Mehmet Can Ugur

Primary immune thrombocytopenia (pITP) is an acquired bleeding disorder related with mainly decreased number of platelets due to destruction or impaired production of platelets. Clinical presentation of pITP can be multifaceted, and thrombotic events may rarely manifest. Thrombosis can develop with treatment or during the untreated period. The primary objective of the study was to examine the frequency of arterial thromboembolism (ATE) in patients with pITP. We also aimed to evaluate the risk factors in these patients and the effect of ITP treatments on ATE. The study was designed as a retrospective, multicenter, conducted under the Turkish Society of Hematology's Scientific Subcommittee on Hemostasis-Thrombosis. Patients over the age of 18 with pITP and who subsequently developed ATE while undergoing follow-up for pITP were evaluated. A total of 2,178 patients with pITP were screened, and 37 patients (1.7%) were observed to have ATE. The mean age was 62 years. Fifteen (40.5%) of the patients who developed ATE were not receiving pITP treatment at the time of thromboembolism. The patients receiving pITP treatment at the time of ATE, 9 patients (24.3%) were receiving eltrombopag and 10 patients (27%) were receiving corticosteroids. Compared to patients who did not develop ATE, multivariate analysis revealed that presence of hypertension, comorbidity, and the history of venous thromboembolism statistically significantly increased the risk of developing ATE (p: 0.008, 0.018, 0.038). It should be noted that the risk of ATE may increase in pITP patients both during and without treatment. It is important to inquire thoroughly about the presence of comorbidities, atherosclerotic risk factors, hypertension and history of thrombosis in patients at the initiation of treatment. Correctable risk factors should be addressed to minimize the number of risk factors present. The treatment of pITP shall be individualized and incorporate age-related disorders.

Introduction: This study aims to examine the relationship between self‐management skills and kinesiophobia in haemophilia patients. The findings of the study may contribute to the development of strategies that can help haemophilia patients overcome their fear of movement and improve their quality of life (QoL). Aim: This study is planned to examine the relationship between disease self‐management in haemophiliac individuals and kinesiophobia that may arise from intra‐articular bleeding associated with haemophilia. Methods: In this study, both descriptive and analytical research was conducted on individuals who are members of patient associations. Patient Identification Form, Chronic Disease Self‐Management Scale and Tampa Scale for Kinesiophobia were used as data collection tools. Data were collected face to face. Descriptive statistics methods (mean, standard deviation) were used in the evaluation of study data. Quantitative data comparisons were made with the Student's t ‐test for normally distributed parameters and the Mann–Whitney U test for non‐normally distributed parameters. When there were more than two groups, One‐Way ANOVA was used, and in advanced analyses, linear regression analysis was used. Results: A positive correlation was found between the ‘self‐stigmatization’ subdimension of the Chronic Disease Self‐Management Scale and kinesiophobia. Additionally, a negative correlation was observed between healing compliance and kinesiophobia levels. Flexibility in healing cracks was found in the reduction of kinesiophobia. Conclusion: Self‐management of the disease in hemophilic individuals affects compliance with treatment, kinesiophobia, activity level and QoL.

Background: Hereditary hemorrhagic telangiectasia (HHT) is a disease characterized by arteriovenous malformations and telangiectases, in which the endothelium and immune system play a role in the pathophysiology. Therefore, treatments with antiangiogenic properties which are also regarded as immunomodulators were demonstrated to play an important role in treatment. This systematic review aimed to gather the accumulated information of the use of thalidomide and its analogs in the treatment of HHT. Methods: In this systematic review, publications that were published up to March 2024 and met the inclusion criteria were compiled using the keywords ‘thalidomide’, ‘lenalidomide’, ‘pomalidomide’, ‘immunomodulatory drugs’ and ‘HHT’ in Medline and Scholars databases. Results: A total of 53 articles were evaluated and 15 were included in the study. Thalidomide was the predominant used agent and was observed to be used in patients with ages ranging from 37 to 77 years, with doses ranging from 50 to 200 mg daily, and the mean follow-up period was observed to be 6–60 months. Assessments regarding efficacy were based on the epistaxis severity score (ESS), hemoglobin level, and transfusion independence. While thalidomide showed significant efficacy, it also had an adverse event rate of any severity of up to 85% of patients. Use of lenalidomide to control bleeding in HHT was reported in a single case report, while the use of pomalidomide was observed to be investigated in Phase 1 and Phase 2 studies in patients aged 48 to 70 years, with doses ranging from 1 to 5 mg daily for 6–24 months. This treatment was reported to provide significant improvement in hemoglobin levels and ESS. Adverse events of any severity were observed at a frequency of 60–66%. Conclusions: Antiangiogenic agents such as thalidomide, lenalidomide, and pomalidomide may be effective in managing HHT. However, further studies are needed to optimize the timing, dose, and sequence.

