Investigation of the Relationship between Platelet, Mean Platelet Volume and White Blood Cell Values and Clinical Complications in Patients with Sickle Cell Anemia

Öz

This study examines the relationship between sickle cell anemia (SCA) and hematological parameters and organ damage. SCA is a disease that can lead to serious complications in various organs such as neurological, pulmonary, reproductive, kidney, liver and eye due to structural disorders of blood cells. In the study, 48 patients diagnosed with SCA, and 48 healthy controls were compared, and white blood cell (WBC), platelet (PLT) and mean platelet volume (MPV) were evaluated. The indicated hemogram values were compared with the cases that developed and did not develop SCA complications such as cerebrovascular accident (CVA), avascular necrosis (AVN), retinopathy, hepatopathy, nephropathy, priapism and pulmonary hypertension (PH). SPSS was used for statistical analyses and statistical significance was accepted as p<0.05 for all data. When healthy group and the SCA group were compared, WBC and PLT increased, while MPV values decreased. When SCD patients were compared with those with and without complications, it was found that only cases with hepatopathy had higher WBC values compared to those without complications. No significant difference was found between the presence or absence of complications in all other hemogram values. Increases in WBC and PLT levels in SCD patients were found to have a strong relationship with vaso-occlusive events, inflammation, and organ damage, but no similar relationship was found for mean platelet volume (MPV). These findings emphasize the importance of hematological markers in the management of disease severity and complications, and indicate that the conflicting literature, especially regarding MPV, should be clarified with more comprehensive studies.

Anahtar Kelimeler

• Sickle cell anemia
• Cerebrovascular accident
• Avascular necrosis
• Retinopathy
• Hepatopathy
• Nephropathy
• Priapism and Pulmonary hypertension

Orak hücreli anemi Hastalarında Trombosit, Ortalama Trombosit Hacmi ve Beyaz Kan Hücresi Değerlerinin Klinik Komplikasyonlar ile İlişkisinin Araştırılması

Öz

Bu çalışma orak hücreli aneminin (OHA) hematolojik parametreler ve organ hasarıyla olan ilişkisini incelemektedir. OHA, kan hücrelerinin yapısal bozukluğu nedeni ile nörolojik, pulmoner, üreme, böbrek, karaciğer ve göz gibi çeşitli organlarda ciddi komplikasyonlara yol açabilen bir hastalıktır. Çalışmada, OHA tanısı almış 48 hasta ve 48 sağlıklı kontrol grubu karşılaştırılmış, beyaz kan hücresi (WBC), trombosit (PLT) ve ortalama trombosit hacmi (MPV) değerlendirilmiştir. Belirtilen hemogram değerleri ile OHA komplikasyonlarından olan serebrovasküler olay (SVO), avasküler nekroz (AVN), retinopati, hepatopati, nefropati, priapism ve pulmoner hipertansiyon (PH) gelişen ve gelişmeyen olgular karşılaştırılmıştır. İstatistiksel analizler SPSS kullanıldı ve tüm verilerde istatistiksel anlamlılık p<0,05 olarak kabul edildi. Sağlıklı grup ile OHA grup karşılaştırıldığında WBC ve PLT artarken, MPV değeri azalmıştır. OHA hastaları arasında komplikasyonları olanlar ve olmayanlar karşılaştırıldığında sadece hepatopatisi olan olgularda komplikasyonu olmayanlara kıyasla WBC değerinin daha yüksek olduğu bulunmuştur. Diğer tüm hemogram değerlerinde komplikasyon olup olmaması arasında anlamlı bir fark bulunamamıştır. OHA hastalarında WBC ve PLT düzeylerindeki artışın, vazo-oklüzif olaylar, inflamasyon ve organ hasarı ile güçlü bir ilişki taşıdığı görülmüş, ancak ortalama trombosit hacmi MPV için benzer bir bağlantı tespit edilememiştir. Bu bulgular, hematolojik belirteçlerin hastalığın şiddeti ve komplikasyonların yönetimindeki önemini vurgulamakta olup, özellikle MPV ile ilgili çelişkili literatürün daha kapsamlı çalışmalarla netleştirilmesi gerektiğini göstermektedir.

