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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.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.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.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.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.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.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.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.

© 2022 by Turkish Society of Hematology.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.

8th International Eurasian Hematology Oncology Congress (EHOC) -- OCT 18-21, 2017 -- Istanbul, TURKEY WOS: 000416742600076 …

8th International Eurasian Hematology Oncology Congress (EHOC) -- OCT 18-21, 2017 -- Istanbul, TURKEY WOS: 000416742600077 …

8th International Eurasian Hematology Oncology Congress (EHOC) -- OCT 18-21, 2017 -- Istanbul, TURKEY WOS: 000416742600129 …

8th International Eurasian Hematology Oncology Congress (EHOC) -- OCT 18-21, 2017 -- Istanbul, TURKEY WOS: 000416742600130 …

8th International Eurasian Hematology Oncology Congress (EHOC) -- OCT 18-21, 2017 -- Istanbul, TURKEY WOS: 000416742600131 …

In this study it has been aimed to scan the most frequently observed types of mutations in imanitib treated Philadelphia positive (Ph+) Chronic Myelositer Leukemia (CML) patients, namely the T315I, F317L, Y253H, and the P-Ioop phosphate binding region E255K mutations, in order to demonstrate any significant correlation between the presence of these mutations and the estimations on the relevant clinical parameters. Fifty four chronic phase (CP) CML patients attending two separate centers between 2000 and 2008 were included in this study, after scanning by the ASO-PCR method, to be positive for T315I, F317L, Y253H and E255K mutations in the BCR-ABL gene. Twenty of the patients were men and 42 were women with a median age of 44.5 (19-78). According to the Sokol classification for diagnosis, 12 cases (22.2%) were in the low risk, 26 (48.1%) were in the middle risk and 16 (29.6%) were in the high risk group. Median time of imatinib usage was 1.8 (0.3-7) years. Response to imatinib treatment was optimal in 24 (%44.4) of the patients and suboptimal in 10 (18.5%) of the patients, while 20 (37%) showed resistance. Expected values in 7 years were found to be 96% for median overall survival (OS) and 80% for progression-free survival (PFS). Scanned mutations were observed in 18 (33.3%) patients and T315I and F317L together were detected in 2 patients. Mutations were detected in 60% (p=0.004) of the imatinib resistant patients and the 7-year PFS in this group was 62%, while it was 90% (p=0.041) in patients without detected mutations. While patients with T3151 mutation were recommended allogeneic stem cell transplantation, patients with F317L mutation were started on nilotinib and dasatinib treatment. To determine the optimal treatment type; resistance should be identified in early stages in CML patients and the rational treatment should be planned according to the determined mutation type.

