<p>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.

p><p>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.

METHODS: A retrospective analysis of records of children 0 -18 years of age diagnosed with HLH from January 2004 to December 2017 was done.

p><p>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.
p><p>CONCLUSION: 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.p>
Human ; Male ; Female ; Adolescent: 13-18 Yrs Old ; Lymphohistiocytosis, Hemophagocytic ; Philippines

OBJECTIVES: The Cancer and Hematology Division of the PCMC receives an average of 24 cases of pediatric intrathoracic masses annually. Comprehensive data on the demographic status, clinical profile, management, and outcome are still not available. This study aims to determine the clinical features, diagnosis, management and outcome of children and adolescents with intrathoracic masses from 2017 to 2019. MATERIALS AND METHODS: Descriptive study design was utilized. Data were collected by doing a chart review. Possible associations between the clinical features and outcome were described. RESULTS: Sixty-eight (68) cases were referred from January 2017 to December 2019. Mean age at diagnosis is 8.8 years with a 2.4:1 male to female ratio. Severe wasting was seen in 21%. All subjects were symptomatic at presentation, 50% with respiratory compromise. Anterior mediastinal lesions are observed at 82% of cases. Elevated LDH was seen in 50% of the patients. Malignant hematologic lesions are the most common etiology. Steroid pretreatment was given in 40% of patients. Only a small percentage (<20%) underwent definitive treatment. Patients were symptomatic for 18 days on average before consult. It took an average of 18 days for a case to be diagnosed definitively, and 10 days from the diagnosis to start of directed treatment. Mortality rate was high at 57.4% CONCLUSION Patients with intrathoracic mass and malnutrition are 1.4x more likely to die. Diagnosis is the most significant factor associated with death. Observed data can be used as basis to formulate protocols which can streamline the diagnostic and therapeutic approach in these patients.
leukemia ; lymphoma

BACKGROUND: Tumor lysis syndrome (TLS) is an oncologic emergency resulting from cancer chemotherapy; delays in its recognition could be life-threatening. Early recognition of associated risk factors and its management may help prevent its occurrence. OBJECTIVE: To identify the risk factors for TLS among cancer patients at the Philippine Children’s Medical Center. METHODS: This was a retrospective case-control study. Categorical variables were compared using chi-square test and continuous variables were compared using independent t-test. The association between TLS and patients’ characteristics was determined through logistic regression analysis. RESULTS: Medical records of 712 patients with cancer seen between 2016-2020 were reviewed. Children with (n=35) and without (n=137) TLS were selected as cases and controls and matched for age and cancer type. Factors associated with TLS are underweight patients with BMI < 18.5 (cOR 0.33, 95% CI 0.11-0.98); patients with both hepatomegaly and splenomegaly were four times more likely to develop TLS (cOR 3.946, 95% CI 1.2-12.94) while patients with lymphadenopathy were twice more likely to develop TLS (cOR 2.309, 95% CI 1.02-5.21). Patients with elevated WBC, low phosphorus and high uric acid at baseline have increased odds of developing TLS. CONCLUSIONS After group matching for age and cancer type, factors associated with increased odds of TLS among pediatric cancer patients in PCMC are hepatosplenomegaly, lymphadenopathy, elevated WBC, low potassium level, low phosphorus and high uric acid at baseline with higher fluid balance.
tumor lysis syndrome

Subcutaneous panniculitis-like T-cell lymphoma (SPTCL) is a rare form of cytotoxic T-cell lymphoma of the skin localized primarily in the subcutaneous adipose tissue. Clinically, the skin lesions mimic lipomas, but histologically resemble panniculitis, which is an inflammation of the subcutaneous fats. Most cases have an excellent prognosis and follow an indolent clinical course with a 5-year overall survival rate of 80%. So far, only a few cases have been reported in the pediatric age group. The diagnosis of SPTCL is based on the combination of clinical presentation, histopathologic examination of the skin lesion, immunohistochemical staining, and molecular analysis. Notably, data on treatment of pediatric SPTCL are even fewer in number, and very few patients undergoing effective treatment have been documented. This is a case report of a 15-year-old female adolescent diagnosed with Subcutaneous panniculitis-like T-cell lymphoma who presented with multiple, non-tender, erythematous to violaceous deep dermal and subcutaneous nodules on the lower extremities associated with myalgia, intermittent moderate-grade fever, and weight loss over the past 7 months. She was initially managed as a case of ―growing pain‖ and acute rheumatic fever, until histopathologic findings of the nodules showed inflammation of the subcutaneous fats, and immunohistochemistry revealed findings consistent with SPTCL. She is currently being treated with multi-agent combination chemotherapy which resulted in improvement of symptoms.

Chronic Granulomatous Disease (CGD) is caused by defects in the phagocyte NADPH oxidase and occurs in approximately 1:200,000 births worldwide. It presents with early onset of severe recurrent bacterial and fungal infections. This is a case of a 9-year old male with severe, recurrent bacterial infections since 3 weeks of age. Initial Nitroblue tetrazolium (NBT) reduction tests were normal but a DNA analysis revealed a previously unreported homozygous mutation in CYBB, p.S418Y. Dihydrorhodamine (DHR) test showed poor neutrophil oxidation consistent with X-linked CGD. Definitive microbiologic diagnosis is essential for directing therapy for recurrent bacterial and fungal infections. Treatment of infections should be aggressive. Lifelong bacterial and fungal prophylaxis is necessary for prolonged survival. We report a case of confirmed CGD with the previously unreported mutation.
Granulomatous Disease

<p style="text-align: justify;">BACKGROUND AND OBJECTIVE: Conventional red cell transfusion formulae used in clinical practice has shown underestimation of the actual post-transfusion hemoglobin level. To address this problem, we aimed to determine if there is an agreement between computed and actual post-transfusion hemoglobin levels using an alternative red cell transfusion formula.p><p style="text-align: justify;">METHODOLOGY: This was a prospective, cross-sectional study. Using Morris' formula, the red cell volume requirements of the participants were computed and post-transfusion hemoglobin levels were obtained for comparison.p><p style="text-align: justify;">RESULT: Majority of the 116participants belongs to age between 2 to 5 years (39.5%) and female (54.3%). Most common indication was hemoglobin level < 7 g/dL with manifestations of anemia (56%). The computed and actual post-transfusion hemoglobin were in agreement. The increase in hemoglobin had direct relationship to the volume of blood transfused and inverse relationship to the age and weight of the patients.p><p style="text-align: justify;">CONCLUSION: Using Morris' formula, the computed and actual post-transfusion hemoglobin values were in agreement. The volume of transfused red cells, age, and weight are predictors of the increase in post-transfusion hemoglobin. This formula can be adopted for Filipino pediatric patients and can obviate the need for hemoglobin determination after transfusion.p>
Human ; Pediatrics ; Blood Transfusion