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Platelet distribution width (PDW) is increased in vaso-occlusive crisis in sickle cell disease

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Abstract

Considering the multigenic and multifactorial nature of the disease, we argue that a generalized bone marrow hyperplasia—and not merely erythroid hyperplasia—will occur in sickle cell disease. Consequently, we expect the hematological parameters to depict erythroid, myeloid as well as megakaryocyte hyperplasia. In the light of this expectation, we hypothesized that platelet distribution width (PDW) will increase in sickle cell disease. Here, we report the results from a cross-sectional study of 216 children admitted with complaints suggestive of vaso-occlusive crisis. We observed a strong association between PDW and sickle cell disease as compared to children who had HbAA genotype. Our findings bridge previous inconsistencies relating to the role of platelets in sickle cell disease. Implications of this finding are discussed.

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References

  1. Freedman ML, Karpatkin S (1975) Elevated platelet count and megathrombocyte number in sickle cell anemia. Blood 46:579–582

    CAS  PubMed  Google Scholar 

  2. Bessman JD, Gilmer PR, Gardner FH (1985) Use of mean platelet volume improves detection of platelet disorders. Blood Cells 11:127–135

    CAS  PubMed  Google Scholar 

  3. Croizat H, Ponchio L, Nicolini FE, Nagel RL, Eaves CJ (2000) Primitive haematopoietic progenitors in the blood of patients with sickle cell disease appear to be endogenously mobilized. Br J Haematol 111:491–497

    Article  CAS  PubMed  Google Scholar 

  4. Blouin M-J, De Paepe ME, Trudel M (1999) Altered hematopoiesis in murine sickle cell disease. Blood 94:1451–1459

    Google Scholar 

  5. Wun T, Paglieroni T, Tablin F, Welborn J, Nelson K, Cheung A (1997) Platelet activation and platelet-erythrocyte aggregates in patients with sickle cell anemia. J Lab Clin Med 129:507–516

    CAS  PubMed  Google Scholar 

  6. Wun T, Paglieroni T, Field CL, Welborn J, Cheung A, Walker NJ, Tablin F (1999) Platelet-erythrocyte adhesion in sickle cell disease. J Investig Med 47:121–127

    Google Scholar 

  7. Antonucci R, Walker R, Herion J, Orringer E (1990) Enhancement of sickle erythrocyte adherence to endothelium by autologous platelets. Am J Hematol 34:44–48

    CAS  PubMed  Google Scholar 

  8. Beurling-Harbury C, Schade SG (1989) Platelet activation during pain crisis in sickle cell anemia patients. Am J Hematol 31:237–241

    CAS  PubMed  Google Scholar 

  9. Westwick J, Watson-Williams EJ, Krishnamurthi S, Marks G, Ellis V, Scully MF, White JM, Kakkar VV (1983) Platelet activation during steady state sickle cell disease. J Med 14:17–36

    CAS  PubMed  Google Scholar 

  10. Semple MJ, Al-Hasani SF, Kioy P, Savidge GF (1984) A double-blind trial of ticlopidine in sickle cell disease. Thromb Haemost 51:303–306

    Google Scholar 

  11. Greenberg J, Ohene-Frempong K, Halus J, Way C, Schwartz E (1983) Trial of low doses of aspirin as prophylaxis in sickle cell disease. J Pediatr 102:781–784

    CAS  PubMed  Google Scholar 

  12. Osselaer J-C, Jamart J, Scheiff J-M (1997) Platelet distribution width for differential diagnosis of thrombocytosis. Clin Chem 43:1072–1076

    CAS  PubMed  Google Scholar 

  13. DeMaeyer EM, Dallman P, Gurney JM, Hallberg L, Sood SK, Srikantia SG (1989) Preventing and controlling iron deficiency anemia through primary health care. World Health Organization, Geneva, pp 8–9

  14. Sherriff A, Emond A, Hawkins N, Golding J, ALSPAC Children in Focus Study Team (1999) Hemoglobin and ferritin concentration in children aged 12 and 18 months. Arch Dis Child 80:153–157

    CAS  PubMed  Google Scholar 

  15. Kapur D, Agarwal KN, Sharma S, Kela K, Kaur I (2002) Iron status of children aged 9–36 months in an urban slum Integrated Child Development Services Project in Delhi. Indian Pediatr 39:136–144

    PubMed  Google Scholar 

  16. Singhal A, Davies P, Sahota A, Thomas PW, Serjeant GR (1993) Resting metabolic rate in homozygous sickle cell disease. Am J Clin Nutr 57:32–34

    CAS  PubMed  Google Scholar 

  17. Cazzola M, Pootrakul P, Bergamaschi G, Huebers HA, Eng M, Finch CA (1987) Adequacy of iron supply for erythropoiesis: in vivo observations in humans. J Lab Clin Med 110:734–739

