T-lenfoblastik lenfoma

Özet

T-lenfoblastik lenfoma (T-LBL), özellikle ergen ve genç erişkinlerde timik/anterior mediastinal kitleyle ortaya çıkabilen, agresif seyirli bir prekürsör T-hücreli neoplazidir. Toraks BT, kitlenin boyut ve yayılımını, trakea ve bronşlar ile büyük damarlar üzerindeki basıyı, plevral/perikardiyal efüzyonları ve eşlik eden nodal hastalığı gösterir; T-LBL/T-ALL adlandırması periferik kan ve kemik iliği bulgularının hematopatolojik değerlendirmesiyle yapılır. Hacimli mediastinal kitle hava yolu daralması, vena kava superior sendromu ve plevral veya perikardiyal efüzyonlarla ilişkili olabilir.

Faz ve pencere

Portal tanısal

İntravenöz kontrastlı toraks BT'de timik yatağı merkez alan ve genellikle eksskapüler yumuşak doku ile birden fazla lenf nodunu içeren hacimli bir kitle izlenebilir. Mediastinal lenf nodları, trakea ve ana bronşlar ile büyük damarların itilme, sarılma veya lümen daralması bulguları değerlendirilir.

Önerilen pencereler: Mediasten (G 350 / M 50), Akciğer (G 1500 / M -600).

BT bulguları

• Timik yatağı merkez alan ve anterior mediastinal yumuşak dokuya uzanabilen geniş kitle; servikal veya supraklaviküler nodlar eşlik edebilir.
• Mediastinal yağ planlarında silinme veya komşu yapıların yer değiştirmesi, kitlenin lokal yayılımı ya da kitle etkisiyle uyumlu olabilir.
• Trakea veya ana bronşlarda kitlenin dıştan basısına bağlı yer değiştirme ve/veya lümen daralması
• Vena kava superior, brakiyosefalik ven veya pulmoner arterlerde bası, sarılma ve yer değiştirme görülebilir; lümen açıklığı ayrıca değerlendirilir.
• Birden fazla mediastinal istasyonda büyümüş lenf nodları veya birbirine bitişik nodal yumuşak doku
• Plevral ve/veya perikardiyal efüzyon
• Plevra ve perikardiyum gibi ekstanodal bölgelerin tutulması veya efüzyon oluşumu beklenir; kitle etkisi ve komşu yapılar birlikte değerlendirilir.
• Anterior mediastinal kitleye ek olarak mediastinal lenf nodları ve ekstranodal yumuşak doku tutulumu saptanabilir.

Normalde

Normal timusun boyutu ve biçimi yaşla değişir; gençlerde belirgin timik doku fizyolojik olabilir. Anterior mediastinal yağ kalp ve büyük damarların önünde seçilebilir. Aynı seviyede trakea ve ana bronş lümenlerinin açık olmasını, vena kava superiorun kontrastla dolu ve basısız görünmesini, karşı akciğerin havalanmasını ve plevral-perikardiyal sıvı bulunmamasını kitle etkisiyle karşılaştır.

Ayırıcı tanı

Primer mediastinal büyük B hücreli lenfoma

benzer bölgede hacimli kitle yapabilir ve komşu yapılara uzanabilir; görüntüleme bulguları örtüşür, kesin alt tip tanısı için doku incelemesi gerekir.

Timoma

timik yatakta düzgün sınırlı, kapsüllü yumuşak doku kitlesi şeklinde görülebilir; yaş ve nodal dağılım ayırıcı tanıda yardımcıdır ancak BT tek başına kesin ayrım sağlamaz.

Hodgkin lenfoma

anterior mediastinal kitle ve mediastinal lenfadenopati yapabilir; BT görünümü T-lenfoblastik lenfomayla örtüşür ve kesin ayrım doku incelemesi gerektirir.

Mediastinal germ hücreli tümör

teratomda yağ, sıvı ve kalsifik bileşenler; nonseminomatöz tümörde heterojen görünüm, kanama veya nekroz görülebilir.

Substernal tiroid dokusu

servikal tiroidle kesintisiz devamlılık ve benzer dansite substernal guatrı destekler; nadiren ektopik tiroid dokusu tiroidle devamlı olmayabilir.

Tuzaklar

• T-lenfoblastik lenfoma ve T-ALL aynı prekürsör T-hücreli neoplazi spektrumundadır; WHO sınıfaması BM'de blast oranına göre ayrım yapar (<%25 lenfoma, >%25 lösemi).
• Ön mediastendeki normal timus özellikle gençlerde belirgin olabilir; yaşa uygun biçim ve simetriyi, komşu yapıların yer değiştirmesi ve nodal hastalıkla birlikte değerlendir.
• Plevral sıvıyı kostofrenik sinüslerde, perikardiyal sıvıyı kalp çevresinde tüm inceleme boyunca ayrıca ara.
• Damarın kitle tarafından çevrelenmesi tek başına tromboz göstermez; kontrastlanan lümeni kesitler boyunca izleyerek dıştan bası ile lümen içi dolum kusurunu ayır.

Kendini dene

1. Genç hastada timik yatağı dolduran kitle trakeayı ve vena kava superioru daraltıyor. BT hangi kritik sonucu doğrudan gösterir?

   • Mediastinal bası
   • Kemik iliği yükü
   • T-hücre fenotipi
   • Kromozom anomalisi
   Cevabı göster

   Mediastinal bası. BT hava yolu ve damarların kitleyle ilişkisini, lümen daralmasını ve eşlik eden efüzyonları gösterir. Kemik iliği yükü, T-hücre fenotipi ve kromozom bulguları görüntüleme ile belirlenmez; kan, kemik iliği ve doku incelemesi gerekir.

2. Anterior mediastinal kitle tiroid dokusuyla boyuna kesintisiz devam ediyor. En uygun ayırıcı tanı hangisidir?

   • Substernal guatr
   • T-lenfoblastik lenfoma
   • Hodgkin lenfoma
   • Timik lenfoma
   Cevabı göster

   Substernal guatr. Servikal tiroidle kesintisiz anatomik devamlılık substernal guatrı destekler. Lenfoma alt tipi BT ile kesinleştirilemez; doku incelemesi gerekir.

İlgili konular

• Primer mediastinal büyük B hücreli lenfoma

Kaynaklar

Bu sayfadaki 38 cümle ve 2 quiz sorusu aşağıdaki kaynaklardan alıntılarla tek tek karşılaştırıldı; 4 cümle bu karşılaştırmada düzeltildi (2026-10-09).

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