Outcome and Prognostic Factors of Hemophagocytic Lymphohistiocytosis in Children: Experience From a Low- and Middle-Income Country.

Autor:	Hira B; Department of Paediatric Oncology, Combined Military Hospital, Rawalpindi, PAK., Siddique AW; Department of Paediatric Oncology, Combined Military Hospital, Rawalpindi, PAK., Ahmed S; Department of Paediatric Oncology, Combined Military Hospital, Rawalpindi, PAK., Latif A; Department of Paediatric Oncology, Combined Military Hospital, Rawalpindi, PAK., Manzoor R; Department of Paediatric Oncology, Combined Military Hospital, Rawalpindi, PAK., Ghafoor T; Armed Forces Bone Marrow Transplant Centre, Department of Paediatrics, Combined Military Hospital, Rawalpindi, PAK., Arshed A; Department of Paediatric Oncology, Combined Military Hospital, Rawalpindi, PAK.
Jazyk:	angličtina
Zdroj:	Cureus [Cureus] 2024 Jun 16; Vol. 16 (6), pp. e62494. Date of Electronic Publication: 2024 Jun 16 (Print Publication: 2024).
DOI:	10.7759/cureus.62494
Abstrakt:	Objective Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening condition especially in low- and middle-income countries (LMICs). This study was done to evaluate the outcome and prognostic factors of HLH in patients presenting to our center. Methods The study was carried out at the Paediatric Oncology Department of Combined Military Hospital (CMH) in Rawalpindi, Pakistan. All cases of HLH, from one month to 15 years of age enrolled between January 1, 2013 to June 30, 2023, were included. IBM SPSS Statistics for Windows, version 25.0 (released 2017, IBM Corp., Armonk, NY) was used for statistical analysis, and t-test and chi-square tests were used for comparison between continuous and categorical variables. Frequencies and percentages were calculated for categorical variables. Results Out of 115 patients, seven (6%) abandoned the treatment. The data of 108 cases, including 58 males (53.7%), were analyzed. The mean age at diagnosis was 31.5 ± 39.03 months. The mean time to reach a pediatric oncologist was 30.20 ± 22.15 days. Fever and pallor were common symptoms occurring in 107 (99.1%) and 98 (90.7%) cases, respectively. Jaundice was present in 44 (40.7%), visceromegaly in 64 (59.3%), and bruising/bleeding in 16 cases (14.8%). Twenty-six (24.1%) patients underwent hematopoietic stem cell transplant (HSCT), out of which 17 (65.4%) children were cured. Overall survival at two years, five years, and 10 years was 38%, 37%, and 36.1%, respectively. Disease-free survival at two years, five years, and 10 years was 33.3%, 32.4%, and 31.5%, respectively. Conclusion HLH leads to high mortality due to delayed or misdiagnosis in LMICs. Early diagnosis and early referral to a pediatric oncologist is the detrimental factor in survival for HLH. HSCT is the treatment of choice for primary, refractory, or relapse cases. Competing Interests: Human subjects: Consent was obtained or waived by all participants in this study. Institutional Review Board/Ethical Committee of Combined Military Hospital, Rawalpindi issued approval 545. The article has been scrutinized thoroughly, and no violation of patient safety and privacy, breach of data, and violations of human rights and institutional rights have been observed. Animal subjects: All authors have confirmed that this study did not involve animal subjects or tissue. Conflicts of interest: In compliance with the ICMJE uniform disclosure form, all authors declare the following: Payment/services info: All authors have declared that no financial support was received from any organization for the submitted work. Financial relationships: All authors have declared that they have no financial relationships at present or within the previous three years with any organizations that might have an interest in the submitted work. Other relationships: All authors have declared that there are no other relationships or activities that could appear to have influenced the submitted work. (Copyright © 2024, Hira et al.)
Databáze:	MEDLINE
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