    CAS  PubMed  Google Scholar 

  18. Tabbara IA (1992) Hemolytic anemias. Diagnosis and management. Med Clin North Am 76:649–668

    Google Scholar 

  19. Rao KR, Patel AR, Honig GR, Vida LN, McGinnis PR (1983) Iron deficiency and sickle cell anemia. Arch Intern Med 143:1030–1032

    Article  CAS  PubMed  Google Scholar 

  20. Grossmann A, Maggio-Price L, Shiota FM, Liggitt DV (1993) Pathologic features associated with decreased longevity of mutant sphha/sphha mice with chronic hemolytic anemia: similarities to sequelae of sickle cell anemia in humans. Lab Anim Sci 43:217–221

    CAS  PubMed  Google Scholar 

  21. Farrugia A (1995) Platelet size analysis in the quality assurance of platelet concentrates for transfusion. Clin Lab Haematol 17:51–55

    CAS  PubMed  Google Scholar 

  22. Okpala I, Daniel Y, Haynes R, Odoemene D, Goldman J (2002) Relationship between the clinical manifestations of sickle cell disease and the expression of adhesion molecules on white blood cells. Eur J Haematol 69:135–144

    Article  CAS  PubMed  Google Scholar 

  23. Anyaegbu CC, Okpala IE, Aken’Ova AY, Salimonu LS (1998) Peripheral blood neutrophil count and candidacidal activity correlate with the clinical severity of sickle cell anaemia. Eur J Haematol 60:267–268

    CAS  PubMed  Google Scholar 

  24. Abboud MR, Laver J, Blau CA (1996) Elevation of neutrophil count after G-CSF therapy leads to vaso-occlusive crisis and acute chest syndrome in a patient with sickle cell anaemia. Blood 88:15b

    Google Scholar 

  25. Olatunji PO, Davies SC (2000) The predictive value of white cell count in assessing clinical severity of sickle cell anaemia in Afro-Caribbean patients. Afr J Med Med Sci 29:27–30

    CAS  PubMed  Google Scholar 

  26. Awogu AU (2000) Leucocyte counts in children with sickle cell anaemia usefulness of stable state values during infections. West Afr J Med 19:55–58

    CAS  PubMed  Google Scholar 

  27. Lopez BL, Griswold SK, Navek A, Urbanski L (1996) The complete blood count and reticulocyte count—are they necessary in the evaluation of acute vasoocclusive sickle-cell crisis? Acad Emerg Med 3:751–757

    CAS  PubMed  Google Scholar 

  28. Grant MM, Gil KM, Floyd MY, Abrams M (2000) Depression and functioning in relation to health are use in sickle cell disease. Ann Behav Med 22:149–157

    CAS  PubMed  Google Scholar 

  29. Midence K, McManus C, Fuggle P, Davies S (1996) Psychological adjustment and family functioning in a group of British children with sickle cell disease: preliminary empirical findings and a meta-analysis.Br J Clin Psychol 35:439–450

    PubMed  Google Scholar 

  30. Yang YM, Cepeda M, Price C, Shah A, Mankad V (1994) Depression in children and adolescents with sickle-cell disease. Arch Pediatr Adolesc Med 148:457–460

    CAS  PubMed  Google Scholar 

  31. Gaston M, Rosse WF (1982) The cooperative study of sickle cell disease: review of study design and objectives Am J Pediatr Hematol Oncol 4:197–201

    Google Scholar 

  32. Nagel RL, Steinberg MH (2001) Role of epistatic (modifier) genes in the modulation of the phenotypic diversity of sickle cell anemia. Pediatr Pathol Mol Med 20:123–136

    Article  CAS  PubMed  Google Scholar 

  33. Steinberg MH, Rodgers GP (2001) Pathophysiology of sickle cell disease: role of cellular and genetic modifiers. Semin Hematol 38:299–306

    Article  CAS  PubMed  Google Scholar 

  34. Nagel RL (2001) Pleiotropic and epistatic effects in sickle cell anemia. Curr Opin Hematol 8:105–110

    Article  CAS  PubMed  Google Scholar 

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Acknowledgements

We acknowledge the administrative support of Drs. N. S. Ingole, Additional Professor; S. M. Sharma, Professor and Head; N. M. Gangane, Additional Professor, A. S. Bhake, Additional Professor; A. D. Gupta, Reader and Anshu, Lecturer, Department of Pathology, the Mahatma Gandhi Institute of Medical Sciences, Sewagram, Wardha, India. We also acknowledge the support given by Dr. P. R. Narang, Dean, the Mahatma Gandhi Institute of Medical Sciences, Sewagram, Wardha, India.

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Correspondence to Hemant R. Kulkarni.

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Amin, M.A., Amin, A.P. & Kulkarni, H.R. Platelet distribution width (PDW) is increased in vaso-occlusive crisis in sickle cell disease. Ann Hematol 83, 331–335 (2004). https://doi.org/10.1007/s00277-003-0833-8

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  • DOI: https://doi.org/10.1007/s00277-003-0833-8

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