All Title Author
Keywords Abstract

Coagulation Changes in Children with Sickle Cell Anaemia during Painful Crises and Steady State at Federal Medical Centre Abeokuta, Nigeria

DOI: 10.4236/oalib.1105081, PP. 1-17

Subject Areas: Pediatrics

Keywords: Sickle Cell Anaemia, Hypercoagulability, D-Dimer, Prothrombin Fragment

Full-Text   Cite this paper   Add to My Lib


Background: The pathophysiology of vaso-occlusive crises in sickle cell anaemia (SCA) is multifactorial and hypercoagulability is believed to play a role. The association between hypercoagulabilty and vaso-occlusive disease has been extensively studied in adult SCA patients, there is however paucity of data on the subject regarding paediatric SCA. Objective: This study set out to determine the presence of hypercoagulable states specifically in paediatric SCA subjects through quantification of specific coagulation markers during painful crises and steady state. Methodology: The study was a hospital- based longitudinal study carried out between May and October 2015 at Federal Medical Center, Abeokuta, Nigeria. Fifty SCA subjects were consecutively recruited during painful crises and followed up into their respective steady states. Twenty-five subjects with HbAA phenotype served as controls. Assays of coagulation markers, D-dimer and prothrombin fragment (F1 2) were carried out by sandwich ELISA method using MyBiosource? D-dimer and F1 2 ELISA kits. Results: Mean D-dimer level was 7358 ± 4354.33 ng/ml in the SCA subjects during painful crises, 5509 ± 3506.2 ng/ml during steady state, and 800 ± 1874.14 ng/ml in HbAA controls. Mean (F1 2) level was 0.84 ± 0.43 nmol/l in the SCA subjects during painful crises, 0.64 ± 0.25 nmol/l during steady state, and 0.41 ± 0.28 nmol/l, in HbAA controls. The mean values of both coagulation markers assayed were significantly higher during painful crises than at steady state (P = 0.002), while steady state values were also significantly higher than that of haemoglobin AA individuals (P = 0.001). Conclusions: This study suggests the presence of hypercoagulable states in paediatric SCA during steady state which is exacerbated during painful crises. The clinical imports of this finding require further elucidation.

Cite this paper

Adebola, M. B. , Olanrewaju, D. M. , Ogundeyi, M. M. and Shonde-Adebola, K. B. (2018). Coagulation Changes in Children with Sickle Cell Anaemia during Painful Crises and Steady State at Federal Medical Centre Abeokuta, Nigeria. Open Access Library Journal, 5, e5081. doi:


