação adquirida de um gene no sistema hematopoético com 16-18% dos casos diagnosticados durante a gravidez. Descrevemos dois casos de gestantes portadoras de hemoglobinúria paroxística noturna com diagnóstico anterior à gestação. A mortalidade materna é de 8-10%, devido principalmente a tromboembolismo e, em menor escala, transformação leucêmica. As perdas fetais chegam a 30% das gestações. Estes dois casos ilustram situação grave e extremamente complexa, que é a conduta obstétrica em mulheres portadoras de doença hematológica muito rara, grave e potencialmente fatal. Com uma abordagem multidisciplinar em serviços terciários é possível obter bons resultados maternos e perinatais.<br<Paroxysmal nocturnal hemoglobinuria is a rare disease caused by an acquired gene mutation of the hematopoietic system, with 16-18% of the cases diagnosed during pregnancy. We describe two cases of pregnancy in women with paroxysmal nocturnal hemoglobinuria. Maternal mortality reaches 8-10%, mainly due to thromboembolism and, less frequently, to leukemic transformation. Fetal losses may reach 30%. These two cases illustrate a serious and extremely complex situation, which is the obstetrical management of a patient with a very rare, serious and potentially fatal hematological condition. Using a multidisciplinary approach in tertiary care centers, it is possible to attain good maternal and perinatal outcomes. Hemoglobinúria Aplasia medular Gestação Hemoglobinuria Medullary aplasia Pregnancy complications Gynecology and obstetrics Fernanda Garanhani de Castro Surita verfasserin aut Mary Angela Parpinelli verfasserin aut Egle Cristina Couto Carvalho verfasserin aut Renato Passini Júnior verfasserin aut In Revista Brasileira de Ginecologia e Obstetrícia Federação Brasileira das Sociedades de Ginecologia e Obstetrícia, 2004 26(2004), 7, Seite 579-582 (DE-627)363749608 (DE-600)2105176-8 01007203 nnns volume:26 year:2004 number:7 pages:579-582 https://doi.org/10.1590/S0100-72032004000700011 kostenfrei https://doaj.org/article/982d8dc07312412bbed24f1e25609e9e kostenfrei http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0100-72032004000700011 kostenfrei https://doaj.org/toc/0100-7203 Journal toc kostenfrei GBV_USEFLAG_A SYSFLAG_A GBV_DOAJ GBV_ILN_20 GBV_ILN_22 GBV_ILN_23 GBV_ILN_24 GBV_ILN_39 GBV_ILN_40 GBV_ILN_60 GBV_ILN_62 GBV_ILN_63 GBV_ILN_65 GBV_ILN_69 GBV_ILN_73 GBV_ILN_74 GBV_ILN_95 GBV_ILN_105 GBV_ILN_110 GBV_ILN_151 GBV_ILN_161 GBV_ILN_170 GBV_ILN_206 GBV_ILN_213 GBV_ILN_230 GBV_ILN_285 GBV_ILN_293 GBV_ILN_602 GBV_ILN_2014 GBV_ILN_4012 GBV_ILN_4037 GBV_ILN_4112 GBV_ILN_4125 GBV_ILN_4126 GBV_ILN_4249 GBV_ILN_4305 GBV_ILN_4306 GBV_ILN_4307 GBV_ILN_4313 GBV_ILN_4322 GBV_ILN_4323 GBV_ILN_4324 GBV_ILN_4325 GBV_ILN_4338 GBV_ILN_4367 GBV_ILN_4700 AR 26 2004 7 579-582
spelling	10.1590/S0100-72032004000700011 doi (DE-627)DOAJ024048488 (DE-599)DOAJ982d8dc07312412bbed24f1e25609e9e DE-627 ger DE-627 rakwb eng por RG1-991 Marcelo Luís Nomura verfasserin aut Hemoglobinúria paroxística noturna e gravidez Paroxysmal nocturnal hemoglobinuria in pregnancy 2004 Text txt rdacontent Computermedien c rdamedia Online-Ressource cr rdacarrier A hemoglobinúria paroxística noturna é doença rara, causada por mutação adquirida de um gene no sistema hematopoético com 16-18% dos casos diagnosticados durante a gravidez. Descrevemos dois casos de gestantes portadoras de hemoglobinúria paroxística noturna com diagnóstico anterior à gestação. A mortalidade materna é de 8-10%, devido principalmente a tromboembolismo e, em menor escala, transformação leucêmica. As perdas fetais chegam a 30% das gestações. Estes dois casos ilustram situação grave e extremamente complexa, que é a conduta obstétrica em mulheres portadoras de doença hematológica muito rara, grave e potencialmente fatal. Com uma abordagem multidisciplinar em serviços terciários é possível obter bons resultados maternos e perinatais.