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Central nervous system (CNS) involvement is a critical prognostic factor for hemophagocytic lymphohistiocytosis
BACKGROUND: Hemophagocytic lymphohistiocytosis (HLH) is a rare multisystem disorder that frequently involves the central nervous system (CNS). We compared the clinical characteristics, treatment, and prognosis of patients with HLH according to the degree of CNS involvement. METHODS: The clinical man...
Autores principales: | , , , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Korean Society of Hematology; Korean Society of Blood and Marrow Transplantation; Korean Society of Pediatric Hematology-Oncology; Korean Society on Thrombosis and Hemostasis
2012
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3538799/ https://www.ncbi.nlm.nih.gov/pubmed/23320006 http://dx.doi.org/10.5045/kjh.2012.47.4.273 |
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author | Kim, Myung-Mi Yum, Mi-Sun Choi, Hae-Won Ko, Tae-Sung Im, Ho Joon Seo, Jong-Jin Koh, Kyung-Nam |
author_facet | Kim, Myung-Mi Yum, Mi-Sun Choi, Hae-Won Ko, Tae-Sung Im, Ho Joon Seo, Jong-Jin Koh, Kyung-Nam |
author_sort | Kim, Myung-Mi |
collection | PubMed |
description | BACKGROUND: Hemophagocytic lymphohistiocytosis (HLH) is a rare multisystem disorder that frequently involves the central nervous system (CNS). We compared the clinical characteristics, treatment, and prognosis of patients with HLH according to the degree of CNS involvement. METHODS: The clinical manifestations, initial laboratory data, treatment, and outcomes for 50 patients diagnosed with HLH and treated at Asan Medical Center between January 1995 and August 2011 were retrospectively reviewed and analyzed. CNS involvement was defined as the presence of neurological symptoms or an elevated white blood cell (WBC) count in the cerebrospinal fluid (CSF). RESULTS: Among these 50 patients, 23 (46%) developed CNS disease. Among patients with CNS disease, 19 had neurological symptoms, including seizures, altered consciousness, facial palsy, dysarthria, and dysphagia. Four patients had elevated CSF WBC counts without neurological symptoms. Twelve patients had abnormal brain imaging results, including high signal intensity lesions on T2-weighted magnetic resonance imaging (MRI) findings, ventriculomegaly, hemorrhage, atrophy, and leptomeningeal enhancement. Patients with CNS disease had lower ferritin, aspartate aminotransferase (AST), and alanine aminotransferase (ALT) levels as well as reduced 5-year survival as compared to those without CNS disease. CONCLUSION: CNS involvement is common among patients with HLH. Overall, patients with CNS disease achieve poorer outcomes than patients without CNS involvement. To improve outcomes, physicians must carefully monitor the neurological manifestations in patients with HLH and administer the appropriate course of intensified chemotherapy to patients with CNS disease. |
format | Online Article Text |
id | pubmed-3538799 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2012 |
publisher | Korean Society of Hematology; Korean Society of Blood and Marrow Transplantation; Korean Society of Pediatric Hematology-Oncology; Korean Society on Thrombosis and Hemostasis |
record_format | MEDLINE/PubMed |
spelling | pubmed-35387992013-01-14 Central nervous system (CNS) involvement is a critical prognostic factor for hemophagocytic lymphohistiocytosis Kim, Myung-Mi Yum, Mi-Sun Choi, Hae-Won Ko, Tae-Sung Im, Ho Joon Seo, Jong-Jin Koh, Kyung-Nam Korean J Hematol Original Article BACKGROUND: Hemophagocytic lymphohistiocytosis (HLH) is a rare multisystem disorder that frequently involves the central nervous system (CNS). We compared the clinical characteristics, treatment, and prognosis of patients with HLH according to the degree of CNS involvement. METHODS: The clinical manifestations, initial laboratory data, treatment, and outcomes for 50 patients diagnosed with HLH and treated at Asan Medical Center between January 1995 and August 2011 were retrospectively reviewed and analyzed. CNS involvement was defined as the presence of neurological symptoms or an elevated white blood cell (WBC) count in the cerebrospinal fluid (CSF). RESULTS: Among these 50 patients, 23 (46%) developed CNS disease. Among patients with CNS disease, 19 had neurological symptoms, including seizures, altered consciousness, facial palsy, dysarthria, and dysphagia. Four patients had elevated CSF WBC counts without neurological symptoms. Twelve patients had abnormal brain imaging results, including high signal intensity lesions on T2-weighted magnetic resonance imaging (MRI) findings, ventriculomegaly, hemorrhage, atrophy, and leptomeningeal enhancement. Patients with CNS disease had lower ferritin, aspartate aminotransferase (AST), and alanine aminotransferase (ALT) levels as well as reduced 5-year survival as compared to those without CNS disease. CONCLUSION: CNS involvement is common among patients with HLH. Overall, patients with CNS disease achieve poorer outcomes than patients without CNS involvement. To improve outcomes, physicians must carefully monitor the neurological manifestations in patients with HLH and administer the appropriate course of intensified chemotherapy to patients with CNS disease. Korean Society of Hematology; Korean Society of Blood and Marrow Transplantation; Korean Society of Pediatric Hematology-Oncology; Korean Society on Thrombosis and Hemostasis 2012-12 2012-12-24 /pmc/articles/PMC3538799/ /pubmed/23320006 http://dx.doi.org/10.5045/kjh.2012.47.4.273 Text en © 2012 Korean Society of Hematology 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 | Original Article Kim, Myung-Mi Yum, Mi-Sun Choi, Hae-Won Ko, Tae-Sung Im, Ho Joon Seo, Jong-Jin Koh, Kyung-Nam Central nervous system (CNS) involvement is a critical prognostic factor for hemophagocytic lymphohistiocytosis |
title | Central nervous system (CNS) involvement is a critical prognostic factor for hemophagocytic lymphohistiocytosis |
title_full | Central nervous system (CNS) involvement is a critical prognostic factor for hemophagocytic lymphohistiocytosis |
title_fullStr | Central nervous system (CNS) involvement is a critical prognostic factor for hemophagocytic lymphohistiocytosis |
title_full_unstemmed | Central nervous system (CNS) involvement is a critical prognostic factor for hemophagocytic lymphohistiocytosis |
title_short | Central nervous system (CNS) involvement is a critical prognostic factor for hemophagocytic lymphohistiocytosis |
title_sort | central nervous system (cns) involvement is a critical prognostic factor for hemophagocytic lymphohistiocytosis |
topic | Original Article |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3538799/ https://www.ncbi.nlm.nih.gov/pubmed/23320006 http://dx.doi.org/10.5045/kjh.2012.47.4.273 |
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