Thalassaemia

Thalassaemias are among the most common genetic disorders worldwide; an estimated 1.5% of the global population carries a thalassaemia mutation. Originally associated with tropical and sub-tropical regions, migration has facilitated wider dispersal, concentrated in larger multi-ethnic conurbations. Thalassaemias are heterogeneous, ranging from asymptomatic to causing severe haemolytic anaemia with pan-corporal sequelae and complications. Carriers benefit from access to pre-conception and antenatal advice and screening. The National Health Service Antenatal Haemoglobinopathy Screening Programme has operated since 2001, but carrier diagnoses may still emerge incidentally. This article provides an overview of the genetics, presentation and management of thalassaemia.

Role of Surgical Intervention in Hereditary Spherocytosis with Haemolytic Anaemia, Splenomegaly and Jaundice

haemolytic anaemia, splenomegaly and jaundice, admitted in Chettinad hospital and research institute during one year period from January 2019 to January 2020. Surgical intervention is indicated for selected patients with hereditary spherocytosis with haemolytic anaemia and jaundice to abate the hemolytic process after correction of anaemia with blood transfusion. Pigmented gallstones are seen in more than 50% cases for which incidence increases with severity of hemolysis and with age. Complications include aplastic anemia (most common after parvovirus B19 infection), haemolytic crisis during inter-current infection, megaloblastic crisis – during folic acid deficiency, cardiomyopathy, hematological malignancies.

Efficacy of Whole Blood Transfusion as an Adjunct Therapy in Severe Cases of Haemolytic Anaemia in Cattle

Background: In view of common occurrence of haemoprotozoan infections leading to severe haemolytic anaemia and increased case fatality rate in cattle in study area the present investigation was planned to evaluate efficacy of whole blood transfusion as an adjunct therapy in severe cases of hemolytic anaemia in cattle. Method: Sixteen cattle suffering from one or other haemoprotozoan infections induced haemolytic anaemia and having haemoglobin values less than 5 gm/dl were randomly divided in to two treatment groups. Group A (n=8) cattle were treated with standard treatment of haemoprotozoan infection without blood transfusion while group B (n=8) cattle were treated with whole blood transfusion in addition to standard treatment for haemoprotozoan infections. Result: Clinical and haematological parameters revealed rapid improvement in group B cattle as compared to group A cattle. The survival rate was significantly higher in group B cattle (100%) as compared to group A cattle (62.5%). The haemoglobin and haematocrit values were also found significantly higher in group B as compared to group A at 12 hours as well as on 21st day post treatment. Thus, it is concluded that, whole blood transfusion could be used as an adjunct therapy to hasten clinical recovery and to increase the survivability in cattle suffering from severe haemolytic anaemia due to haemoprotozoan infections.

Primary Evans syndrome in an adult man

Evans syndrome (ES) is a simultaneous or subsequent development of two haematological disorders, autoimmune haemolytic anaemia (AIHA) and immune thrombocytopenia (ITP). It can be primary (idiopathic) or secondary (associated with an underlying disease). Primary Evans is a diagnosis of exclusion and has a poorer prognosis than AIHA or ITP alone. We present a 55-year-old man who presented with weakness and lethargy and was diagnosed to be suffering from primary ES.