Systemic mastocytosis – a clinical case report
Autor(a) principal: | |
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Data de Publicação: | 2003 |
Outros Autores: | , , , , |
Tipo de documento: | Artigo |
Idioma: | por |
Título da fonte: | Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos) |
Texto Completo: | https://revista.spmi.pt/index.php/rpmi/article/view/1793 |
Resumo: | The A.A. describe a clinical case concerning an adult male, 51 years old, suffering from easytiredness, dry cough, nocturnal fever along with oedema and pigmented lesions on both legs, of 15 days duration. In his past history he had suffered an episode of histaminic shock in 1992.Clinical findings: slightly raised body temperature, paleness of the skin and mucosae, bilateral axillary and inguinal adenopathic conglomerates coexisting with painless and homogeneous hepatic and splenic enlargement. Bothlegs were covered with brown macular diffuse lesions, compatible with pigmented urticaria.Laboratory findings revealed the presence of macrocytic anaemia thrombocytopenia; hypergammaglobulinemia IgG; negative tumour markers; thoracic and abdominal computerized axial tomography showed “(...) slight bilateralpleural effusion, homogeneous hepatic andsplenic enlargement with the presence of enlarged lumbar and aortic lymph nodes”. Bone marrow biopsy result revealed “ a medullary invasion by fibroblastic proliferation nodes associated with a lymphocytic and mast cells infiltration, allowing the diagnosis of Systemic mastocytosis “. Cutaneous and hepatic biopsies also showed the involvement of these organs in the pathologic process.The patient was treated with methylprednisolone and cyclosporine A with remarkable reduction of symptoms. |
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Systemic mastocytosis – a clinical case reportMastocitose sistémica – a propósito de um caso clínicoanemiatrombocitopeniamastocitose sistémicaAnaemiaThrombocytopeniasystemic mastocytosisThe A.A. describe a clinical case concerning an adult male, 51 years old, suffering from easytiredness, dry cough, nocturnal fever along with oedema and pigmented lesions on both legs, of 15 days duration. In his past history he had suffered an episode of histaminic shock in 1992.Clinical findings: slightly raised body temperature, paleness of the skin and mucosae, bilateral axillary and inguinal adenopathic conglomerates coexisting with painless and homogeneous hepatic and splenic enlargement. Bothlegs were covered with brown macular diffuse lesions, compatible with pigmented urticaria.Laboratory findings revealed the presence of macrocytic anaemia thrombocytopenia; hypergammaglobulinemia IgG; negative tumour markers; thoracic and abdominal computerized axial tomography showed “(...) slight bilateralpleural effusion, homogeneous hepatic andsplenic enlargement with the presence of enlarged lumbar and aortic lymph nodes”. Bone marrow biopsy result revealed “ a medullary invasion by fibroblastic proliferation nodes associated with a lymphocytic and mast cells infiltration, allowing the diagnosis of Systemic mastocytosis “. Cutaneous and hepatic biopsies also showed the involvement of these organs in the pathologic process.The patient was treated with methylprednisolone and cyclosporine A with remarkable reduction of symptoms.Os A.A. descrevem o caso clínico de um indivíduo do sexo masculino, de 51 anos de idade,internado para estudo de um quadro clínicocom evolução de cerca de 15 dias, caracterizado por cansaço fácil, tosse não produtiva, edema e lesões tipo urticária pigmentosa em ambas as pernas, para além de hipertermia nocturna. Dos antecedentes pessoais, salienta-seum episódio de shock histamínico em 1992.Em relação ao exame objectivo, verificou-seacentuada palidez cutâneo-mucosa, temperatura subfebril, conglomerados adenopáticos nascadeias axilar e inguinal, bilateralmente, paraalém de hepatoesplenomegalia homogénea e indolor.Dos exames auxiliares de diagnóstico, destacamos: anemia macrocítica e trombocitopenia; hipergamaglobulinemia IgG; marcadores tumorais negativos; T.A.C. toracoabdominal:“(...) ligeiro derrame pleural bilateral, hepato-esplenomegalia homogénea e presença de adenopatias lombo-aórticas”; biópsia óssea: “envolvimento da medula óssea por nódulos de proliferação fibroblástica associada a infiltrado linfóide e de mastócitos, consistente com o diagnóstico de mastocitose sistémica. “. As biópsias cutânea e hepática demonstraram, também,envolvimento daqueles órgãos no processo. Efectuou terapêutica com metilprednisolonae ciclosporina A, obtendo-se melhoria clínica e analítica significativas.Sociedade Portuguesa de Medicina Interna2003-06-30info:eu-repo/semantics/publishedVersioninfo:eu-repo/semantics/articleapplication/pdfhttps://revista.spmi.pt/index.php/rpmi/article/view/1793Internal Medicine; Vol. 10 No. 2 (2003): Abril/ Junho; 59-62Medicina Interna; Vol. 10 N.º 2 (2003): Abril/ Junho; 59-622183-99800872-671Xreponame:Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos)instname:Agência para a Sociedade do Conhecimento (UMIC) - FCT - Sociedade da Informaçãoinstacron:RCAAPporhttps://revista.spmi.pt/index.php/rpmi/article/view/1793https://revista.spmi.pt/index.php/rpmi/article/view/1793/1246Melo, MarcoDeus, AdelaideAragão, AntónioMagalhães, EduardoSantos, FernandoSaldanha, Maria Helenainfo:eu-repo/semantics/openAccess2023-05-27T06:10:36Zoai:oai.revista.spmi.pt:article/1793Portal AgregadorONGhttps://www.rcaap.pt/oai/openaireopendoar:71602024-03-19T17:56:21.116046Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos) - Agência para a Sociedade do Conhecimento (UMIC) - FCT - Sociedade da Informaçãofalse |
