Secondary hemophagocytic syndrome after renal transplantation: two case-reports
Autor(a) principal: | |
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Data de Publicação: | 2020 |
Outros Autores: | , , , , , |
Tipo de documento: | Artigo |
Idioma: | eng |
Título da fonte: | Jornal Brasileiro de Nefrologia |
Texto Completo: | http://old.scielo.br/scielo.php?script=sci_arttext&pid=S0101-28002020000100118 |
Resumo: | ABSTRACT Hemophagocytic syndrome or hemophagocytic lymphohistiocytosis (HLH) is an infrequent and underdiagnosed condition caused by an overactive immune response, resulting in blood cells phagocytosis. After kidney transplantation (KTx), HLH is usually secondary (or reactive) to infectious and neoplastic processes and has a high mortality rate. No effective treatment is available for this condition. Usual procedures include detecting and treating the pathology triggering the immune system dysregulation, other than administration of intravenous human immunoglobulin (IVIG) and high doses of steroids, and plasmapheresis. The best protocol for maintenance immunosuppressive therapy is also unknown. This article presents two cases of post-KTx reactive HLH that underwent adjuvant IVIG treatment and obtained good clinical results. Despite the high morbidity and mortality associated with reactive HLH after KTx, the early and precise diagnosis and the administration of IVIG therapy along with the treatment of the triggering disease, was an effective strategy to control HLH. |
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Secondary hemophagocytic syndrome after renal transplantation: two case-reportsLymphohistiocytosis, HemophagocyticImmunoglobulinsKidney TransplantationABSTRACT Hemophagocytic syndrome or hemophagocytic lymphohistiocytosis (HLH) is an infrequent and underdiagnosed condition caused by an overactive immune response, resulting in blood cells phagocytosis. After kidney transplantation (KTx), HLH is usually secondary (or reactive) to infectious and neoplastic processes and has a high mortality rate. No effective treatment is available for this condition. Usual procedures include detecting and treating the pathology triggering the immune system dysregulation, other than administration of intravenous human immunoglobulin (IVIG) and high doses of steroids, and plasmapheresis. The best protocol for maintenance immunosuppressive therapy is also unknown. This article presents two cases of post-KTx reactive HLH that underwent adjuvant IVIG treatment and obtained good clinical results. Despite the high morbidity and mortality associated with reactive HLH after KTx, the early and precise diagnosis and the administration of IVIG therapy along with the treatment of the triggering disease, was an effective strategy to control HLH.Sociedade Brasileira de Nefrologia2020-03-01info:eu-repo/semantics/articleinfo:eu-repo/semantics/publishedVersiontext/htmlhttp://old.scielo.br/scielo.php?script=sci_arttext&pid=S0101-28002020000100118Brazilian Journal of Nephrology v.42 n.1 2020reponame:Jornal Brasileiro de Nefrologiainstname:Sociedade Brasileira de Nefrologia (SBN)instacron:SBN10.1590/2175-8239-jbn-2018-0246info:eu-repo/semantics/openAccessNarciso Júnior,JoséNeri,Beatriz de OliveiraDantas,Gilberto Loiola de AlencarSilveira,Lara de Holanda JucáSales,Maria Luiza de Mattos Brito OliveiraFreitas,Tainá Veras de SandesEsmeraldo,Ronaldo de Matoseng2020-04-17T00:00:00Zoai:scielo:S0101-28002020000100118Revistahttp://www.bjn.org.br/ONGhttps://old.scielo.br/oai/scielo-oai.php||jbn@sbn.org.br2175-82390101-2800opendoar:2020-04-17T00:00Jornal Brasileiro de Nefrologia - Sociedade Brasileira de Nefrologia (SBN)false |
dc.title.none.fl_str_mv |
Secondary hemophagocytic syndrome after renal transplantation: two case-reports |
title |
Secondary hemophagocytic syndrome after renal transplantation: two case-reports |
spellingShingle |
