Castleman Disease: A Multicenter Case Series from TurkeyEren Gündüz1, Hakkı Onur Kırkızlar2, Elif Gülsüm Ümit2, Sedanur Karaman Gülsaran2, Vildan Özkocaman3, Fahir Özkalemkaş3, Ömer Candar3, Tugrul Elverdi4, Selin Küçükyurt4, Semra Paydaş5, Özcan Çeneli6, Sema Karakuş7, Senem Maral8, Ömer Ekinci9, Yıldız İpek10, Cem Kis11, Zeynep Tuğba Güven12, Aydan Akdeniz13, Tiraje Celkan14, Ayşe Hilal Eroğlu Küçükdiler15, Gülsüm Akgün Çağlıyan16, Ceyda Özçelik Şengöz17, Ayşe Karataş18, Tuba Bulduk19, Alper Özcan12, Fatma Burcu Belen Apak7, Aylin Canbolat20, İbrahim Kartal21, Hale Ören22, Ersin Töret1, Gül Nihal Özdemir14, Şule Mine Bakanay Öztürk231Eskişehir Osmangazi University Faculty of Medicine, Department of Hematology, Eskişehir, Turkey
2Trakya University Faculty of Medicine, Department of Hematology, Edirne, Turkey
3Uludağ University Faculty of Medicine, Department of Hematology, Bursa, Turkey
4İstanbul University-Cerrahpaşa Cerrahpaşa Faculty of Medicine, Department of Hematology, İstanbul, Turkey
5Çukurova University Faculty of Medicine, Department of Hematology, Adana, Turkey
6Necmettin Erbakan University Meram Faculty of Medicine, Department of Hematology, Konya, Turkey
7Ankara Başkent University Faculty of Medicine, Department of Hematology, Ankara, Turkey
8University of Health Sciences Turkey Ankara Dışkapı Yıldırım Beyazıt Training and Research Hospital, Clinic of Hematology, Ankara, Turkey
9Fırat University Faculty of Medicine, Department of Hematology, Elazığ, Turkey
10Kartal Dr. Lütfi Kırdar City Hospital, Clinic of Hematology, İstanbul, Turkey
11Adana Başkent University Faculty of Medicine, Department of Hematology, Adana, Turkey
12Erciyes University Faculty of Medicine, Department of Hematology, Kayseri, Turkey
13Mersin University Faculty of Medicine, Department of Hematology, Mersin, Turkey
14İstinye University Faculty of Medicine, Department of Pediatric Hematology, İstanbul, Turkey
15Adnan Menderes University Faculty of Medicine, Department of Hematology, Aydın, Turkey
16Pamukkale University Faculty of Medicine, Department of Hematology, Denizli, Turkey
17Karadeniz Technical University Faculty of Medicine, Department of Hematology, Trabzon, Turkey
18Hacettepe University Faculty of Medicine, Department of Hematology, Ankara, Turkey
19University of Health Sciences Turkey Gülhane Training and Research Hospital, Clinic of Hematology, Ankara, Turkey
20İstanbul Medeniyet University Faculty of Medicine, Department of Pediatric Hematology, İstanbul, Turkey
21Ondokuz Mayıs University Faculty of Medicine, Department of Pediatric Hematology, Samsun, Turkey
22Dokuz Eylül University Faculty of Medicine, Department of Pediatric Hematology, İzmir, Turkey
23Ankara Yıldırım Beyazıt University Faculty of Medicine, Department of Hematology, Ankara, Turkey
Objective: Castleman disease (CD) is a rare disease also known as angiofollicular lymph node hyperplasia. The two main histological subtypes are the hyaline vascular and plasma cell variants. It is further classified as unicentric CD (UCD) or multicentric CD (MCD) according to the anatomical distribution of the disease and the number of lymph nodes involved. The aim of this multicenter study was to evaluate all cases of CD identified to date in Turkey to set up a national registry to improve the early recognition, treatment, and follow-up of CD.
Materials and Methods: Both adult (n=130) and pediatric (n=10) patients with lymph node or involved field biopsy results reported as CD were included in the study. Patients’ demographic information, clinical and laboratory characteristics, imaging study results, treatment strategies, and clinical outcomes were evaluated retrospectively.