<br<Paroxysmal nocturnal hemoglobinuria is a rare disease caused by an acquired gene mutation of the hematopoietic system, with 16-18% of the cases diagnosed during pregnancy. We describe two cases of pregnancy in women with paroxysmal nocturnal hemoglobinuria. Maternal mortality reaches 8-10%, mainly due to thromboembolism and, less frequently, to leukemic transformation. Fetal losses may reach 30%. These two cases illustrate a serious and extremely complex situation, which is the obstetrical management of a patient with a very rare, serious and potentially fatal hematological condition. Using a multidisciplinary approach in tertiary care centers, it is possible to attain good maternal and perinatal outcomes. Hemoglobinúria Aplasia medular Gestação Hemoglobinuria Medullary aplasia Pregnancy complications Gynecology and obstetrics Fernanda Garanhani de Castro Surita verfasserin aut Mary Angela Parpinelli verfasserin aut Egle Cristina Couto Carvalho verfasserin aut Renato Passini Júnior verfasserin aut In Revista Brasileira de Ginecologia e Obstetrícia Federação Brasileira das Sociedades de Ginecologia e Obstetrícia, 2004 26(2004), 7, Seite 579-582 (DE-627)363749608 (DE-600)2105176-8 01007203 nnns volume:26 year:2004 number:7 pages:579-582 https://doi.org/10.1590/S0100-72032004000700011 kostenfrei https://doaj.org/article/982d8dc07312412bbed24f1e25609e9e kostenfrei http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0100-72032004000700011 kostenfrei https://doaj.org/toc/0100-7203 Journal toc kostenfrei GBV_USEFLAG_A SYSFLAG_A GBV_DOAJ GBV_ILN_20 GBV_ILN_22 GBV_ILN_23 GBV_ILN_24 GBV_ILN_39 GBV_ILN_40 GBV_ILN_60 GBV_ILN_62 GBV_ILN_63 GBV_ILN_65 GBV_ILN_69 GBV_ILN_73 GBV_ILN_74 GBV_ILN_95 GBV_ILN_105 GBV_ILN_110 GBV_ILN_151 GBV_ILN_161 GBV_ILN_170 GBV_ILN_206 GBV_ILN_213 GBV_ILN_230 GBV_ILN_285 GBV_ILN_293 GBV_ILN_602 GBV_ILN_2014 GBV_ILN_4012 GBV_ILN_4037 GBV_ILN_4112 GBV_ILN_4125 GBV_ILN_4126 GBV_ILN_4249 GBV_ILN_4305 GBV_ILN_4306 GBV_ILN_4307 GBV_ILN_4313 GBV_ILN_4322 GBV_ILN_4323 GBV_ILN_4324 GBV_ILN_4325 GBV_ILN_4338 GBV_ILN_4367 GBV_ILN_4700 AR 26 2004 7 579-582
allfields_unstemmed	10.1590/S0100-72032004000700011 doi (DE-627)DOAJ024048488 (DE-599)DOAJ982d8dc07312412bbed24f1e25609e9e DE-627 ger DE-627 rakwb eng por RG1-991 Marcelo Luís Nomura verfasserin aut Hemoglobinúria paroxística noturna e gravidez Paroxysmal nocturnal hemoglobinuria in pregnancy 2004 Text txt rdacontent Computermedien c rdamedia Online-Ressource cr rdacarrier A hemoglobinúria paroxística noturna é doença rara, causada por mutação adquirida de um gene no sistema hematopoético com 16-18% dos casos diagnosticados durante a gravidez. Descrevemos dois casos de gestantes portadoras de hemoglobinúria paroxística noturna com diagnóstico anterior à gestação. A mortalidade materna é de 8-10%, devido principalmente a tromboembolismo e, em menor escala, transformação leucêmica. As perdas fetais chegam a 30% das gestações. Estes dois casos ilustram situação grave e extremamente complexa, que é a conduta obstétrica em mulheres portadoras de doença hematológica muito rara, grave e potencialmente fatal. Com uma abordagem multidisciplinar em serviços terciários é possível obter bons resultados maternos e perinatais.