Increased vasoocclusive crises in "O" blood group sickle cell disease patients

Association with underlying thrombospondin levels

M. Al Huneini, S. Alkindi, V. Panjwani, K. Al Falahi, B. Al Balushi, D. Gravel, C. H. Ho, R. Krishnamoorthy, A. V. Pathare

Research output: Contribution to journalArticle

Abstract

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.

Original languageEnglish
Article numbere2017028
JournalMediterranean Journal of Hematology and Infectious Diseases
Volume9
Issue number1
DOIs
Publication statusPublished - 2017

Fingerprint

Thrombospondins
Sickle Cell Anemia
Blood Group Antigens
Thrombospondin 1
Serum
Blood Grouping and Crossmatching
Ristocetin
Blood Cell Count
Factor VIII
C-Reactive Protein
Oxidoreductases
Milk
Up-Regulation
Collagen
High Pressure Liquid Chromatography
Kidney
Antigens
thrombospondin 2
Liver
Incidence

Keywords

  • Blood groups
  • SCD
  • TSP 1
  • TSP 2
  • VOC
  • vWD

ASJC Scopus subject areas

  • Hematology
  • Infectious Diseases

Cite this

Increased vasoocclusive crises in "O" blood group sickle cell disease patients : Association with underlying thrombospondin levels. / Al Huneini, M.; Alkindi, S.; Panjwani, V.; Al Falahi, K.; Al Balushi, B.; Gravel, D.; Ho, C. H.; Krishnamoorthy, R.; Pathare, A. V.

In: Mediterranean Journal of Hematology and Infectious Diseases, Vol. 9, No. 1, e2017028, 2017.

Research output: Contribution to journalArticle

@article{5f72be84759b4b5ca327a64930d42592,
title = "Increased vasoocclusive crises in {"}O{"} blood group sickle cell disease patients: Association with underlying thrombospondin levels",
abstract = "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.",
keywords = "Blood groups, SCD, TSP 1, TSP 2, VOC, vWD",
author = "{Al Huneini}, M. and S. Alkindi and V. Panjwani and {Al Falahi}, K. and {Al Balushi}, B. and D. Gravel and Ho, {C. H.} and R. Krishnamoorthy and Pathare, {A. V.}",
year = "2017",
doi = "10.4084/MJHID.2017.028",
language = "English",
volume = "9",
journal = "Mediterranean Journal of Hematology and Infectious Diseases",
issn = "2035-3006",
publisher = "Universita Cattolica del Sacro Cuore",
number = "1",

}

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

UR - http://www.scopus.com/inward/record.url?scp=85019052463&partnerID=8YFLogxK

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

VL - 9

JO - Mediterranean Journal of Hematology and Infectious Diseases

JF - Mediterranean Journal of Hematology and Infectious Diseases

SN - 2035-3006

IS - 1

M1 - e2017028

ER -