Pediatric hemophagocytic lymphohistiocytosis in a tropical country : Results of a multicenter study in Thailand
© 2022 John Wiley & Sons Australia, Ltd..
BACKGROUND: Hemophagocytic lymphohistiocytosis (HLH) is a rare, life-threatening condition caused by genetic mutation or various triggers disturbing the immune system.
METHODS: A multicenter retrospective study of pediatric patients with HLH receiving a diagnosis between January 2005 and December 2019 from three pediatric oncology centers was conducted to explore the clinical characteristics and determine prognostic factors associated with outcomes among Thai children.
RESULTS: In all, 78 patients with HLH with a median age at diagnosis of 3.17 (range, .08-17.83) years were enrolled. The male to female ratio was 1.2:1. The most common type of HLH was infection-associated hemophagocytic syndrome (IAHS) (n = 59, 75%) of which Epstein-Barr virus was the most common pathogen. Thrombocytopenia, hyperbilirubinemia, and treatment response at weeks 2 and 8 after initiating treatment were associated with mortality. Platelet count <50,000 cells/mm3 was the only independent prognostic factor to define survival outcome (p-value .035). Two-year overall survival rate was 71.3% (95% confidence interval, 59.2%-80.3%). Survival rates between IAHS, malignant associated HLH, macrophage activation syndrome, and unspecific HLH did not significantly differ (p-value .571).
CONCLUSION: IAHS was the most common cause among pediatric HLH in Thailand. The outcomes of Thai children with HLH were comparable to those of developed countries. Platelet count <50,000 cells/mm3 was the only independent prognostic factor to define survival outcome.
| Medienart: |
E-Artikel |
|---|
| Erscheinungsjahr: |
2023 |
|---|---|
| Erschienen: |
2023 |
| Enthalten in: |
Zur Gesamtaufnahme - volume:19 |
|---|---|
| Enthalten in: |
Asia-Pacific journal of clinical oncology - 19(2023), 2 vom: 15. Apr., Seite e128-e137 |
| Sprache: |
Englisch |
|---|
| Beteiligte Personen: |
Rungrojjananon, Nattapol [VerfasserIn] |
|---|
| Links: |
|---|
| Themen: |
129178-67-2 |
|---|
| Anmerkungen: |
Date Completed 31.03.2023 Date Revised 31.03.2023 published: Print-Electronic Citation Status MEDLINE |
|---|
| doi: |
10.1111/ajco.13805 |
|---|
| funding: |
|
|---|---|
| Förderinstitution / Projekttitel: |
|
| PPN (Katalog-ID): |
NLM342343173 |
|---|
| LEADER | 01000naa a22002652 4500 | ||
|---|---|---|---|
| 001 | NLM342343173 | ||
| 003 | DE-627 | ||
| 005 | 20231226013907.0 | ||
| 007 | cr uuu---uuuuu | ||
| 008 | 231226s2023 xx |||||o 00| ||eng c | ||
| 024 | 7 | |a 10.1111/ajco.13805 |2 doi | |
| 028 | 5 | 2 | |a pubmed24n1141.xml |
| 035 | |a (DE-627)NLM342343173 | ||
| 035 | |a (NLM)35712989 | ||
| 040 | |a DE-627 |b ger |c DE-627 |e rakwb | ||
| 041 | |a eng | ||
| 100 | 1 | |a Rungrojjananon, Nattapol |e verfasserin |4 aut | |
| 245 | 1 | 0 | |a Pediatric hemophagocytic lymphohistiocytosis in a tropical country |b Results of a multicenter study in Thailand |
| 264 | 1 | |c 2023 | |
| 336 | |a Text |b txt |2 rdacontent | ||
| 337 | |a ƒaComputermedien |b c |2 rdamedia | ||
| 338 | |a ƒa Online-Ressource |b cr |2 rdacarrier | ||
| 500 | |a Date Completed 31.03.2023 | ||
| 500 | |a Date Revised 31.03.2023 | ||
| 500 | |a published: Print-Electronic | ||
| 500 | |a Citation Status MEDLINE | ||
| 520 | |a © 2022 John Wiley & Sons Australia, Ltd. | ||
| 520 | |a BACKGROUND: Hemophagocytic lymphohistiocytosis (HLH) is a rare, life-threatening condition caused by genetic mutation or various triggers disturbing the immune system | ||
| 520 | |a METHODS: A multicenter retrospective study of pediatric patients with HLH receiving a diagnosis between January 2005 and December 2019 from three pediatric oncology centers was conducted to explore the clinical characteristics and determine prognostic factors associated with outcomes among Thai children | ||
| 520 | |a RESULTS: In all, 78 patients with HLH with a median age at diagnosis of 3.17 (range, .08-17.83) years were enrolled. The male to female ratio was 1.2:1. The most common type of HLH was infection-associated hemophagocytic syndrome (IAHS) (n = 59, 75%) of which Epstein-Barr virus was the most common pathogen. Thrombocytopenia, hyperbilirubinemia, and treatment response at weeks 2 and 8 after initiating treatment were associated with mortality. Platelet count <50,000 cells/mm3 was the only independent prognostic factor to define survival outcome (p-value .035). Two-year overall survival rate was 71.3% (95% confidence interval, 59.2%-80.3%). Survival rates between IAHS, malignant associated HLH, macrophage activation syndrome, and unspecific HLH did not significantly differ (p-value .571) | ||
| 520 | |a CONCLUSION: IAHS was the most common cause among pediatric HLH in Thailand. The outcomes of Thai children with HLH were comparable to those of developed countries. Platelet count <50,000 cells/mm3 was the only independent prognostic factor to define survival outcome | ||
| 650 | 4 | |a Multicenter Study | |
| 650 | 4 | |a Journal Article | |
| 650 | 4 | |a Thailand | |
| 650 | 4 | |a hemophagocytic lymphohistiocytosis | |
| 650 | 4 | |a outcome | |
| 650 | 4 | |a platelet count | |
| 650 | 4 | |a prognostic factors | |
| 650 | 7 | |a 3-iodo-4-azido-N-hexadecylsalicylamide |2 NLM | |
| 650 | 7 | |a 129178-67-2 |2 NLM | |
| 700 | 1 | |a Pakakasama, Samart |e verfasserin |4 aut | |
| 700 | 1 | |a Winaichatsak, Angkana |e verfasserin |4 aut | |
| 700 | 1 | |a Siriwanawong, Rapee |e verfasserin |4 aut | |
| 700 | 1 | |a Rujkijyanont, Piya |e verfasserin |4 aut | |
| 700 | 1 | |a Traivaree, Chanchai |e verfasserin |4 aut | |
| 700 | 1 | |a Photia, Apichat |e verfasserin |4 aut | |
| 700 | 1 | |a Monsereenusorn, Chalinee |e verfasserin |4 aut | |
| 773 | 0 | 8 | |i Enthalten in |t Asia-Pacific journal of clinical oncology |d 2010 |g 19(2023), 2 vom: 15. Apr., Seite e128-e137 |w (DE-627)NLM197405231 |x 1743-7563 |7 nnns |
| 773 | 1 | 8 | |g volume:19 |g year:2023 |g number:2 |g day:15 |g month:04 |g pages:e128-e137 |
| 856 | 4 | 0 | |u http://dx.doi.org/10.1111/ajco.13805 |3 Volltext |
| 912 | |a GBV_USEFLAG_A | ||
| 912 | |a GBV_NLM | ||
| 951 | |a AR | ||
| 952 | |d 19 |j 2023 |e 2 |b 15 |c 04 |h e128-e137 | ||