Castleman Lenfadenopati
Özet
Castleman hastalığı (CH), lenf nodlarında reaktif ve lenfoproliferatif değişikliklerle karakterize, klinik ve histopatolojik açıdan heterojen bir hastalık grubudur. Klinik olarak unisentrik Castleman hastalığı (UCH) ve multisentrik Castleman hastalığı (MCH) olarak sınıflandırılır. UCH genellikle lokalize lenf nodu tutulumu ile seyrederken, MCH yaygın lenfadenopati, sistemik inflamasyon ve multiorgan tutulumu ile ilişkili daha agresif bir tablo oluşturur. Hastalığın patogenezinde IL-6 aracılı sitokin disregülasyonu merkezi rol oynarken, özellikle HHV-8 ilişkili olgularda viral enfeksiyon önemli bir etkendir. Histopatolojik olarak hiyalin vasküler, plazma hücreli ve mikst tipler tanımlanmıştır. Hiyalin vasküler tipte regresif germinal merkezler, “lollipop” foliküller ve “onion-skin” mantle zonları karakteristik iken; plazma hücreli tipte interfoliküler plazmasitoz ön plandadır. HHV-8 ilişkili MCH’da LANA-1 pozitif plazmablastik hücreler dikkat çekicidir. Castleman hastalığının tanısında histopatolojik değerlendirme temel olmakla birlikte, klinik, laboratuvar ve immünohistokimyasal bulguların birlikte yorumlanması gereklidir. Özellikle lenfomalar, HIV ilişkili lenfadenopati ve otoimmün hastalıklarla ayırıcı tanı önem taşır.
Castleman disease (CD) is a heterogeneous group of disorders characterized by reactive and lymphoproliferative changes in lymph nodes, with diverse clinical and histopathological features. Clinically, CD is classified into unicentric Castleman disease (UCD) and multicentric Castleman disease (MCD). UCD usually presents with localized lymph node involvement, whereas MCD is associated with generalized lymphadenopathy, systemic inflammation, and multiorgan involvement, resulting in a more aggressive clinical course. Dysregulated IL-6–mediated cytokine signaling plays a central role in the pathogenesis of the disease, while viral infection, particularly HHV-8, is an important contributing factor in specific subtypes. Histopathologically, CD is divided into hyaline vascular, plasma cell, and mixed variants. The hyaline vascular type is characterized by regressed germinal centers, “lollipop” follicles, and “onion-skin” mantle zones, whereas interfollicular plasmacytosis is the predominant feature of the plasma cell type. In HHV-8–associated MCD, LANA-1–positive plasmablastic cells are a characteristic finding. Although histopathological examination is fundamental for diagnosis, clinical, laboratory, and immunohistochemical findings should be interpreted in conjunction. Differential diagnosis is particularly important with lymphomas, HIV-associated lymphadenopathy, and autoimmune diseases.
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