Orak Hücre Anemisi
Özet
Orak hücre anemisi, hemoglobinopatiler arasında genetik temeli ve sistemik etkileriyle öne çıkan, kronik hemolitik anemi ve tekrarlayan ağrılı krizlerle karakterize ciddi bir hastalıktır. OHA’lı hastalarda ateş ve enfeksiyonların yönetiminde, erken tanı ve enfeksiyonların hızlı tedavisi yaşamı tehdit eden komplikasyonları önlemede kritik öneme sahiptir. Hastaların bireyselleştirilmiş izlem ve tedavi planlarıyla multidisipliner yaklaşımlar altında takip edilmesi gerekliliği göz önünde bulundurulmalıdır. Bunun yanı sıra, ateşli hastalıkların değerlendirilmesinde kapsamlı anamnez, dikkatli fizik muayene ve uygun laboratuvar testlerinin önemi unutulmamalıdır. Klinisyenlerin akut komplikasyon risklerini belirleme ve zamanında müdahale etme yetkinliği hayati önem taşımaktadır. Bu bağlamda, hastalığın yönetiminde güncel tedavi protokollerinin ve koruyucu önlemlerin uygulanması önerilmektedir.
Referanslar
Herrick JB. Peculiar elongated and sickle-shaped red blood corpuscles in a case of severe anemia. 1910. Yale J Biol Med 2001;74(3):179.
Aytaç N. Orak Hücre Anemisinde Epidemiyoloji ve Bir Halk Sağlığı Sorunu Olarak Ülkemizde Durum. Türkiye Klinikleri Çocuk Hematolojisi ve Onkolojisi- Özel Konular 2021; 2(1):1-4.
Smith-Whitley K, Kwiatkowski JL. Hemoglobinopathies. In: Nelson Textbook of Pediatrics. 21st ed.; 2020:2540–2558.
Heeney MM, Ware RE. Sickle Cell Disease. In: Nathan and Oski’s Hematology and Oncology of Infancy and Childhood. 8th ed.; 2015:675–714.
Noguchi CT, Schechter AN. The Intracellular Polymerization of Sickle Hemoglobin and Its Relevance to Sickle Cell Disease. Blood 1981;58(6):1057–1068.
Keser İ. Hemoglobinopathies in Turkey and the World. Türkiye Klinikleri Tıbbi Genetik 2017;(1):1–6.
Aydınok Y. Hemoglobinopatiler, Bölüm 16. In: Yurdakök Pediatri.; 2017:3332–3338.
Aydoğan G. Anemiler. In: Temel Pediatri.Vol 2. 1st ed. Ankara: Güneş Tıp Kitapevleri; 2020:1820–1851.
Nze C, Fortin B, Freedman R, et al. Sudden death in sickle cell disease: current experience. Br J Haematol 2020;188(4):e43–e45.
Stevens MCG, Padwick M, Serjeant GR. Observations on the natural history of dactylitis in homozygous sickle cell disease. Clin Pediatr (Phila) 1981;20(5):311–317.
Gill FM, Sleeper LA, Weiner SJ, et al. Clinical Events in the First Decade in a Cohort of Infants With Sickle Cell Disease. Blood 1995;86(2):776–783.
Brandow AM, Carroll CP, Creary S, et al. American Society of Hematology 2020 guidelines for sickle cell disease: management of acute and chronic pain. Blood Adv 2020;4(12):2656–2701.
Smith-Whitley K, Zhao H, Hodinka RL, et al. Epidemiology of human parvovirus B19 in children with sickle cell disease. Blood 2004;103(2):422–427.
Ben Khaled M, Ouederni M, Mankai Y, et al. Prevalence and predictive factors of splenic sequestration crisis among 423 pediatric patients with sickle cell disease in Tunisia. Blood CellsMolDis 2020;80. doi:10.1016/J.BCMD.2019.102374.
Klings ES, Steinberg MH. Acute chest syndrome of sickle cell disease: genetics, risk factors, prognosis, and management. Expert Rev Hematol 2022;15(2):117–125.
TheAmerican Academy of Pediatrics. Evidence-Based Management of Sickle Cell Disease: Expert Panel Report, 2014. Pediatrics 2014;134(6):e1775–e1775.
