Hemofagositik Lenfohistiyositoz ve Makrofaj Aktivasyon Sendromları
Özet
Hemofagositik lenfohistiyositoz (HLH) ve makrofaj aktivasyon sendromu (MAS), immün sistemin aşırı aktivasyonu sonucu gelişen, hayatı tehdit eden hiperinflamatuar durumlardır. Hastalık, genetik yatkınlık veya enfeksiyon, malignite ve romatolojik bozukluklar gibi dış faktörler nedeniyle ortaya çıkarak kontrolsüz sitokin salınımı ve çoklu organ yetmezliğine yol açar. Özellikle santral sinir sistemi tutulumu, hastalığın seyri boyunca sık görülen ciddi bir komplikasyon olup, nörolojik sekellere ve artmış mortaliteye neden olabilir. Erken tanı ve immünosupresif tedavilerin yanı sıra kök hücre nakli, bu tablonun yönetiminde kritik bir role sahiptir.
Hemophagocytic lymphohistiocytosis (HLH) and macrophage activation syndrome (MAS) are life-threatening hyperinflammatory conditions characterized by excessive immune system activation and cytokine storms. The pathology arises from primary genetic mutations or secondary triggers such as infections, malignancies, and autoimmune disorders, leading to severe multi-organ dysfunction. Central nervous system involvement is a frequent and critical complication that significantly increases morbidity and mortality, necessitating urgent and comprehensive clinical management. Effective treatment relies on early diagnosis, rapid suppression of hyperinflammation using immunosuppressive therapies, and, where applicable, hematopoietic stem cell transplantation.
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