Current problems in haematology. 2: Hereditary spherocytosis.
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چکیده
منابع مشابه
Current problems in haematology. 2: Hereditary spherocytosis.
Hereditary spherocytosis is a relatively common haematological disorder and will be encountered by all haematologists. The abundance of new information, dealing principally with molecular and genetic aspects of pathophysiology, is beginning to have implications for its investigation and management. While these advances have not yet exerted a large influence at therapeutic level, the promise of ...
متن کاملHereditary 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...
متن کاملHereditary 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...
متن کاملHereditary 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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Probable manifestations of the intrinsic corpuscular defect leading to shortened red blood cell (RBC) survival in hereditary spherocytosis (HS) have been induced by in vitro incubation of these erythrocytes (2-4). The classical in vitro changes that have been described are a marked increase in autohemolysis in the absence of metabolized substrate and an increase in osmotic fragility. Young, Izz...
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ژورنال
عنوان ژورنال: Journal of Clinical Pathology
سال: 1991
ISSN: 0021-9746
DOI: 10.1136/jcp.44.6.441