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Correlation between the plasma level of von willebrand factor and the severity of sickle cell disease
Author(s) -
Mohamed Abdelrahim Osman,
Omer Nawal Eltayeb,
Satti Maria M.H
Publication year - 2007
Publication title -
the faseb journal
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.709
H-Index - 277
eISSN - 1530-6860
pISSN - 0892-6638
DOI - 10.1096/fasebj.21.5.a671
Subject(s) - von willebrand factor , medicine , gastroenterology , disease , hemolytic anemia , acute chest syndrome , sickle cell anemia , immunology , cardiology , platelet
Sickle cell anaemia is a congenital hemolytic disorder caused by mutation in the β‐globin gene at position 6 with replacement of glutamic acid by valine. Patients who are homozygous for this mutation suffer from hemolytic anaemia and other serious complications. The underline pathology of much of these complications is the occurrence of recurrent vasoocclusion due to microthrombi formation resulting in organs ischemia. In this study we investigated the role of vWF as a determinant of sickle cell disease severity through its contribution to the formation of such microthrombi. Seventy Sudanese patients were investigated in this study. They were 35 females and 35 males; the mean of their ages was 6.8 ± 4.7 years. Thirty four patients presented during vasoocclusive crisis and 36 presented in steady state. The clinical disease severity was determined using two different scoring methods, and vWF antigen level in the plasma was estimated by using ELISA technique. Statistical analysis showed positive correlation between severity score and vWF level in the plasma in steady state in the two scoring methods used (r = 0.79, p = 0.008 for method I and r = 0.78, p = 0.009 for method II). So, Severity of sickle cell disease increases with elevation of vWF level in the plasma in the steady state.