Secondary hemophagocytic syndrome after renal transplantation: two case-reports Narciso Júnior,José Lymphohistiocytosis, Hemophagocytic Immunoglobulins Kidney Transplantation |
title_short |
Secondary hemophagocytic syndrome after renal transplantation: two case-reports |
title_full |
Secondary hemophagocytic syndrome after renal transplantation: two case-reports |
title_fullStr |
Secondary hemophagocytic syndrome after renal transplantation: two case-reports |
title_full_unstemmed |
Secondary hemophagocytic syndrome after renal transplantation: two case-reports |
title_sort |
Secondary hemophagocytic syndrome after renal transplantation: two case-reports |
author |
Narciso Júnior,José |
author_facet |
Narciso Júnior,José Neri,Beatriz de Oliveira Dantas,Gilberto Loiola de Alencar Silveira,Lara de Holanda Jucá Sales,Maria Luiza de Mattos Brito Oliveira Freitas,Tainá Veras de Sandes Esmeraldo,Ronaldo de Matos |
author_role |
author |
author2 |
Neri,Beatriz de Oliveira Dantas,Gilberto Loiola de Alencar Silveira,Lara de Holanda Jucá Sales,Maria Luiza de Mattos Brito Oliveira Freitas,Tainá Veras de Sandes Esmeraldo,Ronaldo de Matos |
author2_role |
author author author author author author |
dc.contributor.author.fl_str_mv |
Narciso Júnior,José Neri,Beatriz de Oliveira Dantas,Gilberto Loiola de Alencar Silveira,Lara de Holanda Jucá Sales,Maria Luiza de Mattos Brito Oliveira Freitas,Tainá Veras de Sandes Esmeraldo,Ronaldo de Matos |
dc.subject.por.fl_str_mv |
Lymphohistiocytosis, Hemophagocytic Immunoglobulins Kidney Transplantation |
topic |
Lymphohistiocytosis, Hemophagocytic Immunoglobulins Kidney Transplantation |
description |
ABSTRACT Hemophagocytic syndrome or hemophagocytic lymphohistiocytosis (HLH) is an infrequent and underdiagnosed condition caused by an overactive immune response, resulting in blood cells phagocytosis. After kidney transplantation (KTx), HLH is usually secondary (or reactive) to infectious and neoplastic processes and has a high mortality rate. No effective treatment is available for this condition. Usual procedures include detecting and treating the pathology triggering the immune system dysregulation, other than administration of intravenous human immunoglobulin (IVIG) and high doses of steroids, and plasmapheresis. The best protocol for maintenance immunosuppressive therapy is also unknown. This article presents two cases of post-KTx reactive HLH that underwent adjuvant IVIG treatment and obtained good clinical results. Despite the high morbidity and mortality associated with reactive HLH after KTx, the early and precise diagnosis and the administration of IVIG therapy along with the treatment of the triggering disease, was an effective strategy to control HLH. |
publishDate |
2020 |
dc.date.none.fl_str_mv |
2020-03-01 |
dc.type.driver.fl_str_mv |
info:eu-repo/semantics/article |
dc.type.status.fl_str_mv |
info:eu-repo/semantics/publishedVersion |
format |
article |
status_str |
publishedVersion |
dc.identifier.uri.fl_str_mv |
http://old.scielo.br/scielo.php?script=sci_arttext&pid=S0101-28002020000100118 |
url |
http://old.scielo.br/scielo.php?script=sci_arttext&pid=S0101-28002020000100118 |
dc.language.iso.fl_str_mv |
eng |
language |
eng |
dc.relation.none.fl_str_mv |
10.1590/2175-8239-jbn-2018-0246 |
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 |
Sociedade Brasileira de Nefrologia |
publisher.none.fl_str_mv |
Sociedade Brasileira de Nefrologia |
dc.source.none.fl_str_mv |
Brazilian Journal of Nephrology v.42 n.1 2020 reponame:Jornal Brasileiro de Nefrologia instname:Sociedade Brasileira de Nefrologia (SBN) instacron:SBN |
instname_str |
Sociedade Brasileira de Nefrologia (SBN) |
instacron_str |
SBN |
institution |
SBN |
reponame_str |
Jornal Brasileiro de Nefrologia |
collection |
Jornal Brasileiro de Nefrologia |
repository.name.fl_str_mv |
Jornal Brasileiro de Nefrologia - Sociedade Brasileira de Nefrologia (SBN) |
repository.mail.fl_str_mv |
||jbn@sbn.org.br |
_version_ |
1752122065733812224 |