Granulomatose linfomatóide × hanseníase
Autor(a) principal: | |
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Data de Publicação: | 1989 |
Outros Autores: | , |
Tipo de documento: | Artigo |
Idioma: | por |
Título da fonte: | Hansenologia Internationalis (Online) |
Texto Completo: | https://periodicos.saude.sp.gov.br/hansenologia/article/view/35551 |
Resumo: | Lymphomatoid granulomatosis íLYG) was first described by LIEBOW, A.A. et a19. Histologically Is characterized by an lymphohistiocytic infiltrate with granulomatous features, polimorphous and plelomorphic, anglocentric and angio destructive and mainly involves lungs, skin, kidneys and CNS. LYG does not involves spleen, bone marrow and lymphnodes. Presently there is a concept that LYG is an angiocentric variant of T-cell peripheral Iimphoma and histologically indistinguishable from poiymorfic reticulosis of the upper airways ímidline lethal granuloma). The prognosis for patients with LYG is guarded. Treatment with cyclophosphamide and prednisone may lead to remission in early cases. The skin is the most commonly Involved extrapuimonary organ and in 13 to 34% of patients the skin lesions precede the pulmonary involvement. The clinical features of the skin lesions may vary, but frequently they are erythematous and violaceous plaque lesions or annular Infiltrated lesions with central clearing. The differential diagnosis of these lesions includes granuloma anular, sarcoidosis and Hansen's disease. Since Hansen's disease is common among us and that LYG includes involvement of cutaneous branches and nerve trunks, with hypo or hyperesthesia in skin lesions and paresthesia of limbs, it is of utmost importance to make differential diagnosis. This report deals with a 42 years old male with cutaneous lesions of LYG and concomitant pulmonary and systemic manifestations. A first skin biopsy roughly suggested tuberculoid leprosy due to a granulomatous and perinhural localization of cellular Infiltrate. The patient. died on respiratory insufficiency and the necropsy findings of the skin revealed important histological modifications. The infiltrate was more polymorphous, plelomorphic and angiocentric. The same histological features were found in CNS, heart, digestive tract, liver, prostate, testes, lungs and kidneys. In these two last organs there were large nodules made of the characteristic cellular Infiltrate and also large necrotic areas. |
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Granulomatose linfomatóide × hanseníaseLymphomatoid granulomatosis íLYG) was first described by LIEBOW, A.A. et a19. Histologically Is characterized by an lymphohistiocytic infiltrate with granulomatous features, polimorphous and plelomorphic, anglocentric and angio destructive and mainly involves lungs, skin, kidneys and CNS. LYG does not involves spleen, bone marrow and lymphnodes. Presently there is a concept that LYG is an angiocentric variant of T-cell peripheral Iimphoma and histologically indistinguishable from poiymorfic reticulosis of the upper airways ímidline lethal granuloma). The prognosis for patients with LYG is guarded. Treatment with cyclophosphamide and prednisone may lead to remission in early cases. The skin is the most commonly Involved extrapuimonary organ and in 13 to 34% of patients the skin lesions precede the pulmonary involvement. The clinical features of the skin lesions may vary, but frequently they are erythematous and violaceous plaque lesions or annular Infiltrated lesions with central clearing. The differential diagnosis of these lesions includes granuloma anular, sarcoidosis and Hansen's disease. Since Hansen's disease is common among us and that LYG includes involvement of cutaneous branches and nerve trunks, with hypo or hyperesthesia in skin lesions and paresthesia of limbs, it is of utmost importance to make differential diagnosis. This report deals with a 42 years old male with cutaneous lesions of LYG and concomitant pulmonary and systemic manifestations. A first skin biopsy roughly suggested tuberculoid leprosy due to a granulomatous and perinhural localization of cellular Infiltrate. The patient. died on respiratory insufficiency and the necropsy