Systemic mastocytosis - a diagnostic challenge
Autor(a) principal: | |
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Data de Publicação: | 2014 |
Outros Autores: | , , |
Tipo de documento: | Relatório |
Idioma: | eng |
Título da fonte: | Revista brasileira de hematologia e hemoterapia (Online) |
Texto Completo: | http://old.scielo.br/scielo.php?script=sci_arttext&pid=S1516-84842014000300226 |
Resumo: | Mastocytosis refers to a group of disorders characterized by the infiltration of clonally derived mast cells to the skin or extracutaneous tissues resulting in a heterogeneous clinical picture. It is a rare hematologic disorder in all its forms. The exact incidence is unknown; it affects patients of any age and males and females equally. Its molecular pathogenesis is incompletely understood. The clinical features of mastocytosis result from both chronic and episodic mast cell mediator release, signs and symptoms arising from diffuse or focal tissue infiltration, and, occasionally, the presence of an associated non-mast cell clonal hematologic disease. The histopathologic analysis is essential for definitive diagnosis but there is no curative treatment. The authors report a clinical case of a 72-year-old woman with no history of allergies, with bicytopenia, weight loss, and diffuse axial osteolytic lesions. This is a rare clinical case of aggressive systemic mastocytosis for which palliative treatment can improve survival and quality of life. A brief review of the literature about this pathology is also included. |
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Revista brasileira de hematologia e hemoterapia (Online) |
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Systemic mastocytosis - a diagnostic challengeBiopsyAllergy and immunologyBone marrow cellsMastocytosis refers to a group of disorders characterized by the infiltration of clonally derived mast cells to the skin or extracutaneous tissues resulting in a heterogeneous clinical picture. It is a rare hematologic disorder in all its forms. The exact incidence is unknown; it affects patients of any age and males and females equally. Its molecular pathogenesis is incompletely understood. The clinical features of mastocytosis result from both chronic and episodic mast cell mediator release, signs and symptoms arising from diffuse or focal tissue infiltration, and, occasionally, the presence of an associated non-mast cell clonal hematologic disease. The histopathologic analysis is essential for definitive diagnosis but there is no curative treatment. The authors report a clinical case of a 72-year-old woman with no history of allergies, with bicytopenia, weight loss, and diffuse axial osteolytic lesions. This is a rare clinical case of aggressive systemic mastocytosis for which palliative treatment can improve survival and quality of life. A brief review of the literature about this pathology is also included.Associação Brasileira de Hematologia e Hemoterapia e Terapia Celular2014-06-01info:eu-repo/semantics/reportinfo:eu-repo/semantics/publishedVersiontext/htmlhttp://old.scielo.br/scielo.php?script=sci_arttext&pid=S1516-84842014000300226Revista Brasileira de Hematologia e Hemoterapia v.36 n.3 2014reponame:Revista brasileira de hematologia e hemoterapia (Online)instname:Associação Brasileira de Hematologia e Hemoterapia e Terapia Celular (ABHHTC)instacron:ABHHTC10.1016/j.bjhh.2014.03.003info:eu-repo/semantics/openAccessLladó,Ana Cristina Amorim Oliveira GaiaMihon,Claudia ElenaSilva,MadalenaGalzerano,Antonioeng2015-09-22T00:00:00Zoai:scielo:S1516-84842014000300226Revistahttp://www.rbhh.org/pt/archivo/https://old.scielo.br/oai/scielo-oai.phpsbhh@terra.com.br||secretaria@rbhh.org1806-08701516-8484opendoar:2015-09-22T00:00Revista brasileira de hematologia e hemoterapia (Online) - Associação Brasileira de Hematologia e Hemoterapia e Terapia Celular (ABHHTC)false |
dc.title.none.fl_str_mv |
Systemic mastocytosis - a diagnostic challenge |
