Kikuchi Hastalığı
Özet
Kikuchi hastalığı (KH), kendiliğinden iyileşebilen, maligniteyi taklit edebilen ve farklı histopatolojik paternler gösterebilen bir lenfadenittir. KH en sık genç yetişkinlerde izlenmektedir. Hastalığın gelişiminde T hücrelerinin anormal immün reaksiyonu önemli yer tutar. Hastalığı başlatan olay genellikle enfeksiyöz bir süreç olup daha çok viral enfeksiyonlardır. KH, lenfadenopati ile kendini gösterir. Sistemik B semptomları hastalarda izlenebilir. Histolojisinde proliferatif (lenfohistiyositik), nekrotizan ve ksantomatöz olmak üzere üç faz tanımlanmıştır. Proliferatif fazda, plazmasitik dendritik hücre proliferasyonu, immunoblastlar ve histiyositler mevcuttur. CD8 pozitif T lenfositler, CD4 T lenfositlere baskınlık gösterir. İmmunoblastların çoğu CD8 pozitif T lenfositlerdir ve CD30 boyanabilir. KH’nin ayırıcı tanısında lenf nodu infarktı, sistemik lupus eritematozus (SLE), nekrotizan granulomatöz lenfadenit, HSV lenfadenit, enfeksiyöz mononükleoz, diffüz büyük B hücreli lenfoma, Klasik Hodgkin lenfoma, T hücreli lenfoma, myeloid sarkom ve taşlı yüzük hücreli karsinom bulunmaktadır. SLE, malignite dışı en önemli ayırıcı tanıdır. Ayrıca, KH olgularının bir kısmı da SLE’ye ilerleyebilir. Malignitelerle asıl tanı zorluğu T hücreli lenfomalar olup KH’de de klonal proliferasyonlar görülebilmektedir.
Kikuchi disease (KD) is a lymphadenitis that can resolve spontaneously, mimic malignancy, and exhibit various histopathological patterns. KD is most commonly seen in young adults. Abnormal immune response of T cells plays a significant role in the development of the disease. The initiating event is usually an infectious process, most often viral infections. KD manifests as lymphadenopathy. Systemic B symptoms may be observed in patients. Three phases have been identified in its histology: proliferative (lymphohistiocytic), necrotizing, and xanthomatous. In the proliferative phase, plasmacytic dendritic cell proliferation, immunoblasts, and histiocytes are present. CD8-positive T lymphocytes predominate over CD4 T lymphocytes. Most immunoblasts are CD8-positive T lymphocytes and can stain with CD30. The differential diagnosis of Kikuchi-Fujimoto disease (KH) includes lymph node infarction, systemic lupus erythematosus (SLE), necrotizing granulomatous lymphadenitis, HSV lymphadenitis, infectious mononucleosis, diffuse large B-cell lymphoma, classic Hodgkin lymphoma, T-cell lymphoma, myeloid sarcoma, and signet ring cell carcinoma. SLE is the most important non-malignant differential diagnosis. Furthermore, some KH cases may progress to SLE. The main diagnostic challenge with malignancies is T-cell lymphomas, and clonal proliferations can also be seen in KH.
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