Secondary hemophagocytic lymphohistiocytosis in pediatric patients: a single center experience and factors that influenced patient prognosis
Autor: | Emine Polat, Fatma Zehra Öztek Çelebi, Melahat Melek Oguz, Saliha Senel, Husniye Yucel, Esma Altinel Acoglu, Gürses Şahin, Hilal Unsal, Meltem Akcaboy, Eyup Sari |
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Rok vydání: | 2019 |
Předmět: |
Male
Secondary Hemophagocytic Lymphohistiocytosis endocrine system Pediatrics medicine.medical_specialty Adolescent Malignancy Single Center Disease-Free Survival Lymphohistiocytosis Hemophagocytic 03 medical and health sciences 0302 clinical medicine hemic and lymphatic diseases medicine Humans Mean platelet volume Child Retrospective Studies Hemophagocytic lymphohistiocytosis business.industry fungi Infant Hematology musculoskeletal system medicine.disease Survival Rate Oncology Child Preschool 030220 oncology & carcinogenesis Pediatrics Perinatology and Child Health Female Kawasaki disease business Complication hormones hormone substitutes and hormone antagonists 030215 immunology Immune activation |
Zdroj: | Pediatric Hematology and Oncology. 36:1-16 |
ISSN: | 1521-0669 0888-0018 |
Popis: | Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening syndrome of excessive immune activation. Secondary HLH syndrome develops as a complication of infection, drugs, rheumatologic conditions, or malignancy. The main objectives of this work were to identify the etiology of secondary HLH and prognostic factors associated with mortality. Patients diagnosed with secondary HLH, between January 2011 and December 2016, were retrospectively included in this study. We analyzed clinical and laboratory findings as well as prognostic factors from 24 pediatric patients diagnosed with secondary HLH. The mean age of patients at the time of diagnosis was 79.9 ± 68.7 months (range: 2-202) and 54.2% of the patients were male. The most frequent HLH-2004 criterion was fever (100%). Underlying triggers of HLH were as follows: 13 (54.1%) infections, juvenile idiopathic arthritis in 5 patients (20.8%), drugs in 3 patients (12.5%), malignancies in 2 (0.8%), Kawasaki disease in 1 (0.4%) patient, and 1 (0.4%) with unknown triggers. The median time of diagnosis was 3 days (1-67 days). Overall, the mortality rate was 20.8%. In our logistic regression model, factors associated with mortality were decreased albumin levels (OR1 = 2.3[1.48-3.43]) and etoposide usage (OR2 = 1.22 [1.14-1.89]). The patient's 30-day survival was inferior among patients whose albumin level was 2 g/dL or less compared to those over 2 g/dL. Increased awareness of the underlying condition is critical in HLH patients. Our study emphasizes the prognostic significance of albumin level. |
Databáze: | OpenAIRE |
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