NHLBI. Evidence-Based Management of Sickle Cell Disease -Expert Panel Report 2014 - NationalHeart, Lung, and Blood Institute. [Online] https://www.nhlbi.nih.gov/sites/default/files/media/docs/sickle-cell-disease-report%20020816_0.pdf 2014 [Accesssed10th December 2023].
DeBaun MR, Jordan LC, King AA, et al. American Society of Hematology 2020 guidelines for sickle cell disease: prevention, diagnosis, andtreatment of cerebrovascular disease in children and adults. Blood Adv 2020;4(8):1554–1588.
Serjeant GR. The Natural History of Sickle Cell Disease. Cold Spring Harb Perspect Med 2013;3(10):a011783.
Yee ME, Lai KW, Bakshi N, et al. Bloodstream Infections in Children With Sickle Cell Disease: 2010-2019. Pediatrics 2022;149(1). doi:10.1542/PEDS.2021-051892.
Rineer S, Walsh PS, Smart LR, et al. Risk of Bacteremia in Febrile Children and Young Adults With Sickle Cell Disease in a Multicenter Emergency Department Cohort. JAMA Netw Open 2023;6(6):e2318904–e2318904.
Oligbu G, Collins S, Sheppard C, et al S. Risk of Invasive Pneumococcal Disease in Children with Sickle Cell Disease in England: A National Observational Cohort Study, 2010-2015. Arch Dis Child 2018;103(7):643–647.
Knight-Madden J, Serjeant GR. Invasive pneumococcal disease in homozygous sickle cell disease: Jamaicanexperience 1973-1997. J Pediatr 2001;138(1):65–70.
Piel FB, Jobanputra M, Gallagher M, et al. Co-morbidities and mortality in patients with sickle cell disease in England: A 10-year cohort analysis using hospital episodes statistics (HES) data. Blood Cells Mol Dis 2021;89. doi:10.1016/J.BCMD.2021.102567.
Coria AL, Taylor CM, Tubman VN. Fever Management in Sickle Cell Disease in Low- and Middle-Income Countries: A Survey of SCD Management Programs. Am J Trop Med Hyg 2020;102(4):902–904.
Wang CJ, Kavanagh PL, Little AA, et al. Quality-of-care indicators for children with sickle cell disease. Pediatrics 2011;128(3):484–493.
Vetter CL, Buchanan GR, Quinn CT. Burden of diagnostic radiation exposure in children with sickle cell disease. Pediatr Blood Cancer 2014;61(7):1322–1324.
Ploton MC, Sommet J, Koehl B, et al. Respiratory pathogens and acute chest syndrome in children with sickle cell disease. Arch Dis Child 2020;105(9):891–895.
Lavelle JM, Blackstone MM, Funari MK, et al. Two-Step Processfor ED UTI Screening in Febrile Young Children: Reducing Catheterization Rates. Pediatrics 2016;138(1). doi:10.1542/PEDS.2015-3023.
Lane PA, Buchanan GR, Desposito F, et al. Health Supervision for Children with Sickle Cell Disease. Pediatrics 2002;109(3):526–535.
Gaartman AE, Sayedi AK, Gerritsma JJ, et al. Fluid overload due to intravenous fluid therapy for vaso-occlusive crisis in sickle cell disease: incidenceand risk factors. Br J Haematol 2021;194(5):899–907.
Gaut D, Jones J, Chen C, et al. Outcomes related to intravenous fluid administration in sickle cell patients during vaso-occlusive crisis. AnnHematol 2020;99(6):1217–1223.
Win N, Yeghen T, Needs M, et al. Use of Intravenous Immunoglobulin and Intravenous Methylprednisolone in Hyperhaemolysis Syndrome in Sickle Cell Disease. Hematology 2004;9(5–6):433–436.
Vehapoğlu A, Göknar N, Tuna R, et al. Ceftriaxone-induced hemolytic anemia in a child successfully managed with intravenous immunoglobulin. Turk J Pediatr 2016;58(2):216–219.
Strauß R, Ewig S, Richter K, et al. The Prognostic Significance of Respiratory Rate in Patients With Pneumonia: A retrospective analysis of data from 705 928 hospitalized patients in Germany from 2010–2012. Dtsch Arztebl Int 2014;111(29–30):503.
