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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.

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Relaps veya refrakter akut lösemide klofarabin bazlı kurtarma tedavilerinin genel yanıt oranları %17-48 arasında değişmektedir. Çalışmamızda relaps veya refrakter akut lösemi tanılı hastalarda klofarabin bazlı kurtarma tedavisi sonrası yanıt oranları, enfeksiyon ile ilişkili komplikasyonları ve yan etkileri değerlendirildi. Ocak 2015 ile Aralık 2020 tarihleri arasında kliniğimizde klofarabin bazlı kurtarma tedavisi alan 12 hasta retrospektif olarak değerlendirildi. Hastaların %58 (n=7)’i erkek olup ortalama yaş 36,6±16,4 olarak saptandı. Hastaların %75 (n=9)’i akut lenfoblastik lösemi, %25 (n=3)’i akut myeloid lösemi tanısı ile takip edilmekteydi. Klofarabin öncesi almış oldukları kemoterapi rejim sayısı medyan 3 idi. Hastaların %66 (n=8)’sı son aldığı tedaviye refrakter, %33 (n=4)’ü erken nüks ile başvurdu. Hastalar klofarabin 22,5 mg/m2 + sitozin arabinozid 1000 mg/m2 dozunda intravenöz 1-5. gün olacak şekilde tedavi aldılar. Tedavi esnasında tüm hastalar febril nötropeni ile komplike oldu, hastaların %58 (n=7)’inde sepsis gelişti. Ciddi (grade 3-4) non- hematolojik yan etkiler arasında hepatotoksisite %33, diyare %25, bulantı-kusma %16, mukozit %16 civarındaydı. Klofarabine bağlı nörotoksisite izlenmedi. Hastaların birinde tam yanıt (%8) elde edildi. Hastaların %33 (n=4)’ü tedaviye yanıtsızdı. İlk 30 gün içindeki mortalite oranı %50 (n=6) idi. Mortal seyreden hastaların bir tanesinde erken ölüm (<2 hafta) oldu. Refrakter veya erken nüks akut lösemi hastaları için esas amaç kurtarma tedavisi sonrası allojenik kemik iliği nakline ilerlemektir. Her ne kadar kemik iliği nakli ile kür sağlanabilse de 4 yıllık sağ kalım %25-30’u geçmemektedir. Dolayısıyla relaps veya refrakter akut lösemi hastalarında günümüzde halen güçlü ve etkili bir kurtarma tedavi rejimine ihtiyaç duyulmaktadır.

• ÖZ

Objective: The aim of the present study was to evaluate the efficacyand safety of eltrombopag, an oral thrombopoietin receptor agonist,in patients with chronic immune thrombocytopenia (ITP).Materials and Methods: A total of 285 chronic ITP patients (187women, 65.6%; 98 men, 34.4%) followed in 55 centers were enrolledin this retrospective cohort. Response to treatment was assessedaccording to platelet count (/mm3) and defined as complete (plateletcount of >100,000/mm3), partial (30,000-100,000/mm3 or doublingof platelet count after treatment), or unresponsive (<30,000/mm3).Clinical findings, descriptive features, response to treatment, and sideeffects were recorded. Correlations between descriptive, clinical, andhematological parameters were analyzed.Results: The median age at diagnosis was 43.9±20.6 (range: 3-95)years and the duration of follow-up was 18.0±6.4 (range: 6-28.2)months. Overall response rate was 86.7% (n=247). Complete andpartial responses were observed in 182 (63.8%) and 65 (22.8%)patients, respectively. Thirty-eight patients (13.4%) did not respondto eltrombopag treatment. For patients above 60 years old (n=68),overall response rate was 89.7% (n=61), and for those above 80 years old (n=12), overall response rate was 83% (n=10). Consideringthrombocyte count before treatment, eltrombopag significantlyincreased platelet count at the 1st, 2nd, 3rd, 4th, and 8th weeks oftreatment. As the time required for partial or complete responseincreased, response to treatment was significantly reduced. The timeto reach the maximum platelet levels after treatment was quitevariable (1-202 weeks). Notably, the higher the maximum plateletcount after eltrombopag treatment, the more likely that side effectswould occur. The most common side effects were headache (21.6%),weakness (13.7%), hepatotoxicity (11.8%), and thrombosis (5.9%).Conclusion: Results of the current study imply that eltrombopag isan effective therapeutic option even in elderly patients with chronicITP. However, patients must be closely monitored for response andside effects during treatment. Since both response and side effectsmay be variable throughout the follow-up period, patients should beevaluated dynamically, especially in terms of thrombotic risk factors.