Hereditary spherocytosis and allied disorders

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چکیده

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Hereditary spherocytosis.

Hereditary spherocytosis is a common inherited disorder that is characterised by anaemia, jaundice, and splenomegaly. It is reported worldwide and is the most common inherited anaemia in individuals of northern European ancestry. Clinical severity is variable with most patients having a well-compensated haemolytic anaemia. Some individuals are asymptomatic, whereas others have severe haemolytic...

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Hereditary spherocytosis.

Hereditary spherocytosis is a congenital haemolytic anaemia due to defect in spectrin-a RBC membrane protein and is transmitted as autosomal dominant. Due to this defect there is presence of characteristic spherical cell in peripheral blood smear and osmotic fragility is increased. Haemolytic anaemia, reticulocytosis, jaundice and splenomegaly are present. This article reports a case of a 9 yea...

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Hereditary Spherocytosis

A 12-year-old girl was brought to the Dhaka Hospital of ICDDR,B with diarrhoea. Incidentally, the parents provided a history of repeated episodes of pallor and jaundice since she was two and half years old. Three of her family members had similar problems. History, clinical examination, and laboratory findings of the girl and her family members suggested a case of hereditary spherocytosis. To o...

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Hereditary spherocytosis, elliptocytosis, and other red cell membrane disorders.

Hereditary spherocytosis and elliptocytosis are the two most common inherited red cell membrane disorders resulting from mutations in genes encoding various red cell membrane and skeletal proteins. Red cell membrane, a composite structure composed of lipid bilayer linked to spectrin-based membrane skeleton is responsible for the unique features of flexibility and mechanical stability of the cel...

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Occult hereditary spherocytosis and human parvovirus infection.

The human parvovirus (HPV) is believed to be the causative agent of erythema infectiosum, or fifth disease. 1 Transient aplastic crisis linked to HPV infection has been described in several types of hemolytic anemias, congenital or acquired, such as sickle cell disease, thalassemia, autoimmune hemolytic anemias, and hereditary spherocytosis.2-8 In some instances, the underlying hemolytic anemia...

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ژورنال

عنوان ژورنال: HemaSphere

سال: 2019

ISSN: 2572-9241

DOI: 10.1097/hs9.0000000000000198