|Title:||Outcomes and prognostic factors associated with 180-day mortality in Taiwanese pediatric patients with Hemophagocytic Lymphohistiocytosis||Authors:||Yu T.-Y.
|Issue Date:||2021||Publisher:||Elsevier B.V.||Journal Volume:||120||Journal Issue:||4||Start page/Pages:||1061-1068||Source:||Journal of the Formosan Medical Association||Abstract:||
Background/Purpose: Hemophagocytic lymphohistiocytosis (HLH), a rarely occurring syndrome with various triggers, is associated with early mortality. Owing to a lack of sufficient corresponding data in Taiwan, this study aimed to identify the outcome and potential factors associated with 180-day mortality in pediatric HLH. Methods: This retrospective study analyzed clinical and laboratory data on pediatric patients diagnosed with HLH at our institute (1995–2019). Logistic regression analysis was conducted to determine the associations between various factors and 180-day mortality. Results: Overall, 48 patients had HLH; their median age at diagnosis was 5 years (interquartile range: 2–11 years). Clinical presentations and laboratory parameters required for diagnosis included fever (98%), splenomegaly (79%), hyperferritinemia (98%), hemophagocytosis (94%), thrombocytopenia (90%), anemia (63%), hypertriglyceridemia (68%), and neutropenia (57%). The 5-year overall survival (OS) rate was 49%. Of 22 patients who had died at the last follow-up, 15 (68%) died within 180 days after diagnosis. In the multivariate analysis, hemoglobin (odds ratio [OR]: 0.564, p = 0.024) and triglyceride (OR: 1.004, p = 0.049) were significantly associated with 180-day mortality. Higher triglyceride levels at diagnosis were related to significantly lower 180-day OS rates (52.9% vs. 86.1%, p = 0.018). Conclusion: The overall outcome in our cohort was similar to that reported in some of the largest international cohorts. Hypertriglyceridemia and anemia may be indicative of poor prognoses in pediatric HLH patients independently and may be used to guide treatment strategy formulations for better outcomes. ? 2020
|ISSN:||0929-6646||DOI:||10.1016/j.jfma.2020.10.026||SDG/Keyword:||creatinine; cyclosporine; etoposide; ferritin; hemoglobin; immunoglobulin; lactate dehydrogenase; prednisolone; triacylglycerol; activated partial thromboplastin time; adolescent; anemia; Article; Chediak Higashi syndrome; child; childhood cancer; clinical article; cohort analysis; controlled study; corticosteroid therapy; creatinine blood level; DiGeorge syndrome; edema; Epstein Barr virus infection; erythrophagocytosis; female; fever; follow up; hematopoietic stem cell transplantation; hemophagocytic syndrome; hepatomegaly; human; hyperferritinemia; hypertriglyceridemia; immunocompromised patient; Kikuchi disease; laboratory test; lactate dehydrogenase blood level; lymphadenopathy; macrophage activation syndrome; male; mortality; mucocutaneous lymph node syndrome; neutropenia; neutrophil count; overall survival; pediatric patient; platelet count; postoperative hemorrhage; prognosis; rash; retrospective study; sepsis; splenomegaly; survivor; systemic juvenile idiopathic arthritis; T cell lymphoma; Taiwanese; thrombocytopenia; triacylglycerol blood level; epidemiology; hemophagocytic syndrome; preschool child; prognosis; survival rate; Taiwan; Child; Child, Preschool; Humans; Lymphohistiocytosis, Hemophagocytic; Prognosis; Retrospective Studies; Survival Rate; Taiwan
|Appears in Collections:||醫學系|
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