Priyanjan Banerjee

Hemophagocytotic lymphohistiocytosis (HLH) is a rare but often life-threatening hyperinflammatory syndrome which is characterised by pathological macrophage activation and failure of self-regulation in immunity. This review examines the key role in macrophages as both victims of defective cytotoxic control, but instead of staying as passive victims, become overactivated partners in immune-mediated tissue damage and primary executors of end-organ damage in HLH. Here, exploration of the links between the impaired perforin-granzyme cytotoxicity, sustained presentation of antigen, and the ultimate culminating into M1 macrophage polarization which drives the clinical conditions of fever, cytopenia, organomegaly, and coagulopathy has been studied. Understanding the dysfunction of macrophages in HLH provides critical insights for targeted therapeutic interventions in this life-threatening rapidly fatal syndrome. Also, the therapeutic targeting of the macrophage pathways, and the future therapeutic developments that are needed to be taken into consideration are discussed, followed by the immediate clinical relevance of macrophage-centric understanding of HLH.

Introduction: Nontransfusion dependent Thalassemia (NTDT) like E-beta thalassemia and -thalassemia intermedia are the most common Thalassemia syndrome in India. As compared to transfusion dependent Thalassemia (TDT), Non Transfusion Dependent Thalassemia (NTDT) does not require regular and frequently packed RBC transfusion to prevent anemia related complications. Still NTDT subjects may suffer from the complications of Iron overload due to ineffective erythropoesis and increased intestinal iron absorption. Hydroxyurea therapy which reduces transfusion requirement by raising fetal Hb level and early iron chelation therapy to reduce Liver Iron Concentration (LIC) are mainstay of management in NTDT.Objectives: To study the clinico -hematological profile and management of Non Transfusion Dependent Thalassemia (NTDT)Results: Total 21 children of Non Transfusion Dependent Thalassemia (receiving