Hepatik amiloidoz

Özet

Hepatik amiloidoz, sistemik amiloid birikiminin karaciğeri tuttuğu ve çoğu zaman başka organ tutulumlarıyla birlikte görülen infiltratif bir tablodur. BT'de en sık hepatomegali ve heterojen, görece düşük atenüasyon görülür; bulgular özgül değildir. Kontrastlı incelemede infiltrasyon alanları erken serilerde düşük atenüasyonlu kalıp gecikmiş seride kısmen dolabilir. İleri tutulumda portal hipertansiyon, damarların silikleşmesi veya nadiren kanama gibi komplikasyonların fark edilmemesi klinik tabloyu eksik değerlendirmeye yol açabilir.

Faz ve pencere

Kontrastsız

Karaciğer genellikle büyümüştür ve kontrastsız görüntülerde parankim atenüasyonu yaygın olarak azalmış veya heterojendir.

Portal tanısal

İnfiltre alanlar çevre parankime göre düşük atenüasyonlu veya heterojen kontrastlanan odaklar şeklinde görülebilir; hepatik venler silik ya da daralmış seçilebilir.

Gecikmiş tanısal

Portal fazda düşük atenüasyonlu alanlar geç görüntülerde kısmi kontrast dolumu ve daha homojen görünüm kazanabilir; bu patern amiloidoza özgü değildir.

Önerilen pencereler: Karaciğer (G 150 / M 30), Batın (G 400 / M 50), Yumuşak doku (G 400 / M 40).

BT bulguları

• Diffüz hepatomegali görülebilir.
• BT’de karaciğer atenüasyonu yaygın olarak azalabilir ve parankim heterojenliği görülebilir.
• Portal fazda yama tarzı hipoenhans alanlar ve gecikmiş seride kısmi dolum
• Hepatik venlerin ince, daralmış veya güç seçilir olması; konjestif değişikliklerle karışabilir
• Periportal ödem veya periportal düşük atenüasyon eşlik edebilir
• Hepatik amiloidozda, infiltrasyonun derecesiyle ilişkili olarak portal hipertansiyon gelişebilir; asit ise nadir olmakla birlikte görülebilir.
• Sistemik amiloidozda dalak, böbrek veya kalp tutulumu gibi eşlik eden bulgular araştırılabilir.

Normalde

Normal karaciğer homojen atenüasyon ve kontrastlanma gösterir; hepatik venler ince fakat kesintisiz, portal ven dalları açık seçilir. Aynı portal ve gecikmiş düzeyleri karşılaştırırken karaciğer boyutunu, parankim içindeki bölgesel kontrast farkını, hepatik venlerin izlenebilirliğini ve periportal dokuyu incele.

Ayırıcı tanı

Hepatik steatoz da görüntülemede görülebilir; hepatik amiloidozun görüntüleme bulguları özgül olmadığından bu bulgular tek başına ayırıcı tanı sağlamaz.

Konjestif hepatopati

hepatomegali ve heterojen kontrastlanma yapabilir; geniş IVC ve hepatik venler ile sağ kalp bulguları konjesyonu destekler.

Lenfoma veya metastatik infiltrasyon

fokal ya da diffüz parankim heterojenliği oluşturabilir; eşlik eden kitleler ve lenfadenopati ayırıcı ipucudur.

Budd-Chiari sendromu

hepatik ven çıkış obstrüksiyonu, kaudat lob hipertrofisi ve kollateral venlerle ayrılır.

Glikojenik hepatopati, kontrolsüz diyabet bağlamında hepatomegali ve geçici aminotransferaz yüksekliğiyle görülebilir.

Tuzaklar

• Heterojen düşük atenüasyon steatozla kolayca karışır; gecikmiş kontrast dolumu ve sistemik organ bulguları amiloidoz şüphesini artırır.
• Gecikmiş fazda homojenleşme tek başına amiloidoz tanısı koydurmaz; görüntüleme paterni özgül değildir.
• Hepatik venlerin silik görünmesi infiltrasyonla ilişkili olabilir; venöz tromboz varmış gibi yorumlamadan ven lümeninin devamlılığını ve IVC'yi incele.
• Kesin tanı BT ile konmaz; klinik şüphe sürüyorsa doku örneklemesi ve patoloji gerekir.

Kendini dene

1. Sistemik protein birikimi kuşkusu olan hastada BT, hepatomegali ve portal fazda yama tarzı hipoenhans alanlar gösteriyor; gecikmiş seride alanlar kısmen doluyor. En olası süreç nedir?

   • Hepatik amiloidoz
   • Budd-Chiari sendromu
   • Fokal nodüler hiperplazi
   • Hepatik steatoz
   Cevabı göster

   Hepatik amiloidoz. Hepatomegali, heterojen düşük atenüasyon ve gecikmiş kısmi dolum amiloid infiltrasyonunda tarif edilmiştir; görüntü özgül değildir. Budd-Chiari venöz çıkış tıkanıklığı, FNH fokal arteriyel lezyon, steatoz ise yağ dağılımı ve düşük kontrastsız yoğunlukla ayırt edilir.

2. Hepatik AL amiloidozlu olguların BT bulguları arasında hangi görünüm bildirilmiştir?

   • Diffüz hepatomegali ve azalmış karaciğer atenüasyonu
   • Yalnızca safra yollarında yaygın genişleme
   • Karaciğerde çok sayıda kalsifiye kist
   • Karaciğer boyutu ve atenüasyonunun daima normal olması
   Cevabı göster

   Diffüz hepatomegali ve azalmış karaciğer atenüasyonu. İncelenen hepatik AL amiloidozlu olguların BT yapılanlarında diffüz hepatomegali ve azalmış karaciğer atenüasyonu bildirilmiştir.

İlgili konular

• Siroz morfolojisi ve rejeneratif nodüller
• Konjestif hepatopati (nutmeg liver)

Kaynaklar

Bu sayfadaki 36 cümle ve 2 quiz sorusu aşağıdaki kaynaklardan alıntılarla otomatik olarak karşılaştırıldı; 8 cümle bu karşılaştırmada düzeltildi (2026-10-09).

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