Results: A total of 140 patients (69 male and 71 female) with a diagnosis of UCD (n=73) or MCD (n=67) were included. The mean age was 39 years in the UCD group and 47 years in the MCD group. Female patients were more common in the UCD group. The most common histological subtype was hyaline vascular for both UCD and MCD patients. Asymptomatic patients were more common in the UCD group. Anemia, elevations of acute phase reactants, and hypoalbuminemia were more common in the MCD group. The most commonly used treatment strategies for UCD were surgical excision, rituximab, and radiotherapy, respectively. All UCD patients were alive at a median of 19.5 months of follow-up. The most commonly used treatment strategies for MCD were methyl prednisolone, R-CHOP, R-CVP, and rituximab. Thirteen MCD patients had died at a median of 34 months of follow-up.
Conclusion: This study is important in presenting the patient characteristics and treatment strategies for CD from Turkey, with the potential of increasing awareness about CD. Treatment data may help in making decisions, particularly in countries that do not have access to siltuximab. However, larger prospective studies are needed to make definitive conclusions.
Keywords: Castleman disease, Unicentric, Multicentric, Treatment
Castleman Hastalığı: Türkiye’den Çok Merkezli Bir Olgu SerisiEren Gündüz1, Hakkı Onur Kırkızlar2, Elif Gülsüm Ümit2, Sedanur Karaman Gülsaran2, Vildan Özkocaman3, Fahir Özkalemkaş3, Ömer Candar3, Tugrul Elverdi4, Selin Küçükyurt4, Semra Paydaş5, Özcan Çeneli6, Sema Karakuş7, Senem Maral8, Ömer Ekinci9, Yıldız İpek10, Cem Kis11, Zeynep Tuğba Güven12, Aydan Akdeniz13, Tiraje Celkan14, Ayşe Hilal Eroğlu Küçükdiler15, Gülsüm Akgün Çağlıyan16, Ceyda Özçelik Şengöz17, Ayşe Karataş18, Tuba Bulduk19, Alper Özcan12, Fatma Burcu Belen Apak7, Aylin Canbolat20, İbrahim Kartal21, Hale Ören22, Ersin Töret1, Gül Nihal Özdemir14, Şule Mine Bakanay Öztürk231Eskişehir Osmangazi University Faculty of Medicine, Department of Hematology, Eskişehir, Turkey
2Trakya University Faculty of Medicine, Department of Hematology, Edirne, Turkey
3Uludağ University Faculty of Medicine, Department of Hematology, Bursa, Turkey
4İstanbul University-Cerrahpaşa Cerrahpaşa Faculty of Medicine, Department of Hematology, İstanbul, Turkey
5Çukurova University Faculty of Medicine, Department of Hematology, Adana, Turkey
6Necmettin Erbakan University Meram Faculty of Medicine, Department of Hematology, Konya, Turkey
7Ankara Başkent University Faculty of Medicine, Department of Hematology, Ankara, Turkey
8University of Health Sciences Turkey Ankara Dışkapı Yıldırım Beyazıt Training and Research Hospital, Clinic of Hematology, Ankara, Turkey
9Fırat University Faculty of Medicine, Department of Hematology, Elazığ, Turkey
10Kartal Dr. Lütfi Kırdar City Hospital, Clinic of Hematology, İstanbul, Turkey
11Adana Başkent University Faculty of Medicine, Department of Hematology, Adana, Turkey
12Erciyes University Faculty of Medicine, Department of Hematology, Kayseri, Turkey
13Mersin University Faculty of Medicine, Department of Hematology, Mersin, Turkey
14İstinye University Faculty of Medicine, Department of Pediatric Hematology, İstanbul, Turkey
15Adnan Menderes University Faculty of Medicine, Department of Hematology, Aydın, Turkey
16Pamukkale University Faculty of Medicine, Department of Hematology, Denizli, Turkey
17Karadeniz Technical University Faculty of Medicine, Department of Hematology, Trabzon, Turkey
18Hacettepe University Faculty of Medicine, Department of Hematology, Ankara, Turkey
19University of Health Sciences Turkey Gülhane Training and Research Hospital, Clinic of Hematology, Ankara, Turkey
20İstanbul Medeniyet University Faculty of Medicine, Department of Pediatric Hematology, İstanbul, Turkey
21Ondokuz Mayıs University Faculty of Medicine, Department of Pediatric Hematology, Samsun, Turkey
22Dokuz Eylül University Faculty of Medicine, Department of Pediatric Hematology, İzmir, Turkey
23Ankara Yıldırım Beyazıt University Faculty of Medicine, Department of Hematology, Ankara, Turkey
Amaç: Anjiyofolliküler lenf nodu hiperplazisi olarak da bilinen Castleman hastalığı (CH), nadir bir hastalık olup başlıca hiyalin vasküler ve plazma hücreli olmak üzere 2 histolojik alt tipi vardır. Hastalığın anatomik yayılımı ve tutulan lenf nodu bölgelerinin sayısına göre unisentrik (UCH) ya da multisentrik (MCH) olarak sınıflandırılır. Bu çok merkezli çalışmanın amacı bugüne kadar Türkiye’de tanımlanan tüm CH olgularını tanımlamak, ulusal bir veri tabanı oluşturarak CH’de erken tanı, tedavi ve takip sürecine katkı sağlamaktır.