[1]  Lal, A. and Vinchinsky, E. (2005) Sickle Cell Disease. In: Hoffbrand, A.V., Catovsky, D. and Tuddenham, E., Eds., Postgraduate Hematology, 5th Edition, Blackwell Publishing, USA, 104-108.
[2]  De Baun, M.R., Frei-Jones, M.J. and Vinchinsky, E.P. (2014) Hemoglobinopathies. In: Kliegman, R.M., Stanton, B.F., St Geme, J.W., Schor, N.F., Behrman, R.E., Eds., Nelson Textbook of Paediatrics, 20th Edition, Elsevier, Philadelphia, 2236-2245.
[3]  Corrina, M. (2006) Sickle Cell Disease. In: Arceci, R., Hann, I. and Smith, O., Eds., Pediatric Hematology, Blackwell Publishing Ltd., 213-230.
[4]  Beutler, E. (2010) Disorders of Hemoglobin Structue: Sickle Cell Anemia and Related Abnormalities. In: Kaushansky, K., Lichtman, M., Beutler, E., Kipps, T., Seligsohn, U., Prchal, J., Eds., Williams Hematology, McGraw-Hill Companies Inc.
[5]  Odievre, M., Verger, E., Silva-Pinto, A.C. and Elion, J. (2011) Pathophysiological Insights in Sickle Cell Disease. Indian Journal of Medical Research, 134, 532-537.
[6]  Ataga, K. (2012) Coagulation Activation in Sickle Cell Disease. Plos ONE, 7, 1-9.
[7]  Ataga, K.I. (2009) Hypercoagulability and Thrombotic Complications in Hemolytic Anemias. Haematologica, 94, 1481-1484.
[8]  Ataga, K.I. and Key, N.S. (2007) Hypercoagulability in Sickle Cell Disease: New Approaches to an Old Problem. ASH Education Program Book, 91-96.
[9]  Ataga, K.I., Cappellini, M.D. and Rachmilewitz, E.A. (2007) Thalassaemia and Sickle Cell Anaemia as Paradigms of Hypercoagulability. British Journal of Haematology, 139, 3-13.
[10]  Ataga, K.I. and Orringer, E.P. (2003) Hypercoagulability in Sickle Cell Disease: A Curious Paradox. The American Journal of Medicine, 115, 721-728.
[11]  WHO (2006) Sickle Cell Anaemia. 59th World Health Assembly: Report by the Secretariat; Provisional Agenda Item 114.
[12]  Devine, D.V., Kinney, T.R., Thomas, P.F., Rose, W.F. and Greenberg, C.S. (1986) Fragmenr D-Dimer Levels: An Objective Marker of Vaso-Occlusive Crises and other Complications of Sickle Cell Disease. Blood, 68, 317-319.
[13]  Fakunle, E., Eteng, K. and Shokunbi, W. (2012) D-Dimer Levels in Patients with Sickle Cell Disease during Bone Pain Crises and in Steady State. Pathology and Laboratory Medicine International, 2, 21-25.
[14]  Francis Jr., R. (1989) Elevated Fibrin D-Dimer Fragment in Sickle Cell Anemia: Evidence for Activation of Coagulation during the Steady State as Well as in Painful Crisis. Pathophysiology of Haemostasis Thrombosis, 19, 105-111.
[15]  Peters, M., Plaat, B.E., Ten Cate, H., et al. (1994) Enhanced Thrombin Generation in Children with Sickle Cell Disease. Thrombosis and Haemostasis, 71, 169-172.
[16]  Bauer, K.A., Weiss, L.M., Sparrow, D., Vokonas, P.S. and Rosenberg, R.D. (1987) Aging-Associated Changes in Indices of Thrombin Generation and Protein C Activation in Humans. Journal of Clinical Investigation, 80, 1527-1534.
[17]  Cushman, M., Psaty, B., Macy, E., et al. (1996) Correlates of Thrombin Markers in an Elderly Cohort Free of Clinical Cardiovascular Disease. Arteriosclerosis, Throm- bosis, and Vascular Biology, 16, 1163-1169.
[18]  Hursting, M.J., Stead, A.G., Crout, F.V., Horvath, B.Z. and Moore, B.M. (1993) Effects of Age, Race, Sex and Smoking on Prothrombin Fragment 1.2 in a Healthy Population. Clinical Chemistry, 39, 683-686.
[19]  Chinawa, J.M., Emodi, I.J., Ikefuna, A.N. and Ocheni, S. (2013) Coagulation Profiles of Children with Sickle Cell Anaemia in Steady State and Crisis Attending University of Nigeria Teaching Hospital Ituku-Ozalla, Enugu. Nigerian Journal of Clinical Practice, 16, 159-163.
[20]  Hart, R.G. and Kanter, M.C. (1990) Hematologic Disorders and Ischemic Stroke. A Selective Review. Stroke, 21, 1111-1121.
[21]  Taiwo, I.A., Oloyede, O.A. and Dosumu, A.O. (2011) Frequency of Sickle Cell Genotype among Yorubas in Lagos: Implication of Level of Awareness and Genetic Counseling for Sickle Cell Dis-ease in Nigeria. Journal of Community Genetics, 2, 13-18.
[22]  Lande, W.M., Andrews, D.L., Clark, M.R., et al. (1988) The Incidence of Painful Crisis in Homozygous Sickle Cell Disease: Correlation with Red Cell Deformability. Blood, 72, 2056-2059.
[23]  Araoye, M. (2004) Subjects Selection. In: Research Methodology with Statistics for Health and Social Sciences, Nathadex Publishers, Ilorin, 115-121.
[24]  Pain Scales for Children.
[25]  (2015) Mybiosource.
[26]  (2015) Mybiosource.
[27]  (2015).
g/Clinical and Interpretive/9290
[28]  Ota, S., Wada, H., Abe, Y. and Yamada, E. (2008) Elevated Levels of Prothrombin Fragment Indicate High Risk of Thrombosis. Clinical and Applied Thrombosis/ Hemostasis, 14, 279-285.
[29]  Tampi, R. (2016) Preanalytical and Analytical Variables and Coagulation Tests. Clinipath Pathology.
[30]  Soheir, S.A., Nigel, S.K. and Charles, S.G. (2009) D-Dimer Antigen: Current Concepts and Future Prospects. Blood, 13, 2878-2887.
[31]  Akinbami, A., Dosunmu, A., et al. (2012) Haematological Values in Homozygous Sickle Cell Disease in Steady State and Haemoglobin Phenotypes AA Controls in Lagos, Nigeria. BMC Research Notes, 5, 396.
[32]  Scott, J.P. (2016) Platelet and Blood Vessel Disorders. In: Kliegman, R.M., Stanton, B.F., St Geme, J.W., Schor, N.F. and Behrman, R.E., Eds., Nelson Textbook of Pediatrics, 20th Edition, Elsevier, Philadelphia, 3604.
[33]  Francis, R.B. (1991) Platelets, Coagulation and Fibrinolysis in Sickle Cell Disease: Their Possible Role in Vascular Occlusion. Blood Coagulation & Fibrinolysis, 2, 341-354.
[34]  Chaplin Jr., H., Monroe, M., Malecek, A., Morgan, L., Michael, J. and Murphy, W. (1989) Preliminary Trial of Minidose Heparin Prophylaxis for Painful Sickle Cell Crises. East African Medical Journal, 66, 574-584.
[35]  Schnog, J.B., Kater, A.P., MacGillavry, M.R., et al. (2001) Low Adjusted-Dose Acenocoumarol Therapy in Sickle Cell Disease: A Pilot Study. American Journal of Hematology, 68, 179-183.


comments powered by Disqus