BACKGROUND: Hemophagocytic lymphohistiocytosis (HLH) is a syndrome characterized by extreme immune activation, resulting in pathologic inflammation that may be life-threatening. Early recognition is crucial since survival largely depends on early initiation of treatment which utilizes a combination of chemotherapy, immunotherapy and in some cases even bone marrow transplantation. OBJECTIVES: This study aims to describe the clinical characteristics, treatment received and outcome of patients diagnosed with HLH at the Philippine Children’s Medical Center from 2004 to 2017. MATERIALS AND METHODS: A retrospective analysis of records of children 0-18 years of age diagnosed with HLH from January 2004 to December 2017 was done. RESULTS: A total of 39 patients were included in the study which gave an incidence of 1.22 per 3000 patients admitted under 18 years of age. There were 29 males (74.4%) and 10 females (25.6%) with a male to female ratio of 2.9:1 Mean age was 6.12 ± 3.89 years. The average time from initial presentation to diagnosis was 6 weeks and 2 days. The most commonly seen clinical and laboratory features observed in these patients were fever (100%), splenomegaly (71.8%), anemia (87.17%), thrombocytopenia (79.48%) and hypertriglyceridemia (69.23%). Only 5 patients were confirmed to be familial HLH with 3 having XLP gene mutation, and one each having syntaxin and perforin gene mutations. Majority of patients received a combination of treatment based on the HLH 2004 regimen while almost one third only received antibiotics. Only 23% of patients survived during the study period and all but one of these patients received drug combinations based on the HLH 2004 protocol. CONCLUSIONS AND RECOMMENDATIONS HLH is a rare but important condition that must be recognized early and treated appropriately to optimize survival. The mortality rate of 39 patients seen in this institution is high. There is a need to better utilize the diagnostic criteria of the disease and to employ a more uniform treatment strategy. Increasing awareness among health care personnel can also improve case finding, characterization and treatment.
Human ; Male ; Female ; Adolescent: 13-18 Yrs Old ; Lymphohistiocytosis, Hemophagocytic ; Philippines

Objective: To evaluate the clinical and microbiological profile and factors affecting outcome among pediatric febrile neutropenic (FN) patients with hematologic malignancies (HM) Methodology: This was a cross-sectional study which looked into medical records of Filipino children 0-18years old diagnosed with FN and HM and admitted from June 2016 up to June 2022 at the St. Luke’s Medical Center, Quezon City (SLMC-QC). Data on age, sex, underlying malignancy, stage of treatment, site of infection, presence of central line, initial antibiotic therapy, culture positivity and isolates were retrospectively evaluated. Incomplete records were excluded. The relationship between clinical & microbiologic profile and outcomes were analyzed using T-test and Chi-square test. Significance was set at p<0.05. Results This study included 267 episodes of FN. Patients had a mean age of 8.3 years with male preponderance (59%). The most frequent underlying malignancy was acute lymphoblastic leukemia (61%). Episodes occurred primarily during the induction (40%) and consolidation phases (28%) of chemotherapy. Most (65%) had an absolute neutrophil count (ANC) of <100/mm3 . Central line catheter was present in 59% of episodes and 52% had an implanted port. There was no identifiable focus of infection in 52% of cases. Gram-negative bacteria, specifically Klebsiella pneumoniae (13%) and Escherichia coli (11%) were the most common isolates. Most patients (88%) recovered. Age >10years, male sex, diagnosis of acute myelogenous leukemia, relapse disease, ANC <100/mm3 , presence of a central line, and central line associated bloodstream infection were significantly associated with duration of hospital stay. Presence of central venous line was the most significant factor associated with mortality. Conclusions: Several clinical and microbiological factors, specifically age >10years, male sex, diagnosis of acute myelogenous leukemia, relapse disease, ANC <100/mm3 , presence of a central line, and central line associated bloodstream infection, were documented to significantly affect outcome in Filipino pediatric FN patients with HM.
Febrile Neutropenia ; Hematologic Neoplasms ; Leukemia