Hemophagocytic lymphohistiocytosis, a rare condition in renal transplant - a case report
Autor(a) principal: | |
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Data de Publicação: | 2018 |
Outros Autores: | , , , , , , |
Tipo de documento: | Relatório |
Idioma: | eng |
Título da fonte: | Jornal Brasileiro de Nefrologia |
Texto Completo: | http://old.scielo.br/scielo.php?script=sci_arttext&pid=S0101-28002018000400423 |
Resumo: | Abstract Hemophagocytic lymphohistiocytosis (HLH) is an uncommon and life-threating condition characterized by major immune activation and massive cytokine production by mononuclear inflammatory cells, due to defects in cytotoxic lymphocyte function. It is even more unusual in renal transplant recipients, in which it is often associated with uncontrolled infection. The mortality is high in HLH and differential diagnosis with sepsis is a challenge. The approach and management depend on the underlying trigger and comorbidities. We report a case of a 50-year-old renal transplant female admitted with fever and malaise 3 months post-transplant and presenting anemia, fever, hypertriglyceridemia, high levels of serum ferritin, and positive CMV antigenemia. Urine was positive for decoy cells and BKV-DNA. Graft biopsy showed CMV nephritis. Both blood and urine cultures where positive for E. coli. Hemophagocytosis was confirmed by bone marrow aspiration. Immunosuppression was reduced, and the patient received high-dose intravenous immunoglobulin and dexamethasone, with complete response after 3 weeks. We highlight the importance of early diagnosis and proper management of a rare and serious condition in a renal transplant patient, which can allow a favorable clinical course and improve survival rate. |
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Hemophagocytic lymphohistiocytosis, a rare condition in renal transplant - a case reportKidney TransplantationLymphohistiocytosisHemophagocyticInfectionAbstract Hemophagocytic lymphohistiocytosis (HLH) is an uncommon and life-threating condition characterized by major immune activation and massive cytokine production by mononuclear inflammatory cells, due to defects in cytotoxic lymphocyte function. It is even more unusual in renal transplant recipients, in which it is often associated with uncontrolled infection. The mortality is high in HLH and differential diagnosis with sepsis is a challenge. The approach and management depend on the underlying trigger and comorbidities. We report a case of a 50-year-old renal transplant female admitted with fever and malaise 3 months post-transplant and presenting anemia, fever, hypertriglyceridemia, high levels of serum ferritin, and positive CMV antigenemia. Urine was positive for decoy cells and BKV-DNA. Graft biopsy showed CMV nephritis. Both blood and urine cultures where positive for E. coli. Hemophagocytosis was confirmed by bone marrow aspiration. Immunosuppression was reduced, and the patient received high-dose intravenous immunoglobulin and dexamethasone, with complete response after 3 weeks. We highlight the importance of early diagnosis and proper management of a rare and serious condition in a renal transplant patient, which can allow a favorable clinical course and improve survival rate.Sociedade Brasileira de Nefrologia2018-12-01info:eu-repo/semantics/reportinfo:eu-repo/semantics/publishedVersiontext/htmlhttp://old.scielo.br/scielo.php?script=sci_arttext&pid=S0101-28002018000400423Brazilian Journal of Nephrology v.40 n.4 2018reponame:Jornal Brasileiro de Nefrologiainstname:Sociedade Brasileira de Nefrologia (SBN)instacron:SBN10.1590/2175-8239-jbn-2018-0012info:eu-repo/semantics/openAccessLima,Valentine de A. C. de CastroGouvêa,Ana Luisa FigueiraMenezes,PauloSantos,Jacqueline da F.Rochael,Mayra CarrijoCarvalho,Fabiana RabeAlmeida,Jorge ReisLugon,Jocemir Ronaldoeng2019-02-05T00:00:00Zoai:scielo:S0101-28002018000400423Revistahttp://www.bjn.org.br/ONGhttps://old.scielo.br/oai/scielo-oai.php||jbn@sbn.org.br2175-82390101-2800opendoar:2019-02-05T00:00Jornal Brasileiro de Nefrologia - Sociedade Brasileira de Nefrologia (SBN)false |