<br<Paroxysmal nocturnal hemoglobinuria is a rare disease caused by an acquired gene mutation of the hematopoietic system, with 16-18% of the cases diagnosed during pregnancy. We describe two cases of pregnancy in women with paroxysmal nocturnal hemoglobinuria. Maternal mortality reaches 8-10%, mainly due to thromboembolism and, less frequently, to leukemic transformation. Fetal losses may reach 30%. These two cases illustrate a serious and extremely complex situation, which is the obstetrical management of a patient with a very rare, serious and potentially fatal hematological condition. Using a multidisciplinary approach in tertiary care centers, it is possible to attain good maternal and perinatal outcomes. Hemoglobinúria Aplasia medular Gestação Hemoglobinuria Medullary aplasia Pregnancy complications Gynecology and obstetrics Fernanda Garanhani de Castro Surita verfasserin aut Mary Angela Parpinelli verfasserin aut Egle Cristina Couto Carvalho verfasserin aut Renato Passini Júnior verfasserin aut In Revista Brasileira de Ginecologia e Obstetrícia Federação Brasileira das Sociedades de Ginecologia e Obstetrícia, 2004 26(2004), 7, Seite 579-582 (DE-627)363749608 (DE-600)2105176-8 01007203 nnns volume:26 year:2004 number:7 pages:579-582 https://doi.org/10.1590/S0100-72032004000700011 kostenfrei https://doaj.org/article/982d8dc07312412bbed24f1e25609e9e kostenfrei http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0100-72032004000700011 kostenfrei https://doaj.org/toc/0100-7203 Journal toc kostenfrei GBV_USEFLAG_A SYSFLAG_A GBV_DOAJ GBV_ILN_20 GBV_ILN_22 GBV_ILN_23 GBV_ILN_24 GBV_ILN_39 GBV_ILN_40 GBV_ILN_60 GBV_ILN_62 GBV_ILN_63 GBV_ILN_65 GBV_ILN_69 GBV_ILN_73 GBV_ILN_74 GBV_ILN_95 GBV_ILN_105 GBV_ILN_110 GBV_ILN_151 GBV_ILN_161 GBV_ILN_170 GBV_ILN_206 GBV_ILN_213 GBV_ILN_230 GBV_ILN_285 GBV_ILN_293 GBV_ILN_602 GBV_ILN_2014 GBV_ILN_4012 GBV_ILN_4037 GBV_ILN_4112 GBV_ILN_4125 GBV_ILN_4126 GBV_ILN_4249 GBV_ILN_4305 GBV_ILN_4306 GBV_ILN_4307 GBV_ILN_4313 GBV_ILN_4322 GBV_ILN_4323 GBV_ILN_4324 GBV_ILN_4325 GBV_ILN_4338 GBV_ILN_4367 GBV_ILN_4700 AR 26 2004 7 579-582
allfieldsGer	10.1590/S0100-72032004000700011 doi (DE-627)DOAJ024048488 (DE-599)DOAJ982d8dc07312412bbed24f1e25609e9e DE-627 ger DE-627 rakwb eng por RG1-991 Marcelo Luís Nomura verfasserin aut Hemoglobinúria paroxística noturna e gravidez Paroxysmal nocturnal hemoglobinuria in pregnancy 2004 Text txt rdacontent Computermedien c rdamedia Online-Ressource cr rdacarrier A hemoglobinúria paroxística noturna é doença rara, causada por mutação adquirida de um gene no sistema hematopoético com 16-18% dos casos diagnosticados durante a gravidez. Descrevemos dois casos de gestantes portadoras de hemoglobinúria paroxística noturna com diagnóstico anterior à gestação. A mortalidade materna é de 8-10%, devido principalmente a tromboembolismo e, em menor escala, transformação leucêmica. As perdas fetais chegam a 30% das gestações. Estes dois casos ilustram situação grave e extremamente complexa, que é a conduta obstétrica em mulheres portadoras de doença hematológica muito rara, grave e potencialmente fatal. Com uma abordagem multidisciplinar em serviços terciários é possível obter bons resultados maternos e perinatais.<br<Paroxysmal nocturnal hemoglobinuria is a rare disease caused by an acquired gene mutation of the hematopoietic system, with 16-18% of the cases diagnosed during pregnancy. We describe two cases of pregnancy in women with paroxysmal nocturnal hemoglobinuria. Maternal mortality reaches 8-10%, mainly due to thromboembolism and, less frequently, to leukemic transformation. Fetal losses may reach 30%. These two cases illustrate a serious and extremely complex situation, which is the obstetrical management of a patient with a very rare, serious and potentially fatal hematological condition. Using a multidisciplinary approach in