Gereç ve Yöntem: Çalışmaya lenf nodu ya da tutulan alandan yapılmış biyopsi sonucu CH olarak rapor edilen hem erişkin (n=130) hem de pediyatrik (n=10) hastalar dahil edildi. Hastaların demografik bilgileri, klinik ve laboratuvar özellikleri, görüntüleme bulguları, aldıkları tedaviler ve tedavi sonuçları geriye dönük olarak değerlendirildi.
Bulgular: Dahil edilen 140 hastanın 69’u kadın, 71’i erkekti. Yetmiş üç hasta UCH, 67 hasta MCH olarak sınıflandırılmıştı. Yaş ortalaması UCH’de 39, MCH’de 47 yıl idi. Kadın hastalar UCH’de daha fazlaydı. Hem UCH hem de MCH için en sık histolojik alt tip hiyalin vaskülerdi. Asemptomatik hastalar UCH’de daha fazlaydı. Anemi, akut faz reaktanı yüksekliği ve hipoalbuminemi MCH’de daha sıktı. UCH’de en sık uygulanan tedaviler sırasıyla cerrahi eksizyon, rituksimab ve radyoterapiydi. Median 19,5 aylık takipte tüm UCH’li hastalar hayattaydı. MCH’de 1. basamak tedaviler metil prednizolon, R-CHOP, R-CVP ve rituksimab idi. Median 34 aylık takipte 13 MCH’li hasta kaybedilmişti.
Sonuç: Çalışmamız Türkiye’deki CH hastalarının özellikleri ve tedavi yaklaşımlarını yansıtması açısından önemli olup hastalıkla ilgili farkındalığın arttırılması potansiyeline sahiptir. Tedavi verileri özellikle ülkemizde olduğu gibi siltuksimaba ulaşımı zor olan ülkelerde tedavi seçimi konusunda fikir verebilir. Kesin sonuçlar çıkarmak için büyük ölçekli prospektif çalışmalara ihtiyaç vardır.
Anahtar Kelimeler: Castleman hastalığı, Unisentrik, Multisentrik, Tedavi
Eren Gündüz, Hakkı Onur Kırkızlar, Elif Gülsüm Ümit, Sedanur Karaman Gülsaran, Vildan Özkocaman, Fahir Özkalemkaş, Ömer Candar, Tugrul Elverdi, Selin Küçükyurt, Semra Paydaş, Özcan Çeneli, Sema Karakuş, Senem Maral, Ömer Ekinci, Yıldız İpek, Cem Kis, Zeynep Tuğba Güven, Aydan Akdeniz, Tiraje Celkan, Ayşe Hilal Eroğlu Küçükdiler, Gülsüm Akgün Çağlıyan, Ceyda Özçelik Şengöz, Ayşe Karataş, Tuba Bulduk, Alper Özcan, Fatma Burcu Belen Apak, Aylin Canbolat, İbrahim Kartal, Hale Ören, Ersin Töret, Gül Nihal Özdemir, Şule Mine Bakanay Öztürk. Castleman Disease: A Multicenter Case Series from Turkey. Turk J Hematol. 2022; 39(2): 130-135
Corresponding Author: Eren Gündüz |
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