NADİR BİR LENFOPROLİFERATİF HASTALIK: CASTLEMAN HASTALIĞI

Eren Gündüz

doi:http://dx.doi.org/10.20515/otd.53812

NADİR BİR LENFOPROLİFERATİF HASTALIK: CASTLEMAN HASTALIĞI

Öz

ÖZET: Castleman hastalığı, “anjiyofolliküler lenf nodu hiperplazisi” olarak da bilinen nadir bir lenfoproliferatif hastalıktır. Tükrük bezleri, akciğer, pankreas, larinks, parotis bezi, meninksler hatta ekstremite kasları ekstralenfatik olarak tutulabilir. Histolojik olarak hiyalin vasküler ve plazma hücreli 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. HHV-8 pozitif olgular farklı histolojik özellikler gösterir (plazmablastik varyant). Patogenezi tam olarak anlaşılmamıştır fakat unisentrik hastalıkta IL-6’nın, multisentrik hastalıkta IL-6 ve HHV-8’in rolü iyi tanımlanmıştır. Unisentrik hastalık tipik olarak lokalizedir, semptomlar minimaldir ve tek başına lokal tedavi uygulanır. Multisentrik hastalık sıklıkla HIV varlığında görülen sistemik bir hastalıktır ve klinik olarak yaygın lenfadenopati, splenomegali, anemi ve sistemik inflamatuar semptomlarla karakterizedir. Başlıca sistemik tedaviler uygulanır. Malign bir durum olmamakla birlikte bazı malign hastalıklarla (lenfomalar, POEMS sendromu, folliküler dendritik hücreli sarkomlar, paraneoplastik pemphigus, Kaposi sarkomu, amiloidoz) ile ilişkili olabilir. Bu yazıda nadir ama önemli bir hastalık olan Castleman hastalığı ile ilgili güncel bilgiler özetlenmiştir.

ANAHTAR KELİMELER: Lenfoproliferatif hastalık, Castleman, Tanı, 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

Anahtar Kelimeler

Lenfoproliferatif hastalık,Castleman,Tanı

Kaynakça

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Ayrıntılar

Birincil Dil

Türkçe

Konular

Sağlık Kurumları Yönetimi

Bölüm

Derleme

Yazarlar

Eren Gündüz
ESKISEHIR OSMANGAZI UNIV
Türkiye

Yayımlanma Tarihi

24 Eylül 2016

Gönderilme Tarihi

3 Ocak 2017

Kabul Tarihi

8 Ağustos 2016

Yayımlandığı Sayı

Yıl 2016 Cilt: 38 Sayı: 3

DOI

https://doi.org/http://dx.doi.org/10.20515/otd.53812

IZ

https://izlik.org/JA72RA96KC

Kaynak Göster

RIS / Bibtex

APA

Gündüz, E. (2016). NADİR BİR LENFOPROLİFERATİF HASTALIK: CASTLEMAN HASTALIĞI. Osmangazi Tıp Dergisi, 38(3), 6-16. https://doi.org/http://dx.doi.org/10.20515/otd.53812

AMA

1.Gündüz E. NADİR BİR LENFOPROLİFERATİF HASTALIK: CASTLEMAN HASTALIĞI. Osmangazi Tıp Dergisi. 2016;38(3):6-16. doi:http://dx.doi.org/10.20515/otd.53812

Chicago

Gündüz, Eren. 2016. “NADİR BİR LENFOPROLİFERATİF HASTALIK: CASTLEMAN HASTALIĞI”. Osmangazi Tıp Dergisi 38 (3): 6-16. https://doi.org/http://dx.doi.org/10.20515/otd.53812.

EndNote

Gündüz E (01 Eylül 2016) NADİR BİR LENFOPROLİFERATİF HASTALIK: CASTLEMAN HASTALIĞI. Osmangazi Tıp Dergisi 38 3 6–16.

IEEE

[1]E. Gündüz, “NADİR BİR LENFOPROLİFERATİF HASTALIK: CASTLEMAN HASTALIĞI”, Osmangazi Tıp Dergisi, c. 38, sy 3, ss. 6–16, Eyl. 2016, doi: http://dx.doi.org/10.20515/otd.53812.

ISNAD

Gündüz, Eren. “NADİR BİR LENFOPROLİFERATİF HASTALIK: CASTLEMAN HASTALIĞI”. Osmangazi Tıp Dergisi 38/3 (01 Eylül 2016): 6-16. https://doi.org/http://dx.doi.org/10.20515/otd.53812.

JAMA

1.Gündüz E. NADİR BİR LENFOPROLİFERATİF HASTALIK: CASTLEMAN HASTALIĞI. Osmangazi Tıp Dergisi. 2016;38:6–16.

MLA

Gündüz, Eren. “NADİR BİR LENFOPROLİFERATİF HASTALIK: CASTLEMAN HASTALIĞI”. Osmangazi Tıp Dergisi, c. 38, sy 3, Eylül 2016, ss. 6-16, doi:http://dx.doi.org/10.20515/otd.53812.

Vancouver

1.Eren Gündüz. NADİR BİR LENFOPROLİFERATİF HASTALIK: CASTLEMAN HASTALIĞI. Osmangazi Tıp Dergisi. 01 Eylül 2016;38(3):6-16. doi:http://dx.doi.org/10.20515/otd.53812