dc.title.none.fl_str_mv |
Systemic mastocytosis – a clinical case report Mastocitose sistémica – a propósito de um caso clínico |
title |
Systemic mastocytosis – a clinical case report |
spellingShingle |
Systemic mastocytosis – a clinical case report Melo, Marco anemia trombocitopenia mastocitose sistémica Anaemia Thrombocytopenia systemic mastocytosis |
title_short |
Systemic mastocytosis – a clinical case report |
title_full |
Systemic mastocytosis – a clinical case report |
title_fullStr |
Systemic mastocytosis – a clinical case report |
title_full_unstemmed |
Systemic mastocytosis – a clinical case report |
title_sort |
Systemic mastocytosis – a clinical case report |
author |
Melo, Marco |
author_facet |
Melo, Marco Deus, Adelaide Aragão, António Magalhães, Eduardo Santos, Fernando Saldanha, Maria Helena |
author_role |
author |
author2 |
Deus, Adelaide Aragão, António Magalhães, Eduardo Santos, Fernando Saldanha, Maria Helena |
author2_role |
author author author author author |
dc.contributor.author.fl_str_mv |
Melo, Marco Deus, Adelaide Aragão, António Magalhães, Eduardo Santos, Fernando Saldanha, Maria Helena |
dc.subject.por.fl_str_mv |
anemia trombocitopenia mastocitose sistémica Anaemia Thrombocytopenia systemic mastocytosis |
topic |
anemia trombocitopenia mastocitose sistémica Anaemia Thrombocytopenia systemic mastocytosis |
description |
The A.A. describe a clinical case concerning an adult male, 51 years old, suffering from easytiredness, dry cough, nocturnal fever along with oedema and pigmented lesions on both legs, of 15 days duration. In his past history he had suffered an episode of histaminic shock in 1992.Clinical findings: slightly raised body temperature, paleness of the skin and mucosae, bilateral axillary and inguinal adenopathic conglomerates coexisting with painless and homogeneous hepatic and splenic enlargement. Bothlegs were covered with brown macular diffuse lesions, compatible with pigmented urticaria.Laboratory findings revealed the presence of macrocytic anaemia thrombocytopenia; hypergammaglobulinemia IgG; negative tumour markers; thoracic and abdominal computerized axial tomography showed “(...) slight bilateralpleural effusion, homogeneous hepatic andsplenic enlargement with the presence of enlarged lumbar and aortic lymph nodes”. Bone marrow biopsy result revealed “ a medullary invasion by fibroblastic proliferation nodes associated with a lymphocytic and mast cells infiltration, allowing the diagnosis of Systemic mastocytosis “. Cutaneous and hepatic biopsies also showed the involvement of these organs in the pathologic process.The patient was treated with methylprednisolone and cyclosporine A with remarkable reduction of symptoms. |
publishDate |
2003 |
dc.date.none.fl_str_mv |
2003-06-30 |
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 |
https://revista.spmi.pt/index.php/rpmi/article/view/1793 |
url |
https://revista.spmi.pt/index.php/rpmi/article/view/1793 |
dc.language.iso.fl_str_mv |
por |
language |
por |
dc.relation.none.fl_str_mv |
https://revista.spmi.pt/index.php/rpmi/article/view/1793 https://revista.spmi.pt/index.php/rpmi/article/view/1793/1246 |
dc.rights.driver.fl_str_mv |
info:eu-repo/semantics/openAccess |
eu_rights_str_mv |
openAccess |
dc.format.none.fl_str_mv |
application/pdf |
dc.publisher.none.fl_str_mv |
Sociedade Portuguesa de Medicina Interna |
publisher.none.fl_str_mv |
Sociedade Portuguesa de Medicina Interna |
dc.source.none.fl_str_mv |
Internal Medicine; Vol. 10 No. 2 (2003): Abril/ Junho; 59-62 Medicina Interna; Vol. 10 N.º 2 (2003): Abril/ Junho; 59-62 2183-9980 0872-671X reponame:Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos) instname:Agência para a Sociedade do Conhecimento (UMIC) - FCT - Sociedade da Informação instacron:RCAAP |
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Agência para a Sociedade do Conhecimento (UMIC) - FCT - Sociedade da Informação |
instacron_str |
RCAAP |
institution |
RCAAP |
reponame_str |
Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos) |
collection |
Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos) |
repository.name.fl_str_mv |
Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos) - Agência para a Sociedade do Conhecimento (UMIC) - FCT - Sociedade da Informação |
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1799131638850912256 |