tertiary care centers, it is possible to attain good maternal and perinatal outcomes. Hemoglobinúria Aplasia medular Gestação Hemoglobinuria Medullary aplasia Pregnancy complications Gynecology and obstetrics Fernanda Garanhani de Castro Surita verfasserin aut Mary Angela Parpinelli verfasserin aut Egle Cristina Couto Carvalho verfasserin aut Renato Passini Júnior verfasserin aut In Revista Brasileira de Ginecologia e Obstetrícia Federação Brasileira das Sociedades de Ginecologia e Obstetrícia, 2004 26(2004), 7, Seite 579-582 (DE-627)363749608 (DE-600)2105176-8 01007203 nnns volume:26 year:2004 number:7 pages:579-582 https://doi.org/10.1590/S0100-72032004000700011 kostenfrei https://doaj.org/article/982d8dc07312412bbed24f1e25609e9e kostenfrei http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0100-72032004000700011 kostenfrei https://doaj.org/toc/0100-7203 Journal toc kostenfrei GBV_USEFLAG_A SYSFLAG_A GBV_DOAJ GBV_ILN_20 GBV_ILN_22 GBV_ILN_23 GBV_ILN_24 GBV_ILN_39 GBV_ILN_40 GBV_ILN_60 GBV_ILN_62 GBV_ILN_63 GBV_ILN_65 GBV_ILN_69 GBV_ILN_73 GBV_ILN_74 GBV_ILN_95 GBV_ILN_105 GBV_ILN_110 GBV_ILN_151 GBV_ILN_161 GBV_ILN_170 GBV_ILN_206 GBV_ILN_213 GBV_ILN_230 GBV_ILN_285 GBV_ILN_293 GBV_ILN_602 GBV_ILN_2014 GBV_ILN_4012 GBV_ILN_4037 GBV_ILN_4112 GBV_ILN_4125 GBV_ILN_4126 GBV_ILN_4249 GBV_ILN_4305 GBV_ILN_4306 GBV_ILN_4307 GBV_ILN_4313 GBV_ILN_4322 GBV_ILN_4323 GBV_ILN_4324 GBV_ILN_4325 GBV_ILN_4338 GBV_ILN_4367 GBV_ILN_4700 AR 26 2004 7 579-582
allfieldsSound	10.1590/S0100-72032004000700011 doi (DE-627)DOAJ024048488 (DE-599)DOAJ982d8dc07312412bbed24f1e25609e9e DE-627 ger DE-627 rakwb eng por RG1-991 Marcelo Luís Nomura verfasserin aut Hemoglobinúria paroxística noturna e gravidez Paroxysmal nocturnal hemoglobinuria in pregnancy 2004 Text txt rdacontent Computermedien c rdamedia Online-Ressource cr rdacarrier A hemoglobinúria paroxística noturna é doença rara, causada por mutação adquirida de um gene no sistema hematopoético com 16-18% dos casos diagnosticados durante a gravidez. Descrevemos dois casos de gestantes portadoras de hemoglobinúria paroxística noturna com diagnóstico anterior à gestação. A mortalidade materna é de 8-10%, devido principalmente a tromboembolismo e, em menor escala, transformação leucêmica. As perdas fetais chegam a 30% das gestações. Estes dois casos ilustram situação grave e extremamente complexa, que é a conduta obstétrica em mulheres portadoras de doença hematológica muito rara, grave e potencialmente fatal. Com uma abordagem multidisciplinar em serviços terciários é possível obter bons resultados maternos e perinatais.<br<Paroxysmal nocturnal hemoglobinuria is a rare disease caused by an acquired gene mutation of the hematopoietic system, with 16-18% of the cases diagnosed during pregnancy. We describe two cases of pregnancy in women with paroxysmal nocturnal hemoglobinuria. Maternal mortality reaches 8-10%, mainly due to thromboembolism and, less frequently, to leukemic transformation. Fetal losses may reach 30%. These two cases illustrate a serious and extremely complex situation, which is the obstetrical management of a patient with a very rare, serious and potentially fatal hematological condition. Using a multidisciplinary approach in tertiary care centers, it is possible to attain good maternal and perinatal outcomes. Hemoglobinúria Aplasia medular Gestação Hemoglobinuria Medullary aplasia Pregnancy complications Gynecology and obstetrics Fernanda Garanhani de Castro Surita verfasserin aut Mary Angela Parpinelli verfasserin aut Egle Cristina Couto Carvalho verfasserin aut Renato Passini Júnior verfasserin aut In Revista Brasileira de Ginecologia e Obstetrícia Federação