Hemofagositik lenfohistiyositoz hastalarında kraniyal MRG bulguları
Küçük Resim Yok
Tarih
2018
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Erişim Hakkı
info:eu-repo/semantics/openAccess
Özet
Amaç: Hemofagositik lenfohistiyositoz (HLH) kontrolsüz immün yanıta yol açan hiperinflamasyonla karakterize klinik bir sendromdur. Etiyolojiye bağlı primer ve sekonder olarak sınıflanmaktadır. Klinik bulguları arasında ateş, splenomegali, hepatit yer almaktadır. Tanı doku örneklenmesi ile konulur. Familyal HLH'li hastaların santral sinir sistemi (SSS) tutulumu hastalığın prognozunu ve klinik gidişini olumsuz etkileyen bir faktördür. SSS tutulumunun bulguları; progresif ensefalopati, irritabilite, nöbet, kraniyal sinir paralizisi, ataksi, nistagmus, yürüme ve görme bozukluğu, psikomotor gelişim geriliğidir. Bu çalışmada, SSS tutulumu olan 6 olgunun radyolojik görüntüleme bulgularını sunmayı amaçladık. Gereç ve Yöntem: Bir-onbir yaş aralığında 6 hasta irritabilite, nöbet, ataksi, nistagmus, yürüme ve görme bozukluğu, psikomotor gelişim geriliği gibi nörolojik bulgularla hastanemize baş vurdu ve nörolojik muayene sonrasında kranyal manyetik rezonans görüntüleme (MRG) incelemesi gerçekleştirildi. Bulgular: Hastalarımıza klinik ve laboratuvar değerlendirmeleri sonrasında yapılan kemik iliği biyopsisi ile HLH tanısı konuldu. Hastalarımızın tamamında SSS tutulumunu gösteren kraniyal MRG bulguları saptandı. MRG incelemesinde T2 ağırlıklı ve FLAIR sekanslarında serebral hemisferlerde hiperintens odaklar ve postkontrast serilerde patolojik kontrastlanma ortak bulgular olarak belirlendi. Sonuç: HLH hastalığının santral sinir sistemi tutulumu yüksek mortalite ve morbidite ile seyretmektedir. Kraniyal MRG SSS tutulumunu göstermede önemli rol oynamaktadır.
Aim: Hemophagocytic lymphohistiocytosis (HLH) is a clinical syndrome characterized by hyperinflammation causing uncontrolled immune response. It is classified as primary and secondary according to etiology. Fever, splenomegaly, hepatitis are among the clinical findings. The diagnosis is made with tissue sampling. The central nervous system (CNS) involvement of familial HLH patients is a factor that affects the prognosis and course of the disease. The findings of CNS involvement include progressive encephalopathy, irritability, attack, cranial nerve paralysis, ataxy, nistagmus, walking and visual impairment, psychomotor development deficiency. In this study, we aimed at presenting the radiologic imaging findings of 6 cases with CNS involvement. Materials and Methods: Six patients between the ages of 1-11 years applied to our hospital with neurological findings such as irritability, attack, cranial nerve paralysis, ataxy, nistagmus, walking and visual impairment, psychomotor development deficiency. Following the neurological examination, the cranial magnetic resonance imaging (MRI) examination was conducted. Results: HLH diagnosis was made through bone marrow biopsy after clinical and lab oratory evaluations. Cranial MRI findings indicating CNS involvement were observed in all of our patients. Hyperintense foci in cerebral hemispheres and pathological contrast enhancement in postcontrast series were defined in T2 and FLAIR sequences in MRI examination. Conclusion: The central nervous system involvement of HLH disease is observed with high mortality and morbidity. Cranial MRI plays a significant role in revealing CNS involvement.
Aim: Hemophagocytic lymphohistiocytosis (HLH) is a clinical syndrome characterized by hyperinflammation causing uncontrolled immune response. It is classified as primary and secondary according to etiology. Fever, splenomegaly, hepatitis are among the clinical findings. The diagnosis is made with tissue sampling. The central nervous system (CNS) involvement of familial HLH patients is a factor that affects the prognosis and course of the disease. The findings of CNS involvement include progressive encephalopathy, irritability, attack, cranial nerve paralysis, ataxy, nistagmus, walking and visual impairment, psychomotor development deficiency. In this study, we aimed at presenting the radiologic imaging findings of 6 cases with CNS involvement. Materials and Methods: Six patients between the ages of 1-11 years applied to our hospital with neurological findings such as irritability, attack, cranial nerve paralysis, ataxy, nistagmus, walking and visual impairment, psychomotor development deficiency. Following the neurological examination, the cranial magnetic resonance imaging (MRI) examination was conducted. Results: HLH diagnosis was made through bone marrow biopsy after clinical and lab oratory evaluations. Cranial MRI findings indicating CNS involvement were observed in all of our patients. Hyperintense foci in cerebral hemispheres and pathological contrast enhancement in postcontrast series were defined in T2 and FLAIR sequences in MRI examination. Conclusion: The central nervous system involvement of HLH disease is observed with high mortality and morbidity. Cranial MRI plays a significant role in revealing CNS involvement.
Açıklama
Anahtar Kelimeler
Cerrahi
Kaynak
Ege Tıp Dergisi
WoS Q Değeri
Scopus Q Değeri
Cilt
57
Sayı
1