dc.title.none.fl_str_mv |
Hemophagocytic lymphohistiocytosis, a rare condition in renal transplant - a case report |
title |
Hemophagocytic lymphohistiocytosis, a rare condition in renal transplant - a case report |
spellingShingle |
Hemophagocytic lymphohistiocytosis, a rare condition in renal transplant - a case report Lima,Valentine de A. C. de Castro Kidney Transplantation Lymphohistiocytosis Hemophagocytic Infection |
title_short |
Hemophagocytic lymphohistiocytosis, a rare condition in renal transplant - a case report |
title_full |
Hemophagocytic lymphohistiocytosis, a rare condition in renal transplant - a case report |
title_fullStr |
Hemophagocytic lymphohistiocytosis, a rare condition in renal transplant - a case report |
title_full_unstemmed |
Hemophagocytic lymphohistiocytosis, a rare condition in renal transplant - a case report |
title_sort |
Hemophagocytic lymphohistiocytosis, a rare condition in renal transplant - a case report |
author |
Lima,Valentine de A. C. de Castro |
author_facet |
Lima,Valentine de A. C. de Castro Gouvêa,Ana Luisa Figueira Menezes,Paulo Santos,Jacqueline da F. Rochael,Mayra Carrijo Carvalho,Fabiana Rabe Almeida,Jorge Reis Lugon,Jocemir Ronaldo |
author_role |
author |
author2 |
Gouvêa,Ana Luisa Figueira Menezes,Paulo Santos,Jacqueline da F. Rochael,Mayra Carrijo Carvalho,Fabiana Rabe Almeida,Jorge Reis Lugon,Jocemir Ronaldo |
author2_role |
author author author author author author author |
dc.contributor.author.fl_str_mv |
Lima,Valentine de A. C. de Castro Gouvêa,Ana Luisa Figueira Menezes,Paulo Santos,Jacqueline da F. Rochael,Mayra Carrijo Carvalho,Fabiana Rabe Almeida,Jorge Reis Lugon,Jocemir Ronaldo |
dc.subject.por.fl_str_mv |
Kidney Transplantation Lymphohistiocytosis Hemophagocytic Infection |
topic |
Kidney Transplantation Lymphohistiocytosis Hemophagocytic Infection |
description |
Abstract Hemophagocytic lymphohistiocytosis (HLH) is an uncommon and life-threating condition characterized by major immune activation and massive cytokine production by mononuclear inflammatory cells, due to defects in cytotoxic lymphocyte function. It is even more unusual in renal transplant recipients, in which it is often associated with uncontrolled infection. The mortality is high in HLH and differential diagnosis with sepsis is a challenge. The approach and management depend on the underlying trigger and comorbidities. We report a case of a 50-year-old renal transplant female admitted with fever and malaise 3 months post-transplant and presenting anemia, fever, hypertriglyceridemia, high levels of serum ferritin, and positive CMV antigenemia. Urine was positive for decoy cells and BKV-DNA. Graft biopsy showed CMV nephritis. Both blood and urine cultures where positive for E. coli. Hemophagocytosis was confirmed by bone marrow aspiration. Immunosuppression was reduced, and the patient received high-dose intravenous immunoglobulin and dexamethasone, with complete response after 3 weeks. We highlight the importance of early diagnosis and proper management of a rare and serious condition in a renal transplant patient, which can allow a favorable clinical course and improve survival rate. |
publishDate |
2018 |
dc.date.none.fl_str_mv |
2018-12-01 |
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=S0101-28002018000400423 |
url |
http://old.scielo.br/scielo.php?script=sci_arttext&pid=S0101-28002018000400423 |
dc.language.iso.fl_str_mv |
eng |
language |
eng |
dc.relation.none.fl_str_mv |
10.1590/2175-8239-jbn-2018-0012 |
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.40 n.4 2018 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 |
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1752122065117249536 |