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Pulmonary hemorrhage in pediatric lupus anticoagulant hypoprothrombinemia syndrome

Lupus anticoagulant-hypoprothrombinemia syndrome (LAHPS), a very rare disease that is caused by the presence of antifactor II antibodies, is usually counterbalanced by the prothrombotic effect of lupus anticoagulant (LAC). Patients with LAHPS are treated using fresh frozen plasma, steroids, immunosu...

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Detalles Bibliográficos
Autores principales: Kim, Ji Soo, Kim, Min Jae, Bae, E Young, Jeong, Dae Chul
Formato: Online Artículo Texto
Lenguaje:English
Publicado: The Korean Pediatric Society 2014
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4030123/
https://www.ncbi.nlm.nih.gov/pubmed/24868219
http://dx.doi.org/10.3345/kjp.2014.57.4.202
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author Kim, Ji Soo
Kim, Min Jae
Bae, E Young
Jeong, Dae Chul
author_facet Kim, Ji Soo
Kim, Min Jae
Bae, E Young
Jeong, Dae Chul
author_sort Kim, Ji Soo
collection PubMed
description Lupus anticoagulant-hypoprothrombinemia syndrome (LAHPS), a very rare disease that is caused by the presence of antifactor II antibodies, is usually counterbalanced by the prothrombotic effect of lupus anticoagulant (LAC). Patients with LAHPS are treated using fresh frozen plasma, steroids, immunosuppressive agents, and immunoglobulins for managing the disease and controlling hemorrhages. Notably, steroids are the important treatment for treating hypoprothrombinemia and controlling the bleeding. However, some patients suffer from severe, life-threatening hemorrhages, when factor II levels remain very low in spite of treatment with steroids. Here, we report a case of LAHPS in a 15-year-old girl who experienced pulmonary hemorrhage with rapid progression. She was referred to our hospital owing to easy bruising and prolonged bleeding. She was diagnosed with LAHPS that presented with pancytopenia, positive antinuclear antibody, proloned prothrombin time, activated partial thromboplastin time, positive LAC antibody, and factor II deficiency. Her treatment included massive blood transfusion, high-dose methylprednisolone, vitamin K, and immunoglobulin. However, she died due to uncontrolled pulmonary hemorrhage.
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spelling pubmed-40301232014-05-27 Pulmonary hemorrhage in pediatric lupus anticoagulant hypoprothrombinemia syndrome Kim, Ji Soo Kim, Min Jae Bae, E Young Jeong, Dae Chul Korean J Pediatr Case Report Lupus anticoagulant-hypoprothrombinemia syndrome (LAHPS), a very rare disease that is caused by the presence of antifactor II antibodies, is usually counterbalanced by the prothrombotic effect of lupus anticoagulant (LAC). Patients with LAHPS are treated using fresh frozen plasma, steroids, immunosuppressive agents, and immunoglobulins for managing the disease and controlling hemorrhages. Notably, steroids are the important treatment for treating hypoprothrombinemia and controlling the bleeding. However, some patients suffer from severe, life-threatening hemorrhages, when factor II levels remain very low in spite of treatment with steroids. Here, we report a case of LAHPS in a 15-year-old girl who experienced pulmonary hemorrhage with rapid progression. She was referred to our hospital owing to easy bruising and prolonged bleeding. She was diagnosed with LAHPS that presented with pancytopenia, positive antinuclear antibody, proloned prothrombin time, activated partial thromboplastin time, positive LAC antibody, and factor II deficiency. Her treatment included massive blood transfusion, high-dose methylprednisolone, vitamin K, and immunoglobulin. However, she died due to uncontrolled pulmonary hemorrhage. The Korean Pediatric Society 2014-04 2014-04-30 /pmc/articles/PMC4030123/ /pubmed/24868219 http://dx.doi.org/10.3345/kjp.2014.57.4.202 Text en Copyright © 2014 by The Korean Pediatric Society http://creativecommons.org/licenses/by-nc/3.0/ This is an open-access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/3.0/) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.
spellingShingle Case Report
Kim, Ji Soo
Kim, Min Jae
Bae, E Young
Jeong, Dae Chul
Pulmonary hemorrhage in pediatric lupus anticoagulant hypoprothrombinemia syndrome
title Pulmonary hemorrhage in pediatric lupus anticoagulant hypoprothrombinemia syndrome
title_full Pulmonary hemorrhage in pediatric lupus anticoagulant hypoprothrombinemia syndrome
title_fullStr Pulmonary hemorrhage in pediatric lupus anticoagulant hypoprothrombinemia syndrome
title_full_unstemmed Pulmonary hemorrhage in pediatric lupus anticoagulant hypoprothrombinemia syndrome
title_short Pulmonary hemorrhage in pediatric lupus anticoagulant hypoprothrombinemia syndrome
title_sort pulmonary hemorrhage in pediatric lupus anticoagulant hypoprothrombinemia syndrome
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4030123/
https://www.ncbi.nlm.nih.gov/pubmed/24868219
http://dx.doi.org/10.3345/kjp.2014.57.4.202
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