Brasileira das Sociedades de Ginecologia e Obstetrícia, 2004 26(2004), 7, Seite 579-582 (DE-627)363749608 (DE-600)2105176-8 01007203 nnns volume:26 year:2004 number:7 pages:579-582 https://doi.org/10.1590/S0100-72032004000700011 kostenfrei https://doaj.org/article/982d8dc07312412bbed24f1e25609e9e kostenfrei http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0100-72032004000700011 kostenfrei https://doaj.org/toc/0100-7203 Journal toc kostenfrei GBV_USEFLAG_A SYSFLAG_A GBV_DOAJ GBV_ILN_20 GBV_ILN_22 GBV_ILN_23 GBV_ILN_24 GBV_ILN_39 GBV_ILN_40 GBV_ILN_60 GBV_ILN_62 GBV_ILN_63 GBV_ILN_65 GBV_ILN_69 GBV_ILN_73 GBV_ILN_74 GBV_ILN_95 GBV_ILN_105 GBV_ILN_110 GBV_ILN_151 GBV_ILN_161 GBV_ILN_170 GBV_ILN_206 GBV_ILN_213 GBV_ILN_230 GBV_ILN_285 GBV_ILN_293 GBV_ILN_602 GBV_ILN_2014 GBV_ILN_4012 GBV_ILN_4037 GBV_ILN_4112 GBV_ILN_4125 GBV_ILN_4126 GBV_ILN_4249 GBV_ILN_4305 GBV_ILN_4306 GBV_ILN_4307 GBV_ILN_4313 GBV_ILN_4322 GBV_ILN_4323 GBV_ILN_4324 GBV_ILN_4325 GBV_ILN_4338 GBV_ILN_4367 GBV_ILN_4700 AR 26 2004 7 579-582
language	English Portuguese
source	In Revista Brasileira de Ginecologia e Obstetrícia 26(2004), 7, Seite 579-582 volume:26 year:2004 number:7 pages:579-582
sourceStr	In Revista Brasileira de Ginecologia e Obstetrícia 26(2004), 7, Seite 579-582 volume:26 year:2004 number:7 pages:579-582
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topic_unstemmed	misc RG1-991 misc Hemoglobinúria misc Aplasia medular misc Gestação misc Hemoglobinuria misc Medullary aplasia misc Pregnancy complications misc Gynecology and obstetrics
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title	Hemoglobinúria paroxística noturna e gravidez Paroxysmal nocturnal hemoglobinuria in pregnancy
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title_full	Hemoglobinúria paroxística noturna e gravidez Paroxysmal nocturnal hemoglobinuria in pregnancy
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title_sort	hemoglobinúria paroxística noturna e gravidez paroxysmal nocturnal hemoglobinuria in pregnancy
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title_auth	Hemoglobinúria paroxística noturna e gravidez Paroxysmal nocturnal hemoglobinuria in pregnancy
abstract	A hemoglobinúria paroxística noturna é doença rara, causada por mutação adquirida de um gene no sistema hematopoético com 16-18% dos casos diagnosticados durante a gravidez. Descrevemos dois casos de gestantes portadoras de hemoglobinúria paroxística noturna com diagnóstico anterior à gestação. A mortalidade materna é de 8-10%, devido principalmente a tromboembolismo e, em menor escala, transformação leucêmica. As perdas fetais chegam a 30% das gestações. Estes dois casos ilustram situação grave e extremamente complexa, que é a conduta obstétrica em mulheres portadoras de doença hematológica muito rara, grave e potencialmente fatal. Com uma abordagem multidisciplinar em serviços terciários é possível obter bons resultados maternos e perinatais.<br<Paroxysmal nocturnal hemoglobinuria is a rare disease caused by an acquired gene mutation of the hematopoietic system, with 16-18% of the cases diagnosed during pregnancy. We describe two cases of pregnancy in women with paroxysmal nocturnal hemoglobinuria. Maternal mortality reaches 8-10%, mainly due to thromboembolism and, less frequently, to leukemic transformation. Fetal losses may reach 30%. These two cases illustrate a serious and extremely complex situation, which is the obstetrical management of a patient with a very rare, serious and potentially fatal hematological condition. Using a multidisciplinary approach in tertiary care centers, it is possible to attain good maternal and perinatal outcomes.
