TY - JOUR
T1 - Increased vasoocclusive crises in "O" blood group sickle cell disease patients
T2 - Association with underlying thrombospondin levels
AU - Al Huneini, M.
AU - Alkindi, S.
AU - Panjwani, V.
AU - Al Falahi, K.
AU - Al Balushi, B.
AU - Gravel, D.
AU - Ho, C. H.
AU - Krishnamoorthy, R.
AU - Pathare, A. V.
PY - 2017
Y1 - 2017
N2 - Objectives: To explore the incidence of vaso-occlusive crisis (VOC) in Blood Group "O" sickle cell disease (SCD) patients, and correlate it with the blood group and thrombospondin (TSP) levels. Methods: In 89 consecutive SCD patients, blood samples were obtained for von Williebrand factor (vWF:Ag) antigen, collagen binding activity (CBA), ristocetin binding activity (RCo), blood group typing, C-reactive protein (CRP), high performance liquid chromatography (HPLC), Serum TSP 1 and TSP 2 levels, complete blood counts (CBC), lactic dehydrogenase (LDH) levels, liver function (LFT) and renal function tests (RFT) during VOC episodes and in steady state conditions. Results: In steady state SCD patients (n=72), "O" blood group patients (n=37) showed a significantly higher median serum TSP 1 and TSP 2 levels as compared to non-O blood group patients [n=35] [p <0.05, Mann-Whitney test]; with an inverse relation between vWF:Ag, Factor VIII:C and TSP levels. Furthermore, the serum TSP 1 and TSP 2 levels were significantly higher in patients presenting with acute VOC [n=17], as well as in those with repeated VOC's (group 1, n=16), especially amongst blood group "O" patients [p, <0.05, Mann-Whitney test]. Conclusions: The study demonstrates an inverse relation between TSP and vWF levels, in blood group "O" SCD patients, with an upregulation of the TSP levels. Expectedly, during active VOC crisis, the TSP 1 and TSP 2 levels were significantly elevated.
AB - Objectives: To explore the incidence of vaso-occlusive crisis (VOC) in Blood Group "O" sickle cell disease (SCD) patients, and correlate it with the blood group and thrombospondin (TSP) levels. Methods: In 89 consecutive SCD patients, blood samples were obtained for von Williebrand factor (vWF:Ag) antigen, collagen binding activity (CBA), ristocetin binding activity (RCo), blood group typing, C-reactive protein (CRP), high performance liquid chromatography (HPLC), Serum TSP 1 and TSP 2 levels, complete blood counts (CBC), lactic dehydrogenase (LDH) levels, liver function (LFT) and renal function tests (RFT) during VOC episodes and in steady state conditions. Results: In steady state SCD patients (n=72), "O" blood group patients (n=37) showed a significantly higher median serum TSP 1 and TSP 2 levels as compared to non-O blood group patients [n=35] [p <0.05, Mann-Whitney test]; with an inverse relation between vWF:Ag, Factor VIII:C and TSP levels. Furthermore, the serum TSP 1 and TSP 2 levels were significantly higher in patients presenting with acute VOC [n=17], as well as in those with repeated VOC's (group 1, n=16), especially amongst blood group "O" patients [p, <0.05, Mann-Whitney test]. Conclusions: The study demonstrates an inverse relation between TSP and vWF levels, in blood group "O" SCD patients, with an upregulation of the TSP levels. Expectedly, during active VOC crisis, the TSP 1 and TSP 2 levels were significantly elevated.
KW - Blood groups
KW - SCD
KW - TSP 1
KW - TSP 2
KW - VOC
KW - vWD
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UR - http://www.scopus.com/inward/citedby.url?scp=85019052463&partnerID=8YFLogxK
U2 - 10.4084/MJHID.2017.028
DO - 10.4084/MJHID.2017.028
M3 - Article
C2 - 28512557
AN - SCOPUS:85019052463
SN - 2035-3006
VL - 9
JO - Mediterranean Journal of Hematology and Infectious Diseases
JF - Mediterranean Journal of Hematology and Infectious Diseases
IS - 1
M1 - e2017028
ER -