findings of the skin revealed important histological modifications. The infiltrate was more polymorphous, plelomorphic and angiocentric. The same histological features were found in CNS, heart, digestive tract, liver, prostate, testes, lungs and kidneys. In these two last organs there were large nodules made of the characteristic cellular Infiltrate and also large necrotic areas.A granulomatose linfomatóide é uma entidade nosológica primeiramente descrita por A.A. Liebow, C.R.B. Carrington e P. Friedman9 em 1972, e caracterizada por processo linfoproliferativo e granulomatoso, polimorfo e plelomorfo, angiocêntrico e angiodestrutivo, envolvendo predominantemente pulmões, mas sendo uma doença sistêmica que também compromete, em ordem de freqüência, pele, rins, sistema nervoso central e outras localizações orgânicas poupando, em geral, baço, lifonodos e medula óssea. Atualmente é considerada uma forma peculiar de linfoma T periférico com caráter angiocêntrico, sendo indistinguível histologicamente da reticulose polimorfa (granuloma letal de linha média). Trata-se de uma afecção grave, de mau prognóstico, mas passível de boa resposta terapêutica (corticóide e ciclofosfamida) principalmente nas fases iniciais. A pele é a segunda localização orgânica mais afetada e em 13 a 34% dos casos as lesões cutâneas precedem o comprometimento pulmonar. Há uma grande variedade de aspectos destas lesões, mas frequentemente são placas eritêmato-violáceas, ou lesões anulares infiltradas com clareamento central. 0 diagnóstico diferencial destas lesões inclue principalmente granuloma anular, sarcoidose e hanseníase. Como esta última afecção é muito frequente entre nós e como na granulomatose linfomatóide há envolvimento de ramos nervosos cutâneos e troncos nervosos periféricos, com sintomas de hipo ou hiperestesia nas lesões e parestesias nos membros, é sempre necessário realizar-se o diagnóstico diferencial. Esta apresentação anátomo-clínica se refere a um homem de 42 anos, com manifestações cutâneas de granulomatose linfomatóide que surgiram concomitantemente com manifestações pulmonares e sistêmicas. Uma biópsia cutânea foi altamente sugestiva de hanseníase tuberculóide devido ao caráter granulomatoso do infiltrado e ao envolvimento e penetração de ramos nervosos. O paciente faleceu em insuficiência respiratória e os achados necroscópicos na pele demonstram importantes modificações histológicas, com infiltrado mais polimorfo, plelomórfico e angiocêntrico. As mesmas alterações histológicas foram encontradas no CNS, fígado, coração, parede gástrica, próstata, testículos, pulmões e rins. Nestes últimos órgãos haviam grandes nódulos constituidos pelo Infiltrado característico e extensas áreas de necrose.Instituto Lauro de Souza Lima da Secretaria de Estado da Saúde de São Paulo1989-06-30info:eu-repo/semantics/articleinfo:eu-repo/semantics/publishedVersionAvaliado pelos paresapplication/pdfhttps://periodicos.saude.sp.gov.br/hansenologia/article/view/3555110.47878/hi.1989.v14.35551Hansenologia Internationalis: leprosy and other infectious diseases; Vol. 14 No. 1 (1989); 32-41Hansenologia Internationalis: hanseníase e outras doenças infecciosas; v. 14 n. 1 (1989); 32-411982-5161reponame:Hansenologia Internationalis (Online)instname:Instituto Lauro de Souza Lima (ILSL)instacron:ILSLporhttps://periodicos.saude.sp.gov.br/hansenologia/article/view/35551/33957ADAD, Sheila JorgeFLEURY, Raul NegrãoNUNES, Anísioinfo:eu-repo/semantics/openAccess2021-06-06T02:29:07Zoai:ojs.periodicos.saude.sp.gov.br:article/35551Revistahttps://periodicos.saude.sp.gov.br/hansenologiaPRIhttps://periodicos.saude.sp.gov.br/hansenologia/oaihansen_int@ilsl.br || hansenologia.internationalis@gmail.com || periodicossp@saude.sp.gov.br1982-51610100-3283opendoar:2021-06-06T02:29:07Hansenologia Internationalis (Online) - Instituto Lauro de Souza Lima (ILSL)false |
dc.title.none.fl_str_mv |
Granulomatose linfomatóide × hanseníase |
title |
Granulomatose linfomatóide × hanseníase |
spellingShingle |
Granulomatose linfomatóide × hanseníase ADAD, Sheila Jorge |
title_short |
Granulomatose linfomatóide × hanseníase |
title_full |
Granulomatose linfomatóide × hanseníase |
title_fullStr |
