A retrospective analysis of etiology and outcomes of hemophagocytic lymphohistiocytosis in children and adults
BACKGRUOUND: Hemophagocytic lymphohistiocytosis (HLH) is a rare but severe, life-threatening inflammatory condition if untreated. We aimed to investigate the etiologies, outcomes, and risk factors for death in children and adults with HLH.
METHODS: The medical records of patients who met the HLH criteria of two regional university hospitals in Korea between January 2001 and December 2019 were retrospectively investigated.
RESULTS: Sixty patients with HLH (35 children and 25 adults) were included. The median age at diagnosis was 7.0 years (range, 0.1-83 years), and the median follow-up duration was 8.5 months (range, 0-204 months). Four patients had primary HLH, 48 patients had secondary HLH (20 infection-associated, 18 neoplasm-associated, and 10 autoimmune-associated HLH), and eight patients had HLH of unknown cause. Infection was the most common cause in children (14/35, 40.0%), whereas neoplasia was the most common cause in adults (13/25, 52.0%). Twenty-eight patients were treated with HLH-2004/94 immunochemotherapy. The 5-year overall survival (OS) rate for all HLH patients was 59.9%. The 5-year OS rates for patients with primary, infection-associated, neoplasm-associated, autoimmune-associated, and unknown cause HLH were 25.0%, 85.0%, 26.7%, 87.5%, and 62.5%, respectively. Using multivariate analysis, neoplasm-induced HLH (p=0.001) and a platelet count <50×109/L (p=0.008) were identified as independent risk factors for poor prognosis in patients with HLH.
CONCLUSION: Infection was the most common cause of HLH in children, while it was neoplasia in adults. The 5-year OS rate for all HLH patients was 59.9%. HLH caused by an underlying neoplasm or a low platelet count at the time of diagnosis were risk factors for poor prognosis.
| Medienart: |
E-Artikel |
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| Erscheinungsjahr: |
2021 |
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| Erschienen: |
2021 |
| Enthalten in: |
Zur Gesamtaufnahme - volume:38 |
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| Enthalten in: |
Yeungnam University journal of medicine - 38(2021), 3 vom: 06. Juli, Seite 208-218 |
| Sprache: |
Englisch |
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| Beteiligte Personen: |
Kwak, Abraham [VerfasserIn] |
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| Links: |
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| Themen: |
Hemophagocytic lymphohistiocytosis |
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| Anmerkungen: |
Date Revised 24.04.2022 published: Print-Electronic Citation Status PubMed-not-MEDLINE |
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| doi: |
10.12701/yujm.2020.00591 |
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| funding: |
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| Förderinstitution / Projekttitel: |
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| PPN (Katalog-ID): |
NLM318089335 |
|---|
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| 100 | 1 | |a Kwak, Abraham |e verfasserin |4 aut | |
| 245 | 1 | 2 | |a A retrospective analysis of etiology and outcomes of hemophagocytic lymphohistiocytosis in children and adults |
| 264 | 1 | |c 2021 | |
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| 500 | |a Date Revised 24.04.2022 | ||
| 500 | |a published: Print-Electronic | ||
| 500 | |a Citation Status PubMed-not-MEDLINE | ||
| 520 | |a BACKGRUOUND: Hemophagocytic lymphohistiocytosis (HLH) is a rare but severe, life-threatening inflammatory condition if untreated. We aimed to investigate the etiologies, outcomes, and risk factors for death in children and adults with HLH | ||
| 520 | |a METHODS: The medical records of patients who met the HLH criteria of two regional university hospitals in Korea between January 2001 and December 2019 were retrospectively investigated | ||
| 520 | |a RESULTS: Sixty patients with HLH (35 children and 25 adults) were included. The median age at diagnosis was 7.0 years (range, 0.1-83 years), and the median follow-up duration was 8.5 months (range, 0-204 months). Four patients had primary HLH, 48 patients had secondary HLH (20 infection-associated, 18 neoplasm-associated, and 10 autoimmune-associated HLH), and eight patients had HLH of unknown cause. Infection was the most common cause in children (14/35, 40.0%), whereas neoplasia was the most common cause in adults (13/25, 52.0%). Twenty-eight patients were treated with HLH-2004/94 immunochemotherapy. The 5-year overall survival (OS) rate for all HLH patients was 59.9%. The 5-year OS rates for patients with primary, infection-associated, neoplasm-associated, autoimmune-associated, and unknown cause HLH were 25.0%, 85.0%, 26.7%, 87.5%, and 62.5%, respectively. Using multivariate analysis, neoplasm-induced HLH (p=0.001) and a platelet count <50×109/L (p=0.008) were identified as independent risk factors for poor prognosis in patients with HLH | ||
| 520 | |a CONCLUSION: Infection was the most common cause of HLH in children, while it was neoplasia in adults. The 5-year OS rate for all HLH patients was 59.9%. HLH caused by an underlying neoplasm or a low platelet count at the time of diagnosis were risk factors for poor prognosis | ||
| 650 | 4 | |a Journal Article | |
| 650 | 4 | |a Hemophagocytic lymphohistiocytosis | |
| 650 | 4 | |a Human herpesvirus 4 | |
| 650 | 4 | |a Lymphoproliferative disorders | |
| 650 | 4 | |a Neoplasms | |
| 650 | 4 | |a Survival | |
| 700 | 1 | |a Jung, Nani |e verfasserin |4 aut | |
| 700 | 1 | |a Shim, Ye Jee |e verfasserin |4 aut | |
| 700 | 1 | |a Kim, Heung Sik |e verfasserin |4 aut | |
| 700 | 1 | |a Lim, Hyun Ji |e verfasserin |4 aut | |
| 700 | 1 | |a Lee, Jae Min |e verfasserin |4 aut | |
| 700 | 1 | |a Heo, Mi Hwa |e verfasserin |4 aut | |
| 700 | 1 | |a Do, Young Rok |e verfasserin |4 aut | |
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| 773 | 1 | 8 | |g volume:38 |g year:2021 |g number:3 |g day:06 |g month:07 |g pages:208-218 |
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