Granulomatose linfomatóide × hanseníase

Detalhes bibliográficos
Autor(a) principal: ADAD, Sheila Jorge
Data de Publicação: 1989
Outros Autores: FLEURY, Raul Negrão, NUNES, Anísio
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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spelling 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
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