Slovenský hematolologický portál - Hereditárna sférocytóza

Guidelines for the diagnosis and management of hereditary spherocytosis

P. H. B. Bolton-Maggs 1, R. F. Stevens 2, N. J. Dodd 3, G. Lamont 4, P. Tittensor5and M.-J. King 6 on behalf of the General Haematology Task Force of the British Committee for Standards in Haematology

1 Manchester Royal Infirmary, Manchester
2 Royal Manchester Children's Hospital, Manchester
3 The Ipswich Hospital NHS Trust Ipswich
4 Royal Liverpool Children's Hospital, Alder Hey, Liverpool
5 Patient Representative, and
6 International Blood Group Reference Laboratory, Bristol, UK

British Journal of Haematology 2004, 126, 455-474

Hereditary spherocytosis (HS) was described in 1871 and the first recorded splenectomy was performed soon after. It is the commonest cause of inherited chronic haemolysis in Northern Europe and North America with a quoted incidence of 1 in 5000 births
Hereditary spherocytosis is a heterogeneous group of disorders with regard to clinical severity, protein defects and mode of inheritance. It is relatively common in Caucasian populations; most affected individuals have mild or only moderate haemolysis.

Keywords: spherocytosis, hereditary, splenectomy, child, erythrocyte membrane

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