Kronik Myelositer Lösemi (KML); Philadelphia translokasyonunun t (9;22) (q34;q11) varlığı ile karakterize BCR ve ABL genlerinin füzyonu ile sonuçlanan malign hemotopoetik bir kök hücre hastalığıdır. KML'de İmatinib tedavisi etkin bir tedavi olmasına karşın, hastalarda gözlenen en önemli sorun birincil ve zamanla kazanılmış dirençtir.Bu çalışmada 2 merkezde 2000-2008 tarihleri arasında izlenen 54 kronik faz KML hastasında; literatürde ABL geninde en sık gözlenen (imatinib bağlanma bölgesinde bulunan) T315I, F317L ve Y253H ve genin P-Ioop fosfat bağlanma bölgesinde bulunan E255K mutasyonları Allel Spesifik Oligonükleotit-Polimeraz Zincir Reaksiyonu (ASO-PCR) ile taranmış ve mutasyon doğrulamaları SSCP yöntemi ile yapılmıştır. Belirlenen mutasyonlar klinik verilerle değerlendirilmiştir.Hastaların 12'si erkek, 42'si kadın olup medyan yaş 44.5 (19?78)'dur. Tanıda Sokol risk sınıflamasına göre; hastaların 12'si (%22.2) düşük riskli, 26'sı (% 48.1) orta riskli ve 16'sı (%29.6) yüksek riskli gruptadır. Medyan imatinib kullanım süresi 1.8 (0.3?7) yıldır. İmatinib tedavi yanıtı değerlendirildiğinde; hastaların 24'ü (%44.4) optimal yanıtlı, 10'u (%18.5) suboptimal yanıtlı 20'si (%37) ise yanıtsız olarak değerlendirilmiştir. Medyan total ve progresyonsuz yaşam sürelerine henüz ulaşılmamış olup, 7 yılda beklenen total sağkalım %96, progresyonsuz sağkalım %80 bulunmuştur. Taranan mutasyonlar hastaların 18'inde (%33.3) belirlenmiş olup sırası ile T315I 11 (20.3) ve F317L 9 (%16.6) hastada saptanırken,Y253H ve E255K mutasyonları bulunmamıştır. Mutasyon belirlenen hastaların 2 (%3.7)'sinde T315I ve F317L mutasyonu birlikteliği saptanmıştır. Yanıtsız hastaların %60'ın da mutasyon saptanmıştır (p=0.004). Mutasyon saptanan hastalarda 7 yıllık PFS %62 mutasyon saptanmayanlarda ise % 90 olarak bulunmuştur (p=0.041). T315I mutasyonu saptanan hastalar AKİT'e yönlendirilirken F317L mutasyonu olanlar da nilotinib veya dasatinib tedavisine geçilmiştir.Optimal tedavi şeklinin belirlenmesi açısından; KML hastalarında direnç erken dönemde tanımlanmalı, mutasyon tipi belirlenerek mutasyona göre rasyonel tedavi şekli planlanmalıdır.Anahtar Kelimeler: Kronik Myeloid Lösemi, BCR-ABL Nokta Mutasyonu, İmatinibBu çalışma Gaziantep Üniversitesi Araştırma Fonu tarafından desteklenmiştir. Proje no TF 08.01 Chronic Myeloid Leukemia (CML); is a Malign Hemotopoetic stem cell disease which concluded by the fusions of BCR and ABL genes. It is characterized by the existence of the Philedelphia translocations t (9;22) (q34;q11). In spite of Imatinib is an effective treatment for CML, the important problem that is seen by at the patients is primary and secondary resistance.In this study, the 54 chronic phase CML patients observed in two centre between 2000-2008, mostly observed (in the place of imatinib connection) T315I, F317L, Y253H mutations in ABL gene and E255K mutation observed in the gene?s connection place of P-loop phosphate are scanned by the method of allel specific oligonükleotit-polimeraz chain reaction (ASO-PCR) and proved by the SSCP method.12 of patients are men and 42 of patients are women and the median age of the patients are 44.5 (19-78). According to the Sokol classification in diagnosis; 12 of patients (22.2%) are low risky, 26 of them (48.1%) are middle risky and 16 of them (29.6%) are in high risky group. Median imatinib usage time is 1.8 (0.3-7) year. When imatinib treatment response is evaluated; the 24 (%44.4) of the patients are optimal response, 10 (18.5%) of the patients are suboptimal response and 20 (37%) of them have resistance.Median overall survival (OS) and progression free survival (PFS) can not be obtained yet. Expected values in 7 years are found as 96% for OS and 80% for PFS. Scanned mutations are observed in 18 (33.3%) of the patients. Despite T315I is observed in 11 (20.3%) and F317L is in 9 (16.6%) patients, Y253H and E255K mutations did not observe. In 2 (3.7%) of the mutation determined patients, T315I and F317L mutations are observed together. Mutations are determined in 60% of the resistance patients (p=0.004). 7 years PFS is found as 62% in mutation determined patients and 90% in mutation undetermined patients (p=0.041). While T3151 mutation observed patients guided to AKIT, F317L mutation observed patients are started to nilotinib and dasatinib treatment.To determine the optimal treatment type; resistance should be identified in early stages in CML patients, rational treatment type should be planned according to the determined mutation type.Keywords: Chronic Myeloid Leukemia, BCR-ABL Nokta Mutasyonu, ImatinibThis study is supported by the Gaziantep University Research Fund (TF 08.01). 71