Öz Giriş: Doğru hastaya, doğru kan ürününün uygun zamanda ve miktarda transfüzyonu hayat kurtarıcıdır. Ancak transfüzyona bağlı gelişen komplikasyonlar morbidite ve mortaliteye neden olabilmektedir. Bu komplikasyonların önlenmesi amacıyla hemovijilans programları geliştirilmiştir. Amaç: Bakırçay Üniversitesi Çiğli Eğitim ve Araştırma Hastanesi’nde 2018-2022 yılları arasında Hemovijilans Birimi tarafından kaydedilen transfüzyon ilişkili reaksiyonların retrospektif değerlendirilmesi amaçlanmıştır. Materyal-Metod: Hemovijilans Birimi’nde akut transfüzyon reaksiyonları için tutulan dosya ve bilgisayar kayıtları retrospektif incelenerek 2018-2022 yılına ait veriler kaydedilmiştir. Bulgular: Beş yılda toplam 35948 eritrosit, 11714 taze donmuş plazma (TDP), 2511 trombosit, 243 kriyopresipitat, 161 tam kan ve COVID-19 pandemisinin yaşandığı 2020-2021 yıllarında 605 immun plazma kullanılmıştı. Transfüzyon ilişkili reaksiyon prevelansı eritrosit için 100/100.000, TDP için 128/100.000 ve trombosit için 79/100.000 olarak saptandı. Toplam 36 hafif alerjik reaksiyon, 10 febril nonhemolitik transfüzyon reaksiyonu, 2 anafilaksi ve 1 transfüzyon ilişkili akut akciğeri hasarı görüldü. Sonuç: Hemovijilans programlarına olan farkındalığın artırılması, kan ürünlerinin doğru kullanılması ve transfüzyon ilişkili reaksiyonların önlenmesi açısından önemlidir.

Bruton's tyrosine kinase (BTK) inhibition in cardiac tissue causes inhibition of the PI3K-AKT signaling pathway, which is responsible for protecting cardiac tissue during stress. Therefore, there is an increase in the risk of arrhythmia. This risk can be predicted with the Age-Creatinine-Ejection Fraction (ACEF) score, which is a simple scoring system that can be calculated from age, creatinine and ejection fraction components. Patients diagnosed with Chronic Lymphocytic Leukemia (CLL) and receiving Ibrutinib treatment for at least 1 year were evaluated with echocardiography and holter electrocardiography, and the results were compared with the control group of CLL patients who did not receive treatment. When arrhythmia development of the patients was evaluated, no statistically significant difference was found between the control and Ibrutinib groups in terms of other types of arrhythmias exact Paroxismal Atrial Fibrillation (PAF). PAF was found to be 8% versus 22% (p-value: 0.042) in the Ibrutinib non-users and users groups. In patients receiving ibrutinib, ACEF score of >1.21 predicted the development of PAF with 77% sensitivity and 75% specificity (Receiver Operating Characteristic [ROC] area under curve: 0.830, 95% CI: 0.698-0.962, P<0.001). The ACEF risk score can be used as a risk score that predicts the development of PAF in patients diagnosed with CLL and planned to start ibrutinib.

Objective: We present data of patients with relapsed/ refractory T cell lymphomas treated with brentuximab vedotin in real-world practice. Patients and methods: This study is an observational, multi-center, retrospective study. The data of patients (n: 17) treated with brentuximab vedotin alone from January 2014 until July 2020 in thirteen centers from Turkey were collected. Results: Bv was given as salvage chemotherapy to 17 patient with median age 53. Nine (52.9%) patients had diagnosis of peripheral T cell lymphoma, not otherwise specified; 8 (47.1%) patients had anaplastic large T cell lymphoma. The median follow-up of the cohort was 20 months. Nine (52.9%) patients had complete response, 5 (29.5%) had partial response, 3 (17.6%) had progressive disease. The safety results aligned with the established profile of brentuximab vedotin, included 2 pneumonia and 1 thrombocytopenia with grade 4. Median progression free survival of the cohort was10 months. Brentuximab vedotin cycle and response to brentuximab vedotin therapy were found to have effect on the univariate analysis. Conclusion: In patients with relapsed/ refractory T cell lymphomas, brentuximab vedotin seems to have convincing antitumor activity with favorable safety profile.

Giriş: Endotelyal hasar ve hiperkoagülabilite gelişiminde anahtar rol oynadığı bilinen trombosit aktivasyonu, PTE gelişiminde de önemli bir yere sahiptir. Bu nedenle trombosit indeksleri PTE tanısında yol gösterici olabilir. Amaç: Bu araştırmada, trombosit indekslerinin pulmoner tromboemboli tanısı için öngörüdürücülüğünün araştırılması amaçlanmıştır. Materyal ve Metod: 2020-2023 yılları arasında Aliağa Devlet Hastanesi’nde Bilgisayarlı Tomografi Pulmoner Anjiografi ile tromboemboli tanısı alan 18 yaş üstü hastaların verileri retrospektif olarak tarandı. Herhangi bir ek hastalık ve ilaç kullanım öyküsü olmayan, yaş ve cinsiyet bakımından pulmoner tromboemboli koluna benzer özelliklerde katılımcılardan oluşan bir kontrol grubu oluşturuldu. Bulgular: Çalışmaya 33 PTE grubu, 35 kontrol grubu olmak üzere toplam 68 hasta dahil edildi. PTE kolunda 18 kadın (%54,5), 15 erkek (%45,5); kontrol grubunda 20 kadın (%57,1) 15 erkek (%42,9) yer aldı. PTE ve kontrol grubunun yaş ortalaması sırasıyla 70,9 (40-91) ve 69,2 (49-82) idi. Grupların tanı anındaki trombosit sayısı, MPV, PCT ve PLR karşılaştırıldığında gruplar arasında istatistiksel olarak anlamlı fark saptanmadı. Ancak PTE grubunda PDW değeri istastiksel olarak daha yüksek bulundu (p=0,001). PDW için hesaplanan cut-off değeri 16,35% (%95 Cl, AUC:0,790 [0,675-0,905], sensivite %72,7, spesifite %71,4) saptanmıştır. Sonuç: Tromboz gelişiminde anahtar rol oynayan trombosit aktivasyonunu gösteren trombosit indeksleri, PTE tanısında prediktif değer taşıyabilir. Çalışmamızda PDW değeri PTE gelişimi ile ilişkili bulunmuştur. Bu verilerin desteklenmesi için daha kapsamlı çalışmalara ihtiyaç vardır.