abstractGer	A hemoglobinúria paroxística noturna é doença rara, causada por mutação adquirida de um gene no sistema hematopoético com 16-18% dos casos diagnosticados durante a gravidez. Descrevemos dois casos de gestantes portadoras de hemoglobinúria paroxística noturna com diagnóstico anterior à gestação. A mortalidade materna é de 8-10%, devido principalmente a tromboembolismo e, em menor escala, transformação leucêmica. As perdas fetais chegam a 30% das gestações. Estes dois casos ilustram situação grave e extremamente complexa, que é a conduta obstétrica em mulheres portadoras de doença hematológica muito rara, grave e potencialmente fatal. Com uma abordagem multidisciplinar em serviços terciários é possível obter bons resultados maternos e perinatais.<br<Paroxysmal nocturnal hemoglobinuria is a rare disease caused by an acquired gene mutation of the hematopoietic system, with 16-18% of the cases diagnosed during pregnancy. We describe two cases of pregnancy in women with paroxysmal nocturnal hemoglobinuria. Maternal mortality reaches 8-10%, mainly due to thromboembolism and, less frequently, to leukemic transformation. Fetal losses may reach 30%. These two cases illustrate a serious and extremely complex situation, which is the obstetrical management of a patient with a very rare, serious and potentially fatal hematological condition. Using a multidisciplinary approach in tertiary care centers, it is possible to attain good maternal and perinatal outcomes.
abstract_unstemmed	A hemoglobinúria paroxística noturna é doença rara, causada por mutação adquirida de um gene no sistema hematopoético com 16-18% dos casos diagnosticados durante a gravidez. Descrevemos dois casos de gestantes portadoras de hemoglobinúria paroxística noturna com diagnóstico anterior à gestação. A mortalidade materna é de 8-10%, devido principalmente a tromboembolismo e, em menor escala, transformação leucêmica. As perdas fetais chegam a 30% das gestações. Estes dois casos ilustram situação grave e extremamente complexa, que é a conduta obstétrica em mulheres portadoras de doença hematológica muito rara, grave e potencialmente fatal. Com uma abordagem multidisciplinar em serviços terciários é possível obter bons resultados maternos e perinatais.<br<Paroxysmal nocturnal hemoglobinuria is a rare disease caused by an acquired gene mutation of the hematopoietic system, with 16-18% of the cases diagnosed during pregnancy. We describe two cases of pregnancy in women with paroxysmal nocturnal hemoglobinuria. Maternal mortality reaches 8-10%, mainly due to thromboembolism and, less frequently, to leukemic transformation. Fetal losses may reach 30%. These two cases illustrate a serious and extremely complex situation, which is the obstetrical management of a patient with a very rare, serious and potentially fatal hematological condition. Using a multidisciplinary approach in tertiary care centers, it is possible to attain good maternal and perinatal outcomes.
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title_short	Hemoglobinúria paroxística noturna e gravidez Paroxysmal nocturnal hemoglobinuria in pregnancy
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Descrevemos dois casos de gestantes portadoras de hemoglobinúria paroxística noturna com diagnóstico anterior à gestação. A mortalidade materna é de 8-10%, devido principalmente a tromboembolismo e, em menor escala, transformação leucêmica. As perdas fetais chegam a 30% das gestações. Estes dois casos ilustram situação grave e extremamente complexa, que é a conduta obstétrica em mulheres portadoras de doença hematológica muito rara, grave e potencialmente fatal. Com uma abordagem multidisciplinar em serviços terciários é possível obter bons resultados maternos e perinatais.<br<Paroxysmal nocturnal hemoglobinuria is a rare disease caused by an acquired gene mutation of the hematopoietic system, with 16-18% of the cases diagnosed during pregnancy. We describe two cases of pregnancy in women with paroxysmal nocturnal hemoglobinuria. Maternal mortality reaches 8-10%, mainly due to thromboembolism and, less frequently, to leukemic transformation. Fetal losses may reach 30%. These two cases illustrate a serious and extremely complex situation, which is the obstetrical management of a patient with a very rare, serious and potentially fatal hematological condition. 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Hemoglobinúria paroxística noturna e gravidez Paroxysmal nocturnal hemoglobinuria in pregnancy

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