title |
Systemic mastocytosis - a diagnostic challenge |
spellingShingle |
Systemic mastocytosis - a diagnostic challenge Lladó,Ana Cristina Amorim Oliveira Gaia Biopsy Allergy and immunology Bone marrow cells |
title_short |
Systemic mastocytosis - a diagnostic challenge |
title_full |
Systemic mastocytosis - a diagnostic challenge |
title_fullStr |
Systemic mastocytosis - a diagnostic challenge |
title_full_unstemmed |
Systemic mastocytosis - a diagnostic challenge |
title_sort |
Systemic mastocytosis - a diagnostic challenge |
author |
Lladó,Ana Cristina Amorim Oliveira Gaia |
author_facet |
Lladó,Ana Cristina Amorim Oliveira Gaia Mihon,Claudia Elena Silva,Madalena Galzerano,Antonio |
author_role |
author |
author2 |
Mihon,Claudia Elena Silva,Madalena Galzerano,Antonio |
author2_role |
author author author |
dc.contributor.author.fl_str_mv |
Lladó,Ana Cristina Amorim Oliveira Gaia Mihon,Claudia Elena Silva,Madalena Galzerano,Antonio |
dc.subject.por.fl_str_mv |
Biopsy Allergy and immunology Bone marrow cells |
topic |
Biopsy Allergy and immunology Bone marrow cells |
description |
Mastocytosis refers to a group of disorders characterized by the infiltration of clonally derived mast cells to the skin or extracutaneous tissues resulting in a heterogeneous clinical picture. It is a rare hematologic disorder in all its forms. The exact incidence is unknown; it affects patients of any age and males and females equally. Its molecular pathogenesis is incompletely understood. The clinical features of mastocytosis result from both chronic and episodic mast cell mediator release, signs and symptoms arising from diffuse or focal tissue infiltration, and, occasionally, the presence of an associated non-mast cell clonal hematologic disease. The histopathologic analysis is essential for definitive diagnosis but there is no curative treatment. The authors report a clinical case of a 72-year-old woman with no history of allergies, with bicytopenia, weight loss, and diffuse axial osteolytic lesions. This is a rare clinical case of aggressive systemic mastocytosis for which palliative treatment can improve survival and quality of life. A brief review of the literature about this pathology is also included. |
publishDate |
2014 |
dc.date.none.fl_str_mv |
2014-06-01 |
dc.type.driver.fl_str_mv |
info:eu-repo/semantics/report |
dc.type.status.fl_str_mv |
info:eu-repo/semantics/publishedVersion |
format |
report |
status_str |
publishedVersion |
dc.identifier.uri.fl_str_mv |
http://old.scielo.br/scielo.php?script=sci_arttext&pid=S1516-84842014000300226 |
url |
http://old.scielo.br/scielo.php?script=sci_arttext&pid=S1516-84842014000300226 |
dc.language.iso.fl_str_mv |
eng |
language |
eng |
dc.relation.none.fl_str_mv |
10.1016/j.bjhh.2014.03.003 |
dc.rights.driver.fl_str_mv |
info:eu-repo/semantics/openAccess |
eu_rights_str_mv |
openAccess |
dc.format.none.fl_str_mv |
text/html |
dc.publisher.none.fl_str_mv |
Associação Brasileira de Hematologia e Hemoterapia e Terapia Celular |
publisher.none.fl_str_mv |
Associação Brasileira de Hematologia e Hemoterapia e Terapia Celular |
dc.source.none.fl_str_mv |
Revista Brasileira de Hematologia e Hemoterapia v.36 n.3 2014 reponame:Revista brasileira de hematologia e hemoterapia (Online) instname:Associação Brasileira de Hematologia e Hemoterapia e Terapia Celular (ABHHTC) instacron:ABHHTC |
instname_str |
Associação Brasileira de Hematologia e Hemoterapia e Terapia Celular (ABHHTC) |
instacron_str |
ABHHTC |
institution |
ABHHTC |
reponame_str |
Revista brasileira de hematologia e hemoterapia (Online) |
collection |
Revista brasileira de hematologia e hemoterapia (Online) |
repository.name.fl_str_mv |
Revista brasileira de hematologia e hemoterapia (Online) - Associação Brasileira de Hematologia e Hemoterapia e Terapia Celular (ABHHTC) |
repository.mail.fl_str_mv |
sbhh@terra.com.br||secretaria@rbhh.org |
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1754213112347623424 |