Kasabach-Merritt syndrome: clinical vs. surgical treatment
Autor(a) principal: | |
---|---|
Data de Publicação: | 2014 |
Outros Autores: | , , , , , , |
Tipo de documento: | Artigo |
Idioma: | eng |
Título da fonte: | Repositório Institucional da UNESP |
Texto Completo: | http://dx.doi.org/10.1590/1677-5449.0102 http://hdl.handle.net/11449/136816 |
Resumo: | Kassabach-Merritt syndrome is a combination of capillary hemangioma and thrombocytopenia that predisposes to bleeding with petechiae, ecchymosis and spontaneous bruising. Treatment is generally started with corticosteroids, interferon alpha or chemotherapy. We present the case of a child (aged 1 year and 9 months) with a giant hemangioma, from the root of the thigh to the knee, and thrombocytopenia. Treatment was started with corticosteroids, without improvement, and then intra-tumor and cutaneous bleeding appeared spontaneously. The patient’s clinical condition precluded prescription of vincristine and interferon and emergency tumor resection was conducted because of extreme thrombocytopenia and bleeding. The child then began to develop sepsis with hypotension and ischemia of remnant tissues. This case presented a therapeutic challenge, which is the subject of this article. |
id |
UNSP_66ddf650164c7a10aaf035c9743a27be |
---|---|
oai_identifier_str |
oai:repositorio.unesp.br:11449/136816 |
network_acronym_str |
UNSP |
network_name_str |
Repositório Institucional da UNESP |
repository_id_str |
2946 |
spelling |
Kasabach-Merritt syndrome: clinical vs. surgical treatmentSíndrome de Kasabach-Merritt: tratamento clínico versus cirúrgicoKasabach-Merritt syndromeAmputationCorticosteroidsHemangiomaSíndrome de Kasabach-MerrittAmputaçãoCorticoidesHemangiomaKassabach-Merritt syndrome is a combination of capillary hemangioma and thrombocytopenia that predisposes to bleeding with petechiae, ecchymosis and spontaneous bruising. Treatment is generally started with corticosteroids, interferon alpha or chemotherapy. We present the case of a child (aged 1 year and 9 months) with a giant hemangioma, from the root of the thigh to the knee, and thrombocytopenia. Treatment was started with corticosteroids, without improvement, and then intra-tumor and cutaneous bleeding appeared spontaneously. The patient’s clinical condition precluded prescription of vincristine and interferon and emergency tumor resection was conducted because of extreme thrombocytopenia and bleeding. The child then began to develop sepsis with hypotension and ischemia of remnant tissues. This case presented a therapeutic challenge, which is the subject of this article.A síndrome de Kassabach-Merritt é uma associação de hemangioma capilar e trombocitopenia, que promove sangramentos com petéquias, equimoses e hematomas espontâneos. A conduta é tratar com corticoide, interferon alfa ou quimioterápicos. Apresentamos um caso de criança (com 1 ano e 9 meses anos de idade) com um hemangioma gigante desde a raiz da coxa até altura do joelho, e plaquetopenia. O tratamento foi iniciado com corticoterapia sem melhora do quadro, surgindo de forma espontânea pontos de sangramento intratumoral e cutâneo. Sem condições clínicas de receber vincristina e o interferon, foi feita a ressecção tumoral de urgência por extrema plaquetopenia e sangramento. A criança começou então a desenvolver um quadro séptico com hipotensão e isquemia do tecido residual. Diante deste quadro, configurou-se um desafio terapêutico que será objeto deste artigo.Universidade Estadual Paulista Júlio de Mesquita Filho (UNESP), Faculdade de Medicina de Botucatu (FMB), Departamento de Cirurgia e Ortopedia, Botucatu, SP, BrasilUniversidade Estadual Paulista Júlio de Mesquita Filho (UNESP), Faculdade de Medicina de Botucatu (FMB), Departamento de Cirurgia e Ortopedia, Botucatu, SP, BrasilUniversidade Estadual Paulista (Unesp)Moura, Regina [UNESP]Sobreira, Marcone Lima [UNESP]Bertanha, Matheus [UNESP]Jaldin, Rodrigo Gibin [UNESP]Silva, Maria Madalena [UNESP]Pereira, Lied [UNESP]Takegawa, Bonifacio Katsunori [UNESP]Yoshida, Winston Bonetti [UNESP]2016-04-01T18:42:43Z2016-04-01T18:42:43Z2014info:eu-repo/semantics/publishedVersioninfo:eu-repo/semantics/article330-335application/pdfapplication/pdfhttp://dx.doi.org/10.1590/1677-5449.0102Jornal Vascular Brasileiro, v. 13, n. 4, p. 330-335, 2014.1677-5449http://hdl.handle.net/11449/13681610.1590/1677-5449.0102S1677-54492014000400330S1677-54492014000400330-en.pdfS1677-54492014000400330-pt.pdf36138352316549323086448481970671308644848197067136138352316549323613835231654932308644848197067196093248325913824513014379461383Currículo Lattesreponame:Repositório Institucional da UNESPinstname:Universidade Estadual Paulista (UNESP)instacron:UNESPengJornal Vascular Brasileiro0,136info:eu-repo/semantics/openAccess2024-01-02T06:19:17Zoai:repositorio.unesp.br:11449/136816Repositório InstitucionalPUBhttp://repositorio.unesp.br/oai/requestopendoar:29462024-01-02T06:19:17Repositório Institucional da UNESP - Universidade Estadual Paulista (UNESP)false |
