| Autor: |
Cetinkaya PG; Department of Pediatrics, Division of Pediatric Immunology., Cagdas D; Department of Pediatrics, Division of Pediatric Immunology., Gumruk F; Department of Pediatrics, Division of Pediatric Hematology, Faculty of Medicine, Hacettepe University, Ankara, Turkey., Tezcan I; Department of Pediatrics, Division of Pediatric Immunology. |
| Jazyk: |
angličtina |
| Zdroj: |
Journal of pediatric hematology/oncology [J Pediatr Hematol Oncol] 2020 Aug; Vol. 42 (6), pp. e434-e439. |
| DOI: |
10.1097/MPH.0000000000001803 |
| Abstrakt: |
Hemophagocytic lymphohistiocytosis (HLH) is characterized by uncontrolled and excessive immune responses with high mortality. We aimed to define mortality-related parameters in HLH secondary to primary immunodeficiency (PID). A total of 28 patients with HLH between the years 2013 and 2017 were enrolled in the study. The patients were evaluated in 2 groups including PID with hypopigmentation (n=7) (Chédiak-Higashi syndrome [CHS] and Griscelli syndrome type 2 [GS2]) and other PIDs (n=21). The median age of the study population was 23 (4.3 to 117.0) months at the time of the diagnosis of HLH. Central nervous system involvement was recorded in 7 (GS2/CHS patients [n=4], other PIDs [n=3], P=0.026), and death was observed in 9 patients (GS2/CHS patients [n=1], other PIDs [n=8], P=0.371). Five patients (3 GS2/CHS and 2 other PID patients) underwent hematopoietic stem cell transplantation. Low serum albumin level was the only variable associated with the mortality and albumin levels less than the cut-off value of 3.07 g/dL increased mortality 5.8 times in patients with HLH secondary to PID. We presented a single-center experience consisting of patients with HLH secondary to PID with a mortality rate of 32.1%. Hypoalbuminemia was the only risk factor to increase the overall mortality rate of HLH. |
| Databáze: |
MEDLINE |
| Externí odkaz: |
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