Approach to the patient with neutropenia in childhood

Neutrophils have an important role in host defense and acute inflammation. It is well known that susceptibility to infection increases when the neutrophil count is low. Neutropenia were classified as mild, moderate and severe according to the neutrophil counts, or acute and chronic depending on the duration of neutropenia, or congenital and acquired according to the mechanism. The patients with neutropenia are clinically different due to underlying mechanism, they have life- threatening infections or no infection may be observed. The most common cause of acquired neutropenia is viral infection, followed by drugs and autoimmune neutropenia. Congenital neutropenia are usually diagnosed by acute and life- threatening invasive bacterial and fungal infections. Immune system disorders and other systemic abnormalities may be accompanied or not. Recent years, novel single gen defects causing congenital neutropenia were defined through advanced genetic techniques. Molecular diagnosis is useful for risk stratification, choice of therapy and prognosis on follow- up. This review was prepared for pediatricians as a guide focused on approach neutropenia, which tests should be performed and when should be referred to a specialist

Predictive Factors for Response to a Standard Dose of Intravenous Immunoglobulin Therapy in Children with Immune Thrombocytopenia

Objective: Acute immune thrombocytopenic purpura (ITP) is a common acquired bleeding disorder. Intravenous immunoglobulin (IVIG) therapy is commonly given as initial treatment to pediatric patients with ITP. Factors that can predict the response to IVIG have not been fully determined. We retrospectively evaluated whether the clinical and laboratory findings of pediatric patients with ITP at the time of diagnosis could predict the response to IVIG and progression to chronic ITP. Methods: A total of 45 patients with newly diagnosed ITP who were initially treated with IVIG were evaluated between January 2016 and December 2019. Short-term response was estimated by platelet counts 2 weeks after IVIG, and long- term response was assessed by thrombocytopenia-free survival (TFS). TFS was defined as the probability of survival without treatment failure after initial IVIG, such as relapse, requiring additional therapeutic interventions, or progression to chronic ITP. Results: In univariate analysis, age ≥25 months (p=0.002), platelet count ≤6.9x109 /L (p=0.034), and hemoglobin (Hb) level >12.4 g/dl (p=0.001) were considered to be unfavourable factors for short-term response. Univariate analysis of unfavourable factors for longterm response showed that age ≥25 months (p=0.002), platelet count ≤6.9x109 /L (p=0.034), and Hb level >12.4 g/dl (p=0.001) were significant factors. Conclusion: These results suggest that in newly diagnosed ITP patients older than 25 months and/or with platelet count <6.9x109 /L, other therapeutic options such as corticosteroids alone or in combination with IVIG may be considered as initial therap