Clinical features and outcome analysis of 83 childhood Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis with HLH-2004 protocol / 中华血液学杂志
Chinese Journal of Hematology
;
(12): 668-672, 2011.
Article
in Chinese
| WPRIM
| ID: wpr-251476
ABSTRACT
<p><b>OBJECTIVE</b>To investigate the clinical features of Epstein-Barr virus-related hemophagocytic lymphohistiocytosis (EBV-HLH), to analysis the outcome of HLH-2004 protocol, and to explore the prognostic factors in EBV-HLH patients.</p><p><b>METHODS</b>The clinical features at onset and outcome of HLH-2004 protocol from 83 pediatric patients with EBV-HLH enrolled from January 2006 to December 2009 in our hospital were analyzed retrospectively. Univariate and multivariate COX regression analysis were used to identify statistically significant prognostic factors.</p><p><b>RESULTS</b>(1) Among the 83 patients, 45 were males and 38 were females. The age of onset ranged from 6 months to 14 years 4 months. 44 patients were treated with HLH-2004, and 3-year overall survival (OS) was (55.8 ± 7.9)%. (2) The most common clinical features of EBV-HLH included high fever, cytopenia, hepatosplenomegaly, and coagulopathy; The respiratory symptoms, angina phlogistic, skin rashes, neurologic abnormality were rare. 97.3% of patients showed an elevation of serum ferritin, liver dysfunction and lipid metabolism disorders was found in most of EBV-HLH patients. 89.0% of patient had hemophagocytosis in bone marrow at diagnosis of EBV-HLH. (3) COX regression analysis revealed that anemia degree, serum albumin < 30 g/L, CD4CD8 abnormity, NK cell < 3%, treatment protocol were related with the prognosis significantly (P < 0.05).</p><p><b>CONCLUSION</b>EBV-HLH in pediatric patients has severe clinical feature and poor prognosis. HLH-2004 protocol is an effective treatment for patients with EBV-HLH. Symptomatic treatment can't rescue the patients of EBV-HLH.</p>
Full text:
Available
Index:
WPRIM (Western Pacific)
Main subject:
Prognosis
/
Virology
/
Killer Cells, Natural
/
Retrospective Studies
/
Treatment Outcome
/
Herpesvirus 4, Human
/
Epstein-Barr Virus Infections
/
Drug Therapy
/
Lymphohistiocytosis, Hemophagocytic
Type of study:
Practice guideline
/
Observational study
/
Prognostic study
/
Risk factors
Limits:
Adolescent
/
Child
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Child, preschool
/
Female
/
Humans
/
Infant
/
Male
Language:
Chinese
Journal:
Chinese Journal of Hematology
Year:
2011
Type:
Article
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