Clinico-etiological Profile & Outcome of Children with Ferritin >10,000 Microgram/Liter

INTRODUCTION: Ferritin levels above 10,000 microgram/L, help to identify underlying systemic conditions like primary HLH or secondary HLH/Macrophage Activation Syndrome due to infections, autoimmune conditions and malignancies with a higher specificity of 98%. We utilized quantitative ferritin levels as an indirect measure of pro-inflammatory cytokine activation as the magnitude of elevation in serum ferritin has been linked with a worse prognosis of the disease be it primary HLH/secondary HLH/MAS. OBJECTIVES: Etiology, clinical course and outcome of children with ferritin >10000 μg/L will be recorded and correlated with severity of hyperferritinemia. MATERIALS AND METHODS: INCLUSION CRITERIA: Children aged between 1 month and 18 years of age admitted with ferritin level of >10,000 microgram/L. EXCLUSION CRITERIA: Children of age group 1 month -18 years who are already diagnosed with Hemochromatosis/Hemoglobinopathy (Received multiple blood transfusions in the past). Sample size:65 RESULTS: √ Mean age of presentation was 5.5 years with male predominance. Mean day of illness at presentation is 6.7 +/- 4 days. Average length of hospital stay was 8.8 +/- 8.37 days and PICU stay was 6.9 +/- 8.25 days. Mean ferritin value was 34,358 microgram/L. √ Common disease associations among the study populations were shortlisted in the descending order were infections being more common (58/65-89.2%) followed by rheumatological conditions (2/65-3.07%). Among the infectious causes viral infections topped with dengue infection -most common (46/65-73.84%) followed by Influenza A infection (4/65-6.15%), respiratory syncytial viral infection (3/65-4.61%). √ Clinically fever was the only parameter noted in 100% of our population followed by positive CRP (56/65-86.1%), coagulopathy (53/65-86.1%), thrombocytopenia (47/65-72.3%), encephalopathy (45/65-69.2%) and transaminitis (44/65-67.6%). The above parameters directly correlated with mortality rate in combination with ferritin >10000μg/L. √ Review of the data related to organ support therapy showed 36/65-55.4% required inotrope support, 29/65-44.6% required mechanical ventilation, 28/65-43.1% required blood product transfusions and 11/65-16.9% required renal replacement therapy during the hospital stay. √ Immuno-modulatory therapy was required in 53/65-81.5% cases where combination of anakinra and steroid therapy was more commonly received by our study population. But mortality rate was high in the same group of patients signifies the severity of hyperferritnemic mutiorgan dysfunction; √ Mortality rate was highly associated (100%) with ferritin of >1,00,000 microgram/L with statistically significant P-values. CONCLUSIONS: Viral infections are strongly associated with very high ferritin responses. Ferritin levels above 10,000 μg/L carry a 16.98% mortality rate, even with immunotherapy. Ferritin levels exceeding 50,000 μg/L are linked to very high mortality despite immunotherapy efforts. Higher ferritin levels correlate with an increased incidence of end-organ dysfunction. The need for supportive treatments, such as mechanical ventilation, vasopressor support, and immunomodulation, increases with the severity of hyper-ferritinemia.