NADİR BİR LENFOPROLİFERATİF HASTALIK: CASTLEMAN HASTALIĞI / A RARE LYMPHOPROLIFERATIVE DISEASE: CASTLEMAN DISEASE

OZET: Castleman hastaligi, “anjiyofollikuler lenf nodu hiperplazisi” olarak da bilinen nadir bir lenfoproliferatif hastaliktir. Tukruk bezleri, akciger, pankreas, larinks, parotis bezi, meninksler hatta ekstremite kaslari ekstralenfatik olarak tutulabilir. Histolojik olarak hiyalin vaskuler ve plazma hucreli varyant olarak siniflandirilir ancak nadiren iki tipe ait ozellikler bir arada bulunabilir. Unisentrik hastaligi olan olgularin cogu hiyalin vaskuler, multisentrik hastaligi olan olgularin cogu ise plazma hucreli histolojik tipindedir. HHV-8 pozitif olgular farkli histolojik ozellikler gosterir (plazmablastik varyant). Patogenezi tam olarak anlasilmamistir fakat unisentrik hastalikta IL-6’nin, multisentrik hastalikta IL-6 ve HHV-8’in rolu iyi tanimlanmistir. Unisentrik hastalik tipik olarak lokalizedir, semptomlar minimaldir ve tek basina lokal tedavi uygulanir. Multisentrik hastalik siklikla HIV varliginda gorulen sistemik bir hastaliktir ve klinik olarak yaygin lenfadenopati, splenomegali, anemi ve sistemik inflamatuar semptomlarla karakterizedir. Baslica sistemik tedaviler uygulanir. Malign bir durum olmamakla birlikte bazi malign hastaliklarla (lenfomalar, POEMS sendromu, follikuler dendritik hucreli sarkomlar, paraneoplastik pemphigus, Kaposi sarkomu, amiloidoz) ile iliskili olabilir. Bu yazida nadir ama onemli bir hastalik olan Castleman hastaligi ile ilgili guncel bilgiler ozetlenmistir. ANAHTAR KELIMELER: L enfoproliferatif hastalik, Castleman, Tani, Tedavi A RARE LYMPHOPROLIFERATIVE DISEASE: CASTLEMAN DISEASE ABSTRACT: Castleman disease, also known as angiofollicular lymph node hyperplasia, is a rare lymphoproliferative disease. Salivary glands, lung, pancreas, larynx, parotid gland, meninges and even extremity muscles can be involved extralymphatically. It is classified as hyaline vascular and plasma cell variant histologically but the characteristics of both types can coexist. Most of unicentric disease is hyalin vascular and most of multicentric disease is plasma cell type histologically. HHV-8 positive cases show different histological characteristics (plasmablastic variant). Pathogenesis is not completely understood however the role of IL-6 in unicentric disease and the role of IL-6 and HHV-8 in multicentric disease is well defined. Unicentric disease is typically localized, symptoms are minimal and treated locally. Multicentric disease is a systemic disease and frequently accompanies HIV positivity. It is clinically characterized with generalized lymphadenopathy, splenomegaly, anemia and systemic inflammatory symptoms. Systemic therapies are given primarily. Although it is not a malignant condition can be associated with several malignant diseases (lymphomas, POEMS syndrome, follicular dendritic cell sarcomas, paraneoplastic pemphigus, Kaposi sarcoma, amyloidosis). In this paper; recent information about Castleman disease, which is a rare but important disease, is summarized. KEYWORDS: Lenfoproliferative disease, Castleman, Diagnosis, Treatment