Haemophagocytic lymphohistiocytosis (HLH) in adult with dengue infection

Haemophagocytic lymphohistiocytosis (HLH) or haemophagocytic syndrome is a rare but life-threatening syndrome of excessive immune activation with nonspecificclinical presentation. HLH is one of the complications in dengue infection. A 69-year-old lady was treated for severe dengue with multi-org...

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Main Authors: Wong, KCS, Ahmad Khaldun Ismail, Wan Mohd Shukri WNA, Cheah SK
Format: Article
Language:English
Published: Pusat Perubatan Universiti Kebangsaan Malaysia 2021
Online Access:http://journalarticle.ukm.my/18285/
http://journalarticle.ukm.my/18285/1/23_ms0417_pdf_12268.pdf
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author Wong, KCS
Ahmad Khaldun Ismail,
Wan Mohd Shukri WNA,
Cheah SK,
author_facet Wong, KCS
Ahmad Khaldun Ismail,
Wan Mohd Shukri WNA,
Cheah SK,
author_sort Wong, KCS
building UKM Institutional Repository
collection Online Access
description Haemophagocytic lymphohistiocytosis (HLH) or haemophagocytic syndrome is a rare but life-threatening syndrome of excessive immune activation with nonspecificclinical presentation. HLH is one of the complications in dengue infection. A 69-year-old lady was treated for severe dengue with multi-organ dysfunction with superimposed pneumonia, requiring mechanical ventilation. However, persistent cytopenia despite blood transfusion without evidence of haemorrhage raised the suspicion of HLH. Further blood investigations revealed hypertriglyceridaemia, hypofibrinogenaemia and hyperferritinaemia. Bone marrow aspiration showed haemophagocytosis. Patient fulfilled the diagnostic criteria for HLH by HLH-2004 trial. Her HScore is 281, with the probability of having HLH is 99.9%. Patient’s condition improved after administration of intravenous immunoglobulin (IVIG) and intravenous dexamethasone in tapering doses. Early specific treatment of HLH with IVIG and/or corticosteroid is important but diagnosis is usually delayed due to nonspecific clinical findings and laboratory results. High index of suspicion with the aid of diagnostic criteria by HLH-2004 trial and HScore is helpful to recognise this syndrome.
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spelling oai:generic.eprints.org:182852022-03-25T01:58:22Z http://journalarticle.ukm.my/18285/ Haemophagocytic lymphohistiocytosis (HLH) in adult with dengue infection Wong, KCS Ahmad Khaldun Ismail, Wan Mohd Shukri WNA, Cheah SK, Haemophagocytic lymphohistiocytosis (HLH) or haemophagocytic syndrome is a rare but life-threatening syndrome of excessive immune activation with nonspecificclinical presentation. HLH is one of the complications in dengue infection. A 69-year-old lady was treated for severe dengue with multi-organ dysfunction with superimposed pneumonia, requiring mechanical ventilation. However, persistent cytopenia despite blood transfusion without evidence of haemorrhage raised the suspicion of HLH. Further blood investigations revealed hypertriglyceridaemia, hypofibrinogenaemia and hyperferritinaemia. Bone marrow aspiration showed haemophagocytosis. Patient fulfilled the diagnostic criteria for HLH by HLH-2004 trial. Her HScore is 281, with the probability of having HLH is 99.9%. Patient’s condition improved after administration of intravenous immunoglobulin (IVIG) and intravenous dexamethasone in tapering doses. Early specific treatment of HLH with IVIG and/or corticosteroid is important but diagnosis is usually delayed due to nonspecific clinical findings and laboratory results. High index of suspicion with the aid of diagnostic criteria by HLH-2004 trial and HScore is helpful to recognise this syndrome. Pusat Perubatan Universiti Kebangsaan Malaysia 2021-12 Article PeerReviewed application/pdf en http://journalarticle.ukm.my/18285/1/23_ms0417_pdf_12268.pdf Wong, KCS and Ahmad Khaldun Ismail, and Wan Mohd Shukri WNA, and Cheah SK, (2021) Haemophagocytic lymphohistiocytosis (HLH) in adult with dengue infection. Medicine & Health, 16 (2). pp. 287-294. ISSN 2289-5728 https://medicineandhealthukm.com/toc/16/2
spellingShingle Wong, KCS
Ahmad Khaldun Ismail,
Wan Mohd Shukri WNA,
Cheah SK,
Haemophagocytic lymphohistiocytosis (HLH) in adult with dengue infection
title Haemophagocytic lymphohistiocytosis (HLH) in adult with dengue infection
title_full Haemophagocytic lymphohistiocytosis (HLH) in adult with dengue infection
title_fullStr Haemophagocytic lymphohistiocytosis (HLH) in adult with dengue infection
title_full_unstemmed Haemophagocytic lymphohistiocytosis (HLH) in adult with dengue infection
title_short Haemophagocytic lymphohistiocytosis (HLH) in adult with dengue infection
title_sort haemophagocytic lymphohistiocytosis (hlh) in adult with dengue infection
url http://journalarticle.ukm.my/18285/
http://journalarticle.ukm.my/18285/
http://journalarticle.ukm.my/18285/1/23_ms0417_pdf_12268.pdf