Dalak amiloidozu

Özet

Dalak amiloidozu, sistemik amiloid birikiminin dalak parankimini ve damarlarını tutmasıyla oluşur; görüntüleme bulguları tanıyı kesinleştirecek şekilde özgül değildir. Splenomegali tüm tiplerde görülmez; AA tipinde belirgin iken AL ve ALECT2 tiplerinde dalak boyutu genellikle normal yakın seyreder. BT amiloid birikimini doğrulamaz. Akut ağrı geliştiğinde nadir fakat ciddi spontan rüptür ve hemoperitoneum bulguları aranmalıdır.

Faz ve pencere

Portal

T2 ağırlıklı MR'da dalak parankiminde diffüz düşük sinyal görülebilir; bu bulgu amiloidoz için ipucu olsa da özgül değildir.

Kontrastsız

Kontrastsız BT dalak boyutunu, konturunu ve varsa kan ürünlerini gösterir; amiloid birikimini göstererek tanı koydurmaz.

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

BT bulguları

• Splenomegali görülebilir ancak şart değildir; tek başına amiloidoz göstergesi sayılmaz.
• Parankimde diffüz atenüasyon farkı veya yamalı heterojenlik görülebilir; bu bulgular özgül değildir.
• Dalakta görece az kontrastlanma (hipoperfüzyon) nadiren görülebilir; bu bulgu teknik değişkenlerden etkilenebilir ve amiloidozu tek başına kanıtlamaz.
• Nodüler veya fokal hipoattenüan alanlar görüldüğünde lenfoma, enfarkt ve enfeksiyon gibi ayırıcı tanılar da değerlendirilir.
• Eşlik eden hepatomegali, böbrek boyut değişikliği ya da bağırsak duvar kalınlaşması sistemik abdominal tutulumu destekleyebilir.
• Rüptür gelişmişse düzensiz dalak konturu veya hilusta kapsül kesintisiyle birlikte perisplenik ve intraperitoneal kan görülebilir.

Normalde

Normal dalak, aynı kontrast fazındaki komşu kesitlerde düzgün konturlu ve homojen parankimli görünür; portal venöz fazda parankim yaygın biçimde kontrastlanır. Dalak konturunu ve kraniokaudal uzanımını, parankim atenüasyonunun homojenliğini, hilusu ve perisplenik yağ planlarını normal referansla karşılaştır.

Ayırıcı tanı

Splenik sarkoidoz

çoklu parankimal nodüller ile hiler veya mediastinal lenfadenopati birlikteliği ipucu olabilir.

Konjestif splenomegali

portal hipertansiyon bulguları, kollateraller ve karaciğer morfolojisi büyümeyi açıklayabilir.

Splenik enfarkt

kontrastlı BT'de düzensiz hipoattenüan, kontrastlanmayan lezyonlar olarak izlenir; bu görünüm damarsal oklüzyona bağlıdır.

Tuzaklar

• Splenomegaliyi tek başına amiloidoz kabul etme; enfeksiyon, hematolojik hastalık ve portal hipertansiyon da benzer büyüme yapabilir.
• Fokal hipoattenüan alanları amiloid birikimi diye adlandırma; lenfoma, enfarkt ve enfeksiyonla görüntü örtüşebilir.
• Kontrastlanmanın görece az olması teknik ve dolaşım koşullarından etkilenebilir; karşılaştırmayı aynı fazdaki komşu organlarla yap.
• Akut karın tablosunda heterojen sıvıyı basit asit sayma; hilus ve kapsül bütünlüğü ile hemoperitoneumu birlikte incele.

Kendini dene

1. Bilinen sistemik amiloidozu olan kişide BT’de dalak parankiminde yamalı heterojenlik var. BT bulguları özgül olmadığından tek başına dalak amiloidozu tanısını kesinleştiremez.

   • Görüntüleme amiloid tutulumunu tek başına doğrulamaz
   • Dalak amiloidozu kesinleşmiştir
   • Splenik lenfoma dışlanmıştır
   • Dalak sarkoidozu kanıtlanmıştır
   Cevabı göster

   Görüntüleme amiloid tutulumunu tek başına doğrulamaz. Dalak parankimindeki değişiklik amiloidozda görülebilir, ancak BT bulguları özgül değildir; tek başına tanıyı doğrulamaz veya lenfoma ve sarkoidoz gibi ayırıcı tanıları dışlamaz.

2. Bilinen amiloidozu olan ve travma olmadan aniden sol üst kadran ağrısı başlayan kişide BT ile hangi ciddi komplikasyon dışlanmalıdır?

   • Spontan dalak rüptürü
   • Splenik apse
   • Splenik infarkt
   • Dalak lenfoması
   Cevabı göster

   Spontan dalak rüptürü. Amiloid tutulumlu dalakta nadiren spontan rüptür gelişebilir; BT’de kapsül kesintisi ve hemoperitoneum aranır. Apse, enfarkt ve lenfoma alternatif tanılardır ancak bu klinik tabloda rüptür bulgularını atlamamak gerekir.

Kaynaklar

Bu sayfadaki 32 cümle ve 2 quiz sorusu aşağıdaki kaynaklardan alıntılarla tek tek karşılaştırıldı; 6 cümle bu karşılaştırmada düzeltildi (2026-10-09).

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