Granulomatose linfomatóide × hanseníase |
title_full_unstemmed |
Granulomatose linfomatóide × hanseníase |
title_sort |
Granulomatose linfomatóide × hanseníase |
author |
ADAD, Sheila Jorge |
author_facet |
ADAD, Sheila Jorge FLEURY, Raul Negrão NUNES, Anísio |
author_role |
author |
author2 |
FLEURY, Raul Negrão NUNES, Anísio |
author2_role |
author author |
dc.contributor.author.fl_str_mv |
ADAD, Sheila Jorge FLEURY, Raul Negrão NUNES, Anísio |
description |
Lymphomatoid granulomatosis íLYG) was first described by LIEBOW, A.A. et a19. Histologically Is characterized by an lymphohistiocytic infiltrate with granulomatous features, polimorphous and plelomorphic, anglocentric and angio destructive and mainly involves lungs, skin, kidneys and CNS. LYG does not involves spleen, bone marrow and lymphnodes. Presently there is a concept that LYG is an angiocentric variant of T-cell peripheral Iimphoma and histologically indistinguishable from poiymorfic reticulosis of the upper airways ímidline lethal granuloma). The prognosis for patients with LYG is guarded. Treatment with cyclophosphamide and prednisone may lead to remission in early cases. The skin is the most commonly Involved extrapuimonary organ and in 13 to 34% of patients the skin lesions precede the pulmonary involvement. The clinical features of the skin lesions may vary, but frequently they are erythematous and violaceous plaque lesions or annular Infiltrated lesions with central clearing. The differential diagnosis of these lesions includes granuloma anular, sarcoidosis and Hansen's disease. Since Hansen's disease is common among us and that LYG includes involvement of cutaneous branches and nerve trunks, with hypo or hyperesthesia in skin lesions and paresthesia of limbs, it is of utmost importance to make differential diagnosis. This report deals with a 42 years old male with cutaneous lesions of LYG and concomitant pulmonary and systemic manifestations. A first skin biopsy roughly suggested tuberculoid leprosy due to a granulomatous and perinhural localization of cellular Infiltrate. The patient. died on respiratory insufficiency and the necropsy findings of the skin revealed important histological modifications. The infiltrate was more polymorphous, plelomorphic and angiocentric. The same histological features were found in CNS, heart, digestive tract, liver, prostate, testes, lungs and kidneys. In these two last organs there were large nodules made of the characteristic cellular Infiltrate and also large necrotic areas. |
publishDate |
1989 |
dc.date.none.fl_str_mv |
1989-06-30 |
dc.type.driver.fl_str_mv |
info:eu-repo/semantics/article info:eu-repo/semantics/publishedVersion Avaliado pelos pares |
format |
article |
status_str |
publishedVersion |
dc.identifier.uri.fl_str_mv |
https://periodicos.saude.sp.gov.br/hansenologia/article/view/35551 10.47878/hi.1989.v14.35551 |
url |
https://periodicos.saude.sp.gov.br/hansenologia/article/view/35551 |
identifier_str_mv |
10.47878/hi.1989.v14.35551 |
dc.language.iso.fl_str_mv |
por |
language |
por |
dc.relation.none.fl_str_mv |
https://periodicos.saude.sp.gov.br/hansenologia/article/view/35551/33957 |
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 |
Instituto Lauro de Souza Lima da Secretaria de Estado da Saúde de São Paulo |
publisher.none.fl_str_mv |
Instituto Lauro de Souza Lima da Secretaria de Estado da Saúde de São Paulo |
dc.source.none.fl_str_mv |
Hansenologia Internationalis: leprosy and other infectious diseases; Vol. 14 No. 1 (1989); 32-41 Hansenologia Internationalis: hanseníase e outras doenças infecciosas; v. 14 n. 1 (1989); 32-41 1982-5161 reponame:Hansenologia Internationalis (Online) instname:Instituto Lauro de Souza Lima (ILSL) instacron:ILSL |
instname_str |
Instituto Lauro de Souza Lima (ILSL) |
instacron_str |
ILSL |
institution |
ILSL |
reponame_str |
Hansenologia Internationalis (Online) |
collection |
Hansenologia Internationalis (Online) |
repository.name.fl_str_mv |
Hansenologia Internationalis (Online) - Instituto Lauro de Souza Lima (ILSL) |
repository.mail.fl_str_mv |
hansen_int@ilsl.br || hansenologia.internationalis@gmail.com || periodicossp@saude.sp.gov.br |
_version_ |
1796797579750539264 |