Extreme hypertriglyceridemia in an infant with hemophagocytic lymphohistiocytosis and hydroxycobalamin deficiency
Introduction. Hemophagocytic lymphohistiocytosis (HLH) is a severe hyperinflammatory condition characterized by fever, cytopenias, hepatosplenomegaly and hemophagocytosis. HLH may be primary or secondary to infection, autoimmune disease or malignancy. Hypertriglyceridemia is a common abn...
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doaj-ddddedd8889841c0abef373779a331912021-01-02T09:14:31ZengSerbian Medical SocietySrpski Arhiv za Celokupno Lekarstvo0370-81792406-08952015-01-0114311-1274474710.2298/SARH1512744D0370-81791512744DExtreme hypertriglyceridemia in an infant with hemophagocytic lymphohistiocytosis and hydroxycobalamin deficiencyDokmanović Lidija0Krstovski Nada1Lazić Jelena2Rodić Predrag3Milošević Goran4Janković Srđa5Janić Dragana6School of Medicine, Belgrade + University Children’s Hospital, BelgradeSchool of Medicine, Belgrade + University Children’s Hospital, BelgradeSchool of Medicine, Belgrade + University Children’s Hospital, BelgradeSchool of Medicine, Belgrade + University Children’s Hospital, BelgradeUniversity Children’s Hospital, BelgradeUniversity Children’s Hospital, BelgradeSchool of Medicine, Belgrade + University Children’s Hospital, BelgradeIntroduction. Hemophagocytic lymphohistiocytosis (HLH) is a severe hyperinflammatory condition characterized by fever, cytopenias, hepatosplenomegaly and hemophagocytosis. HLH may be primary or secondary to infection, autoimmune disease or malignancy. Hypertriglyceridemia is a common abnormality in HLH and one of the HLH-2004 diagnostic criteria. Case Outline. We present an infant with severe hypotonia and hypoproteinemic edema who also had extreme hypertriglyceridemia (21 mmol/l) and was diagnosed with HLH based on six of eight HLH- 2004 criteria (fever, hepatosplenomegaly, bicytopenia, hypertriglyceridemia with hypofibrinogenemia, sIL-2R > 2400 IU/ml, hemophagocytosis). The presence of IgM antibodies to Epstein-Barr virus and cytomegalovirus indicated a probable infectious trigger. The child was cured by the HLH-2004 protocol for secondary HLH (consisting of dexamethasone and cyclosporine). He was also found to have low serum hydroxycobalamin levels, promptly corrected upon hydroxycobalamin administration. Conclusion. The presented case history underlines the need to ascertain the presence or absence of each of the eight HLH-2004 criteria in any patient suspected to suffer from HLH. [Projekat Ministarstva nauke Republike Srbije, br. 41004]http://www.doiserbia.nb.rs/img/doi/0370-8179/2015/0370-81791512744D.pdfhemophagocytic lymphohistiocytosisinfanthypertriglyceridemiahydroxycobalam indeficiency |
collection |
DOAJ |
language |
English |
format |
Article |
sources |
DOAJ |
author |
Dokmanović Lidija Krstovski Nada Lazić Jelena Rodić Predrag Milošević Goran Janković Srđa Janić Dragana |
spellingShingle |
Dokmanović Lidija Krstovski Nada Lazić Jelena Rodić Predrag Milošević Goran Janković Srđa Janić Dragana Extreme hypertriglyceridemia in an infant with hemophagocytic lymphohistiocytosis and hydroxycobalamin deficiency Srpski Arhiv za Celokupno Lekarstvo hemophagocytic lymphohistiocytosis infant hypertriglyceridemia hydroxycobalam indeficiency |
author_facet |
Dokmanović Lidija Krstovski Nada Lazić Jelena Rodić Predrag Milošević Goran Janković Srđa Janić Dragana |
author_sort |
Dokmanović Lidija |
title |
Extreme hypertriglyceridemia in an infant with hemophagocytic lymphohistiocytosis and hydroxycobalamin deficiency |
title_short |
Extreme hypertriglyceridemia in an infant with hemophagocytic lymphohistiocytosis and hydroxycobalamin deficiency |
title_full |
Extreme hypertriglyceridemia in an infant with hemophagocytic lymphohistiocytosis and hydroxycobalamin deficiency |
title_fullStr |
Extreme hypertriglyceridemia in an infant with hemophagocytic lymphohistiocytosis and hydroxycobalamin deficiency |
title_full_unstemmed |
Extreme hypertriglyceridemia in an infant with hemophagocytic lymphohistiocytosis and hydroxycobalamin deficiency |
title_sort |
extreme hypertriglyceridemia in an infant with hemophagocytic lymphohistiocytosis and hydroxycobalamin deficiency |
publisher |
Serbian Medical Society |
series |
Srpski Arhiv za Celokupno Lekarstvo |
issn |
0370-8179 2406-0895 |
publishDate |
2015-01-01 |
description |
Introduction. Hemophagocytic lymphohistiocytosis (HLH) is a severe
hyperinflammatory condition characterized by fever, cytopenias,
hepatosplenomegaly and hemophagocytosis. HLH may be primary or secondary to
infection, autoimmune disease or malignancy. Hypertriglyceridemia is a common
abnormality in HLH and one of the HLH-2004 diagnostic criteria. Case Outline.
We present an infant with severe hypotonia and hypoproteinemic edema who also
had extreme hypertriglyceridemia (21 mmol/l) and was diagnosed with HLH based
on six of eight HLH- 2004 criteria (fever, hepatosplenomegaly, bicytopenia,
hypertriglyceridemia with hypofibrinogenemia, sIL-2R > 2400 IU/ml,
hemophagocytosis). The presence of IgM antibodies to Epstein-Barr virus and
cytomegalovirus indicated a probable infectious trigger. The child was cured
by the HLH-2004 protocol for secondary HLH (consisting of dexamethasone and
cyclosporine). He was also found to have low serum hydroxycobalamin levels,
promptly corrected upon hydroxycobalamin administration. Conclusion. The
presented case history underlines the need to ascertain the presence or
absence of each of the eight HLH-2004 criteria in any patient suspected to
suffer from HLH. [Projekat Ministarstva nauke Republike Srbije, br. 41004] |
topic |
hemophagocytic lymphohistiocytosis infant hypertriglyceridemia hydroxycobalam indeficiency |
url |
http://www.doiserbia.nb.rs/img/doi/0370-8179/2015/0370-81791512744D.pdf |
work_keys_str_mv |
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