A Rare Lymphoproliferative Disease: Castleman DiseaseEren Gündüz1, Nihal Özdemir2, Şule Mine Bakanay3, Sema Karakuş41Eskişehir Osmangazi University Faculty of Medicine, Department of Hematology, Eskişehir, Turkey
2İstinye University Medical School, Department of Hematology, İstanbul, Turkey
3Ankara Yıldırım Beyazıt University Medical School, Department of Hematology, Ankara, Turkey
4Başkent University Faculty of Medicine, Department of Hematology, Ankara, Turkey
Castleman disease is a rare lymphoproliferative disease also known as angiofollicular lymph node hyperplasia. It is classified as hyaline vascular and plasmacytic variants histologically but characteristics of both types can coexist. Most unicentric cases of the disease are hyaline vascular while most multicentric cases are of the plasmacytic type. Although the pathogenesis is not completely understood, the role of interleukin (IL)-6 in unicentric disease and the roles of IL-6 and human herpes virus-8 in multicentric disease are well defined. Unicentric disease is typically localized and symptoms are minimal and treated locally. Multicentric disease is systemic and clinically characterized by generalized lymphadenopathy, splenomegaly, anemia, and systemic inflammatory symptoms. Systemic therapies are primarily given. Several malignant diseases including lymphomas, POEMS syndrome, follicular dendritic cell sarcomas, paraneoplastic pemphigus, Kaposi sarcoma, and amyloidosis can be associated with Castleman disease. In this paper, recent information about Castleman disease, which is a rare disease, is summarized. Keywords: Castleman disease, Diagnosis, Treatment
Nadir Bir Lenfoproliferatif Hastalık: Castleman HastalığıEren Gündüz1, Nihal Özdemir2, Şule Mine Bakanay3, Sema Karakuş41Eskişehir Osmangazi University Faculty of Medicine, Department of Hematology, Eskişehir, Turkey
2İstinye University Medical School, Department of Hematology, İstanbul, Turkey
3Ankara Yıldırım Beyazıt University Medical School, Department of Hematology, Ankara, Turkey
4Başkent University Faculty of Medicine, Department of Hematology, Ankara, Turkey
Castleman hastalığı, anjiyofolliküler lenf nodu hiperplazisi olarak da bilinen nadir bir lenfoproliferatif hastalıktır. Histolojik olarak hiyalin vasküler ve plazmasitik varyant olarak sınıflandırılır ancak nadiren iki tipe ait özellikler bir arada bulunabilir. Unisentrik hastalığı olan olguların çoğu hiyalin vasküler, multisentrik hastalığı olan olguların çoğu ise plazma hücreli histolojik tipindedir. Patogenezi tam olarak anlaşılmamıştır fakat unisentrik hastalıkta interlökin (IL)-6’nın, multisentrik hastalıkta IL-6 ve human herpes virüs-8’in rolü iyi tanımlanmıştır. Unisentrik hastalık tipik olarak lokalizedir, semptomlar minimaldir ve tek başına lokal tedavi uygulanır. Multisentrik hastalık sistemik bir hastalıktır ve klinik olarak yaygın lenfadenopati, splenomegali, anemi ve sistemik inflamatuar semptomlarla karakterizedir. Başlıca sistemik tedaviler uygulanır. Lenfomalar, POEMS sendromu, folliküler dendritik hücreli sarkomlar, paraneoplastik pemphigus, Kaposi sarkomu, amiloidoz Castleman hastalığı ile ilişkili olabilir. Bu yazıda nadir bir hastalık olan Castleman hastalığı ile ilgili güncel bilgiler özetlenmiştir. Anahtar Kelimeler: Castleman hastalığı, Tanı, Tedavi
Eren Gündüz, Nihal Özdemir, Şule Mine Bakanay, Sema Karakuş. A Rare Lymphoproliferative Disease: Castleman Disease. Turk J Hematol. 2021; 38(4): 314-320
Corresponding Author: Eren Gündüz |
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