Castleman disease: a multicenter case series from Turkey
Kirkizlar, Hakki Onur
Umit, Elif Gulsum
Karaman Gulsaran, Sedanur
MetadataShow full item record
CitationGündüz E, Kırkızlar HO, Ümit EG, Karaman Gülsaran S, Özkocaman V, Özkalemkaş F, Candar Ö, Elverdi T, Küçükyurt S, Paydaş S, Ceneli Ö, Karakuş S, Maral S, Ekinci Ö, İpek Y, Kis C, Güven ZT, Akdeniz A, Celkan T, Eroğlu Küçükdiler AH, Akgün Çağlıyan G, Özçelik Şengöz C, Karataş A, Bulduk T, Özcan A, Belen Apak FB, Canbolat A, Kartal İ, Ören H, Töret E, Özdemir GN, Bakanay Öztürk ŞM. Castleman Disease: A Multicenter Case Series from Turkey. Turk J Haematol. 2022 Feb 18.
Objective: Castleman disease (CD) is a rare disease also known as angiofollicular lymph node hyperplasia. Two main histological subtypes are hyaline vascular and plasma cell variant. It is classified as unicentric (UCD) or multicentric CD (MCD) according to the anatomical distribution of disease and the number of lymph nodes involved. The aim of this multicenter study is to evaluate all cases of CD identified to date in Turkey to set up a national registry aiming to improve CD early recognition, treatment and follow-up. Materials and methods: Both adult (n=130) and pediatric (n=10) patients with a lymph node or involved field biopsy result reported as CD were included in the study. Patients' demographic information, clinical and laboratory characteristics, imaging studies, treatment strategies and clinical outcome were evaluated retrospectively. Results: 140 patients (69 male and 71 female) with a diagnosis of UCD (n = 73) and MCD (n = 67) were included. Mean age was 39 in UCD and 47 in MCD. Female patients were more common in UCD. The most common histologic subtype was hyaline vascular for both UCD and MCD. Asymptomatic patients were more common in UCD. Anemia, elevation of acute phase reactants and hypoalbuminemia were more common in MCD. Most commonly used treatment strategies in UCD were surgical excision, rituximab and radiotherapy respectively. All UCD patients were alive at a median 19.5 month follow up. Most commonly used treatment strategies in MCD were methyl prednisolone, R-CHOP, R-CVP and rituximab. Thirteen patients died at a median 34 month follow up. Conclusion: Our study is important due to representing the patient characteristics and treatment strategies from Turkey and have a potential of increasing awareness about CD. Treatment data may help making decision particularly in countries who can not get access to siltuximab. Larger prospective studies are needed to make definitive conclusions.