Beta talasemi hastalarında immatür granülosit sayısı kemik iliği aktivasyonunu değerlendirmede pratik bir belirteç olabilir mi?

Year 2022, Volume: 15 Issue: 2, 320 - 327, 31.08.2022

Banu İnce Feryal Karahan Aydan Akdeniz Selma Ünal

https://doi.org/10.26559/mersinsbd.1111602

Abstract

Amaç: Çalışmamızda çocuk ve erişkin beta talasemi major ve intermedia hastalarımızın immatür granülosit ve immatür granülosit yüzdelerinin kemik iliği aktivasyonlarını değerlendirmede pratik bir belirteç olarak kullanılıp kullanılamayacağını araştırmayı amaçladık. Yöntem: Ocak 2017-Aralık 2021 tarihleri arasında Mersin Üniversitesi Tıp Fakültesi Çocuk ve Erişkin Hematoloji kliniğinde takipli 38 Beta talasemi major ve 12 Beta talasemi intermedia hastasının yaş, hemoglobin (gr/dl), hematokrit, beyaz küre sayısı (x103/µL), trombosit değeri (x103/µL), immatür granülosit sayısı ( x103/µL) , immatür granülosit yüzdesi (%), ferritin değeri (ng/mL), şelasyon durumu, transfüzyon sıklığı ve düzeni, son bir yıldaki ortalama hemoglobin düzeyi gibi parametreleri incelendi. Düzenli transfüzyon alan ve almayan hastalar immatür granülosit sayısı, yüzdesi ve diğer parametreler açısından karşılaştırıldı. Bulgular: Hastalar çocuk ve erişkin olarak gruplandığında; çocuk hastalarda son bir yıldaki hemoglobin düzeyi daha yüksek iken erişkinlerde beyaz kan hücresi, immatür granülosit ve trombosit sayısının anlamlı olarak daha yüksek olduğu görüldü (p<0.05). Çocuk hastaların daha düzenli transfüzyon aldığı gözlendi. Düzensiz transfüzyon alan hastalarda son bir yıldaki ortalama hemoglobin düzeyinin anlamlı oranda daha düşük ancak immatür granülosit düzeyi, trombosit ve beyaz küre sayısının daha yüksek olduğu görüldü (p<0.05). Son bir yıldaki ortalama hemoglobin düzeyi arttıkça ortalama immatür granülosit sayısı ve yüzdesinin azaldığı saptandı. Sonuç: Çalışmamızda immatür granülosit sayısı ve yüzdesi, total beyaz küre ve trombosit sayısının düzensiz transfüzyon alan hastalarda istatistiksel açıdan anlamlı olacak şekilde yüksek olması ve son bir yıldaki ortalama hemoglobin düzeyi azaldıkça immatür granülosit ve immatür granülosit yüzdesinin artması bu hastalarda kemik iliğindeki hematopoezin aktif olduğunu düşündürdü. Bu nedenle beta talasemili hastalarda inflamatuvar aktivitenin derecesini ve transfüzyon etkinliğini değerlendirmede hemen her merkezde bakılabilen immatür granülosit ve immatür granülosit yüzde parametrelerinin kullanılabileceğini düşünmekteyiz.  

Keywords

Beta talasemi, inflamasyon, immatür granülosit, nötrofil

Can immature granulocyte count be a practical marker for evaluating bone marrow activation in beta thalassemia patients?

Abstract

Aim: In our study, we aimed to investigate whether the immature granulocyte and immature granulocyte percentages of our pediatric and adult beta thalassemia major and intermedia patients could be used as a practical marker in evaluating bone marrow activation. Method: Between January 2017 and December 2021, 38 Beta thalassemia major and 12 Beta thalassemia intermedia patients who applied to Mersin University Faculty of Medicine Pediatric and Adult Hematology Department were included in the study, hemoglobin (gr/dl), hematocrit, white blood cell count (x103/µL), platelet count (x103/µL), immature granulocyte count (x103/µL), immature granulocyte percentage, ferritin (ng/mL), chelation status, transfusion frequency and order, mean hemoglobin level in the last year were recorded. Patients who received and did not receive regular transfusion were compared in terms of immature granulocyte and other parameters. Results: When the patients were grouped as children and adults; mean hemoglobin levels in the last year were higher in children but white blood cell, immature granulocyte and platelet counts were significantly higher in adults (p<0.05). It was seen that those who received regular transfusion were mostly pediatric patients. The mean hemoglobin level in the last year was significantly lower in those who received irregular transfusion, but immature granulocyte level, platelet and white blood cell count were higher (p<0.05). It was observed that as the mean hemoglobin levels in the last year increased, the mean immature granulocyte count and immature granulocyte percentage decreased. Conclusion: In our study, immature granulocyte count and percentage, white blood cell and platelet count were found to be statistically significantly higher in patients with irregular transfusion and the increase in immature granulocyte and percentage parameters of immature granulocyte as the mean hemoglobin level in the last year decreased, suggesting that hematopoiesis in the bone marrow is active in these patients. We think that immature granulocyte and immature granulocyte percentage parameters, which can be measured in almost every center, can be used to evaluate the degree of inflammatory activity and transfusion efficiency in patients with beta thalassemia.

