Nergis AKAY, Deniz TUĞCU, Rumeysa TUNA, Süheyla OCAK, Serap KARAMAN, Ayşegül ÜNÜVAR, Sevgi KKALAYOĞLU BEŞIŞIK, Zeynep KARAKAŞ

Orak Hücreli Anemili Hastada Akut Göğüs Sendromu: Vaka Sunumu ve Literatürün Gözden Geçirilmesi

Orak hücre anemisi OHA dünyada en sık görülen hemoglobinopatilerdendir. OHA taşıyıcılığı sıklığı Türkiye genelinde %0.3-0.6 arasındayken, özellikle Çukurova bölgesinde bazı yörelerde bu sıklık %3-44’e ulaşmaktadır. Sıklığının artması öngörülen bu hastalığın en önemli komplikasyonu vazookluzif ağrılı krizler olup patofizyolojisinde HbS polimerizasyonu sorumludur. Akut komplikasyonlar arasında gösterilen akut göğüs sendromu; ikinci en sık hastanede yatış ve mortalite nedeni olarak gösterilmektedir. Akut göğüs sendromu gelişen hastalara ilk yaklaşım destekleyici tedaviler iken, solunum bulgularında ve akciğer grafisinde kötüleşme, oksijen desteği gerektirecek kadar hipokseminin derinleşmesi durumunda; basit transfüzyon tedavi için yeterli olamamaktadır. Terapötik eritrosit aferezi; orak hücre hastalığı komplikasyonlarını tedavi etmek amacı ile yapılabilmektedir. Bu makalede orak hücre anemili tanısı ile izlenen ve akut göğüs sendromu gelişen vakamızın, destek tedavileri ve terapötik eritrosit aferezi ile başarılı tedavisi sunulmuştur

Anahtar Kelimeler:

Orak hücreli anemi, akut göğüs sendromu, tedavi

Acute Chest Syndrome in Children with Sickle Cell Disease: Case Report and Review of the Literature

Sickle cell anemia SCA is the most common hemoglobinopathy all over the world. While the overall incidence of sickle cell disease trait in Turkey is %0.3-0.6, especially some places in Çukurova area it reaches the level of 3-44%. Painful vaso- occlusive crisis which is the most important complication of this disease, is predicted to increase in frequency and HbS polymerization is responsible in its pathophysiology. As an acute complication of SCA, acute chest syndrome is the second most common cause of hospitalization and mortality. İnitial approach to patients with an acute chest syndrome includes supportive treatments. Whereas, solely transfusion is not enough for treatment if there is worsening in respiratory signs and chest radiography; deepening of hypoxemia which requires an oxygen support. Therapeutic red cell apheresis can be used with the aim to treat acute complication of sickle cell disease. In this article we present successful therapeutic red cell apheresis and supportive treatments that we practise in our case who developed acute chest syndrome, and followed up by us with the diagnosis of sickle cell disease

Keywords:

Sickle cell anemia, acute chest syndrome, treatment,

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