Anahtar Kelimeler

• Orak hücreli anemi
• Serebrovasküler olay
• Avasküler nekroz
• Retinopati
• Hepatopati
• Nefropati
• Priapism ve Pulmoner hipertansiyon

Kaynakça

1. 1. Stuart MJ, Nagel RL. Sickle-cell disease. In: Lancet. Elsevier B.V.; 2004. p. 1343–60.
2. 2. Morris CR. Mechanisms of Vasculopathy in Sickle Cell Disease and Thalassemia [Internet]. Hematology. 2008. Available from: http://ashpublications.org/hematology/article-pdf/2008/1/177/786434/177_185ash.pdf
3. 3. Kasar M, Boğa C, Yeral M, Asma S, Kozanoglu I, Ozdogu H. Clinical significance of circulating blood and endothelial cell microparticles in sickle-cell disease. J Thromb Thrombolysis. 2014;38(2):167–75.
4. 4. Lliott E, Ichinsky P V, Eumayr YDN, Arles NNE, Oger R, Illiams W, et al. Volume 342 Number 25 · 1855 causes and outcomes of the acute chest syndrome ın sıckle cell dısease causes and outcomes of the acute chest syndrome ın sıckle cell dısease. 2000.
5. 5. Rees DC, Williams TN, Gladwin MT. Sickle-cell disease. www.thelancet.com [Internet]. 2018;376:2018–49. Available from: www.thelancet.com
6. 6. Ware RE, Rees RC, Sarnaik SA, Iyer R V., Alvarez OA, Casella JF, et al. Renal Function in Infants with Sickle Cell Anemia: Baseline Data from the BABY HUG Trial. Journal of Pediatrics. 2010;156(1).
7. 7. Liu FF, Tu TT, Zhang HF, Hu F, Huang L, Deng LF, et al. Coexpression network analysis of platelet genes in sickle cell disease. Platelets. 2019 Nov 17;30(8):1022–9.
8. 8. Abdalla Elsayed MEA, Mura M, Dhibi H Al, Schellini S, Malik R, Kozak I, et al. Sickle cell retinopathy. A focused review. Vol. 257, Graefe’s Archive for Clinical and Experimental Ophthalmology. Springer Science and Business Media Deutschland GmbH; 2019. p. 1353–64.