Keywords

Beta thalassemia, inflammation, immature granulocyte, neutrophil

References

• Taher AA, Khaled M, Musallam K, Cappellini MD. β-Thalassemias: Review article. N. Engl. J. Med. 2021; 384(8): 727-743
• Quintiliani L, Mastromonaco A, Giuliani E, et al. Immune profile alterations in thalassaemic patients. Boll Ist Sieroter Milan. 1983; 62(6): 524–530.
• Dwyer J, Wood C, McNamara J, et al. Abnormalities in the immune system of children with beta-thalassaemia major. Clin Exp Immunol. 1987; 68(3): 621–629.
• Khalifa AS, Maged Z, Khalil R, et al. T Cell functions in infants and children with beta-thalassemia. Acta Haematol. 1988; 79(3): 153–156.
• Vento S, Cainelli F, Cesario F. Infections and thalassaemia. Lancet Infect Dis. 2006; 6(4): 226–233.
• May JE, Marques MB, Reddy VV, Gangaraju R. Three neglected numbers in the CBC: The RDW, MPV, and NRBC count. Cleve Clin J Med. 2019; 86(3) :167-172.
• Orfanu AE, Popescu C, Leuștean A, et al. The Importance of haemogram parameters in the diagnosis and prognosis of septic patients. J Crit Care Med. 2017; 3(3): 105-110.
• Danise P, Maconi M, Barrella F,et al. Evaluation of nucleated red blood cells in the peripheral blood of hematological diseases. Clin Chem Lab Med. 2012; 50(2) :357–360.
• Park JS, Kim JS, Kim YJ, Kim WY. Utility of the immature granulocyte percentage for diagnosing acute appendicitis among clinically suspected appendicitis in adult. J Clin Lab Anal. 2018; 32(7):e22458.
• Narcı H, Berkeşoğlu M, Üçbilek E, Ayrık C. The usefulness of the percentage of immature granulocytes in predicting in-hospital mortality in patients with upper gastrointestinal bleeding. Am J Emerg Med. 2021; 46: 646-650.
• Güngör A, Göktuğ A, Tekeli A, et al. Evaluation of the accuracy of immature granulocyte percentage in predicting pediatric serious bacterial infection. Int J Lab Hematol. 2021; 43(4): 632-637.
• Karahan F, Ünal S, Topçu DB, Öztaş Y, Bozlu G. The role of immature granulocyte percentage in predicting acute chest syndrome and the severity of the vaso-occlusive crisis in sickle cell disease. The Turk J Pediat.2022; 64(1): 92-97.
• Rahav G, Volach V, Shapiro M, et al. Severe infections in thalassaemic patients: Prevalence and predisposing factors. Br J Haematol. 2006; 133(6): 667–674.
• Ricerca BM, Girolamo A, Rund D. Infections in thalassemia and hemoglobinopathies: Focus on therapy-related complications. Mediterr J Hematol Infect Dis. 2009;1(1): e2009028.
• Skoutelis AT, Lianou E, Papavassiliou T, et al. Defective phagocytic and bactericidal function of polymorphonuclear leucocytes in patients with β-thalassaemia major. J Inf Secur. 1984; 8(2):118–122.
• Matzner Y, Goldfarb A, Abrahamov A, et al. Impaired neutrophil chemotaxis in patients with thalassaemia major. Br J Haematol. 1993; 85(1) :153–158.
• Pillay J, Ramakers BP, Kamp VM, et al. Functional heterogeneity and differential priming of circulating neutrophils in human experimental endotoxemia. J Leukoc Biol. 2010; 88(1) :211–220.
• Leliefeld PHC, Pillay J, Vrisekoop N, et al. Differential antibacterial control by neutrophil subsets. Blood Adv. 2018; 2(11): 1344–1354.
• Fibach E, Dana M. Oxidative stress in β Thalassemia. Molecular Diagnosis & Therapy. 2019; 23:245–261.
• Siwaponanan P, Siegers J.Y, Ghazali R, et al. Reduced PU.1 expression underlies aberrant neutrophil maturation and function in β-thalassemia mice and patients. Blood. 2017; 129(23): 3087-3099.
• Dröge W. Free radicals in the physiological control of cell function. Physiol Rev. 2002; 82(1) :47–95.
• Buttari B, Profumo E, Caprari P, et al. Phenotypical and functional abnormalities of circulating neutrophils in patients with β-thalassemia. Annals of Hematology. 2020; 99(10): 2265–2277.
• Wessling RM. Iron homeostasis and the inflammatory response. Annu Rev Nutr. 2010; 30:105-122.
• Hershko C. Iron loading and its clinical implications. Am J Hematol. 2007; 82(S12): 1147–1148.