dc.title.none.fl_str_mv |
Kasabach-Merritt syndrome: clinical vs. surgical treatment Síndrome de Kasabach-Merritt: tratamento clínico versus cirúrgico |
title |
Kasabach-Merritt syndrome: clinical vs. surgical treatment |
spellingShingle |
Kasabach-Merritt syndrome: clinical vs. surgical treatment Moura, Regina [UNESP] Kasabach-Merritt syndrome Amputation Corticosteroids Hemangioma Síndrome de Kasabach-Merritt Amputação Corticoides Hemangioma |
title_short |
Kasabach-Merritt syndrome: clinical vs. surgical treatment |
title_full |
Kasabach-Merritt syndrome: clinical vs. surgical treatment |
title_fullStr |
Kasabach-Merritt syndrome: clinical vs. surgical treatment |
title_full_unstemmed |
Kasabach-Merritt syndrome: clinical vs. surgical treatment |
title_sort |
Kasabach-Merritt syndrome: clinical vs. surgical treatment |
author |
Moura, Regina [UNESP] |
author_facet |
Moura, Regina [UNESP] Sobreira, Marcone Lima [UNESP] Bertanha, Matheus [UNESP] Jaldin, Rodrigo Gibin [UNESP] Silva, Maria Madalena [UNESP] Pereira, Lied [UNESP] Takegawa, Bonifacio Katsunori [UNESP] Yoshida, Winston Bonetti [UNESP] |
author_role |
author |
author2 |
Sobreira, Marcone Lima [UNESP] Bertanha, Matheus [UNESP] Jaldin, Rodrigo Gibin [UNESP] Silva, Maria Madalena [UNESP] Pereira, Lied [UNESP] Takegawa, Bonifacio Katsunori [UNESP] Yoshida, Winston Bonetti [UNESP] |
author2_role |
author author author author author author author |
dc.contributor.none.fl_str_mv |
Universidade Estadual Paulista (Unesp) |
dc.contributor.author.fl_str_mv |
Moura, Regina [UNESP] Sobreira, Marcone Lima [UNESP] Bertanha, Matheus [UNESP] Jaldin, Rodrigo Gibin [UNESP] Silva, Maria Madalena [UNESP] Pereira, Lied [UNESP] Takegawa, Bonifacio Katsunori [UNESP] Yoshida, Winston Bonetti [UNESP] |
dc.subject.por.fl_str_mv |
Kasabach-Merritt syndrome Amputation Corticosteroids Hemangioma Síndrome de Kasabach-Merritt Amputação Corticoides Hemangioma |
topic |
Kasabach-Merritt syndrome Amputation Corticosteroids Hemangioma Síndrome de Kasabach-Merritt Amputação Corticoides Hemangioma |
description |
Kassabach-Merritt syndrome is a combination of capillary hemangioma and thrombocytopenia that predisposes to bleeding with petechiae, ecchymosis and spontaneous bruising. Treatment is generally started with corticosteroids, interferon alpha or chemotherapy. We present the case of a child (aged 1 year and 9 months) with a giant hemangioma, from the root of the thigh to the knee, and thrombocytopenia. Treatment was started with corticosteroids, without improvement, and then intra-tumor and cutaneous bleeding appeared spontaneously. The patient’s clinical condition precluded prescription of vincristine and interferon and emergency tumor resection was conducted because of extreme thrombocytopenia and bleeding. The child then began to develop sepsis with hypotension and ischemia of remnant tissues. This case presented a therapeutic challenge, which is the subject of this article. |
publishDate |
2014 |
dc.date.none.fl_str_mv |
2014 2016-04-01T18:42:43Z 2016-04-01T18:42:43Z |
dc.type.status.fl_str_mv |
info:eu-repo/semantics/publishedVersion |
dc.type.driver.fl_str_mv |
info:eu-repo/semantics/article |
format |
article |
status_str |
publishedVersion |
dc.identifier.uri.fl_str_mv |
http://dx.doi.org/10.1590/1677-5449.0102 Jornal Vascular Brasileiro, v. 13, n. 4, p. 330-335, 2014. 1677-5449 http://hdl.handle.net/11449/136816 10.1590/1677-5449.0102 S1677-54492014000400330 S1677-54492014000400330-en.pdf S1677-54492014000400330-pt.pdf 3613835231654932 3086448481970671 3086448481970671 3613835231654932 3613835231654932 3086448481970671 9609324832591382 4513014379461383 |
url |
http://dx.doi.org/10.1590/1677-5449.0102 http://hdl.handle.net/11449/136816 |
identifier_str_mv |
Jornal Vascular Brasileiro, v. 13, n. 4, p. 330-335, 2014. 1677-5449 10.1590/1677-5449.0102 S1677-54492014000400330 S1677-54492014000400330-en.pdf S1677-54492014000400330-pt.pdf 3613835231654932 3086448481970671 9609324832591382 4513014379461383 |
dc.language.iso.fl_str_mv |
eng |
language |
eng |
dc.relation.none.fl_str_mv |
Jornal Vascular Brasileiro 0,136 |
dc.rights.driver.fl_str_mv |
info:eu-repo/semantics/openAccess |
eu_rights_str_mv |
openAccess |
dc.format.none.fl_str_mv |
330-335 application/pdf application/pdf |
dc.source.none.fl_str_mv |
Currículo Lattes reponame:Repositório Institucional da UNESP instname:Universidade Estadual Paulista (UNESP) instacron:UNESP |
instname_str |
Universidade Estadual Paulista (UNESP) |
instacron_str |
UNESP |
institution |
UNESP |
reponame_str |
Repositório Institucional da UNESP |
collection |
Repositório Institucional da UNESP |
repository.name.fl_str_mv |
Repositório Institucional da UNESP - Universidade Estadual Paulista (UNESP) |
repository.mail.fl_str_mv |
|
_version_ |
1797790179777314816 |