NADİR BİR LENFOPROLİFERATİF HASTALIK: CASTLEMAN HASTALIĞI

Year 2016, Volume: 38 Issue: 3, 6 - 16, 24.09.2016

Eren Gündüz

Abstract

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

 

Keywords

Lenfoproliferatif hastalık, Castleman, Tanı

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