9. 9. Celik T, Unal S, Ekinci O, Ozer C, Ilhan G, Oktay G, et al. Mean platelet volume can predict cerebrovascular events in patients with Sickle Cell Anemia. Pak J Med Sci. 2015 Jan 1;31(1):203–8.
10. 10. Choi S, Bush AM, Borzage MT, Joshi AA, Mack WJ, Coates TD, et al. Hemoglobin and mean platelet volume predicts diffuse T1-MRI white matter volume decrease in sickle cell disease patients. Neuroimage Clin. 2017;15:239–46.
11. 11. Antwi-Boasiako C, Ekem I, Abdul-Rahman M, Sey F, Doku A, Dzudzor B, et al. Hematological parameters in Ghanaian sickle cell disease patients. J Blood Med. 2018;9:203–9.
12. 12. Okpala I. The intriguing contribution of white blood cells to sickle cell disease - A red cell disorder. Vol. 18, Blood Reviews. Churchill Livingstone; 2004. p. 65–73.
13. 13. Freedman ML, Karpatkin S. Short Communication: Elevated Platelet Count and Megathrombocyte Number in Sickle Cell Anemia. Vol. 46, Blood. 1975.
14. 14. Silva CM, de Souza Medina S, de Melo Campos P, Costa FF, Saad STO, Benites BD. Platelet counts on peripheral blood and Mean Platelet Volume as markers of clinical severity in Sickle Cell Disease. Blood Cells Mol Dis. 2021 Nov 1;91.
15. 15. Krishnan S, Setty Y, Betal SG, Vijender V, Rao K, Dampier C, et al. Increased levels of the inflammatory biomarker C-reactive protein at baseline are associated with childhood sickle cell vasocclusive crises. Br J Haematol. 2010 Mar;148(5):797–804.
16. 16. Ahmed AE, Ali YZ, Al-Suliman AM, Albagshi JM, Salamah M Al, Elsayid M, et al. The prevalence of abnormal leukocyte count, and its predisposing factors, in patients with sickle cell disease in Saudi Arabia. J Blood Med. 2017 Oct 25;8:185–91.
17. 17. Bagul R, Chandan S, Sane VD, Patil S, Yadav D. Comparative Evaluation of C-Reactive Protein and WBC Count in Fascial Space Infections of Odontogenic Origin. J Maxillofac Oral Surg. 2017 Jun 1;16(2):238–42.
18. 18. Hendra TJ, Oswald GA, Yudkin JS. Increased mean platelet volume after acute myocardial infarction relates to diabetes and to cardiac failure. Vol. 5, Diahetes Research aml Clinical Practice. 1988.
19. 19. Shet AS, Hoffmann TJ, Jirouskova M, Janczak CA, Stevens JRM, Adamson A, et al. Morphological and functional platelet abnormalities in Berkeley sickle cell mice. Blood Cells Mol Dis. 2008 Jul;41(1):109–18.
20. 20. Mohan JS, Lip GYH, Bareford D, Blann AD. Platelet P-selectin and platelet mass, volume and component in sickle cell disease: Relationship to genotype. Thromb Res. 2006;117(6):623–9.
21. 21. Fonseca AC, Silva DP, Infante J, Ferro JM. Cerebrovascular Complications of Anemia. Vol. 21, Current Neurology and Neuroscience Reports. Springer; 2021.
22. 22. Frelinger AL, Jakubowski JA, Brooks JK, Carmichael SL, Berny-Lang MA, Barnard MR, et al. Platelet Activation and Inhibition in Sickle cell disease (PAINS) study. Platelets. 2014;25(1):27–35.
23. 23. Hamali HA. Hypercoagulability in Sickle Cell Disease: A Thrombo-Inflammatory Mechanism. Vol. 47, Hemoglobin. Taylor and Francis Ltd.; 2023. p. 205–14.
24. 24. Yang M, Pan Y, Li Z, Yan H, Zhao X, Liu L, et al. Platelet count predicts adverse clinical outcomes after ischemic stroke or TIA: Subgroup analysis of CNSR II. Front Neurol. 2019;10(APR).
25. 25. Shome D, Jaradat A, Mahozi A, Sinan A, Ebrahim A, Alrahim M, et al. The platelet count and its implications in sickle cell disease patients admitted for intensive care. Indian Journal of Critical Care Medicine. 2018 Aug 1;22(8):585–90.
26. 26. Leandro MP, Almeida ND, Hocevar LS, Sá CKC de, Souza AJ de, Matos MA. Polymorphisms and avascular necrosis in patients with sickle cell disease - A systematic review. Rev Paul Pediatr. 2022;40:e2021013.
27. 27. Bedair EMA, Almaslamani NJ, Yassin M. Radiological manifestation of avascular necrosis (AVN) in sickle cell disease (SCD): a review of diagnostic imaging. Vol. 94, Acta Biomedica. Mattioli 1885; 2023.
28. 28. Ribeiro MVMR, De Omena Jucá JV, Dos Santos Alves ALC, Ferreira CVO, Barbosa FT, Ribeiro ÊAN. Sickle cell retinopathy: A literature review. Vol. 63, Revista da Associacao Medica Brasileira. Associacao Medica Brasileira; 2017. p. 1100–3.
29. 29. Do BK, Rodger DC. Sickle cell disease and the eye. Vol. 28, Current Opinion in Ophthalmology. Lippincott Williams and Wilkins; 2017. p. 623–8.
30. 30. Hassan T, Badr M, Hanna D, Arafa M, Elhewala A, Dabour S, et al. Retinopathy in Egyptian patients with sickle cell disease: A cross-sectional study. Medicine (United States). 2021 Dec 23;100(51).
31. 31. Kinger NP, Moreno CC, Miller FH, Mittal PK. Abdominal manifestations of sickle cell disease. Current Problems in Diagnostic Radiology. 2021 April. P. 241-251.
32. 32. Lacaille F, Allali S, Montalembert M De. The liver in sickle cell disease. J Pediatr Gastroenterol Nutr. 2021 Jan 1;72(1):5–10.
33. 33. Duvoux C, Blaise L, Matimbo JJ, Mubenga F, Ngongang N, Hurtova M, et al. The liver in sickle cell disease. Vol. 52, Presse Medicale. Elsevier Masson s.r.l.; 2023.
34. 34. Ahn H, Li CS, Wang W. Sickle cell hepatopathy: Clinical presentation, treatment, and outcome in pediatric and adult patients. Pediatr Blood Cancer. 2005 Aug;45(2):184–90.
35. 35. Ataga KI, Saraf SL, Derebail VK. The nephropathy of sickle cell trait and sickle cell disease. Vol. 18, Nature Reviews Nephrology. Nature Research; 2022. p. 361–77.
36. 36. Aygun B, Mortier NA, Smeltzer MP, Shulkin BL, Hankins JS, Ware RE. Hydroxyurea treatment decreases glomerular hyperfiltration in children with sickle cell anemia. Am J Hematol. 2013 Feb;88(2):116–9.
37. 37. Efobi CC, Nri-Ezedi CA, Madu CS, Ikediashi CC, Ejiofor O, Ofiaeli CI. Neutrophil-Lymphocyte, Platelet-Neutrophil, and Platelet-Lymphocyte Ratios as Indicators of Sickle Cell Anaemia Severity. Ethiop J Health Sci. 2023 Sep 1;33(5):821–30.
38. 38. Olujohungbe A, Burnett AL. How I manage priapism due to sickle cell disease. Vol. 160, British Journal of Haematology. 2013. p. 754–65.
39. 39. Adawi EA, Ghanem MA. Platelet volume parameters as a tool in the evaluation of acute ischemic priapism in patients with sickle cell anemia. Archivio Italiano di Urologia e Andrologia. 2022 Jun 30;94(2):217–21.
40. 40. Ufuk Y, Hasan Y, Murat U, Seyfettin C, Kerem T, Melih C. Does platelet activity play a role in the pathogenesis of idiopathic ischemic priapism? International Braz J Urol. 2016 Jan 1;42(1):118–22.