Children with Behçet Disease-associated Thrombosis: A Single-Center Experience

Behçet disease (BD) is a systemic vasculitis that can be complicated with thrombosis, which is an important cause of mortality and morbidity. The course of BD is more severe, and the diagnosis is usually delayed. In children, thrombosis associated with BD is very rare. In this study, we aimed to evaluate the characteristics of children with BD complicated with thrombosis. Forty-six patients with BD who were followed-up at a pediatric rheumatology department between January 2012 and September 2019 were evaluated retrospectively. Thrombosis was detected in 10 patients (21.7%), and it was the first sign of BD in 7 patients. Four patients had cerebral sinus venous thrombosis, 4 patients had deep-vein thrombosis, 1 patient had renal vein thrombosis, 1 had pulmonary artery thrombosis, and 1 had intracardiac thrombosis. None of the patients had arterial thrombosis. All patients had received anticoagulant therapy with immunosuppressive treatment. Any complication due to anticoagulant therapy was not detected. One patient had recurrent thrombosis, and none of the patients died during follow-up. Vasculitic diseases such as BD may cause a predisposition to thrombosis, and thrombosis might be the first sign of BD. Therefore, in children presenting with unprovoked thrombosis, BD should also be investigated. © 2021 Lippincott Williams and Wilkins. All rights reserved

Successful Off-Label Use of Recombinant Factor VIIa and Coil Embolization in an Adolescent with Massive Hemoptysis Due to Invasive Pulmonary Aspergillosis

Invasive fungal infections have turned out to be a significant cause of morbidity and mortality in pediatric patients with malignant disorders. Massive hemoptysis, a rare complication of invasive pulmonary aspergillosis, may threaten the lives of patients, usually during the resolution of neutropenia. In this report, we describe a patient with massive hemoptysis due to invasive pulmonary aspergillosis whose bleeding was controlled successfully with off-label use of recombinant factor VIIa and subsequent coil embolization of the right pulmonary artery

Venous Thromboembolism after Allogeneic Pediatric Hematopoietic Stem Cell Transplantation: A Single-Center Study

Objective: Venous thromboembolism (VTE) in children who undergo hematopoietic stem cell transplantation (HSCT) has high morbidity. The aim of this study is to assess the incidence of VTE in allogeneic pediatric HSCT recipients and the contribution of pretransplant prothrombotic risk factors to thrombosis. Materials and Methods: We retrospectively evaluated 92 patients between April 2010 and November 2012 undergoing allogeneic HSCT who had completed 100 days post-HSCT. Before HSCT, coagulation profiles; acquired and inherited prothrombotic risk factors including FV G1691A (factor V Leiden), prothrombin G20210A, methylenetetrahydrofolate reductase (MTHFR) C677T, and MTHFR A1298C mutations; and serum homocysteine and lipoprotein (a), plasma antithrombin III, protein C, and protein S levels were obtained from all patients. Results: In the screening of thrombophilia, 8 patients (9%) were heterozygous for factor V Leiden, 5 (6%) were homozygous for MTHFR 677TT, 12 (14%) were homozygous for MTHFR 1298CC, and 2 (2%) were heterozygous for prothrombin G20210A mutation. We observed VTE in 5 patients (5.4%); a prothrombotic risk factor was found in 3 out of these 5 patients, while 4 out of 5 patients had central venous catheters. It was determined there was no significant relationship between VTE and inherited prothrombotic risk factors. Conclusion: VTE after HSCT seems to be a low-frequency event that may be due to low-dose, low-molecular-weight heparin prophylaxis, and the role of inherited prothrombotic risk factors cannot be entirely excluded without a prospective study

Türkiye´de hematologların transfüzyon tıbbı uzmanlık eğitim hedeflerine ulaşımının öz yeterlilik anketi ile değerlendirilmesi

Background: Proper clinical use of blood andblood products requires competent theoreticaland practical knowledge of transfusion medicine.The Curriculum Development and StandardDetermination System Medical SpecializationBoard is prepared Hematology SpecialistEducation Core Curriculum in Turkey. In thisstudy, we aimed to determine the access ofhematologists to the learning objectives definedby curriculum for the transfusion medicine andthe factors affecting it.Methods: Hematologists who have beenmembers of Turkish Hematology Society since2013 have been included in the study, Thesurvey questions were prepared based on thecurriculum for transfusion medicine. The studywas applied to hematologists with “surveymonkey” application. The questionnaireconsisted of a competence self-assessment withLikert scale and theoretical multiple-choiceknowledge questions.Results: Of the 213 hematologists, 54 (25%)were included in the study. Hematologists ratedtheir competences in the clinical competenceareas as 3,65 ± 0,73 (median 3,60) as “I knowbut not t a sufficient level”. The participants‘perception of competence was “I know, butnot at a sufficient level’” with an average of3.31 ± 0.84 (median3.5) in the blood bankingfield, while the average in hemapheresis andtransfusion medicine was 4.04 ± 0.63 (median4) as “enough”. In interventional procedures,hematologists stated that their vocationalcompetences were 2,79± 0,92 (median 2,93)on average as “I have an idea- I know, but notenough”. The correct answer to 13 theoreticalquestions was an average of 6,96 ± 1,89(median 7). Hematologists performing bloodrotation felt significantly more competent thanthe physicians who could not do the rotation inthe blood bank, blood banking t(52) = -3.9, p <.001 , transfusion medicine and interventionalcompetence t(52) = -2.2, p = .04 . Physicianswho believed that they are sufficient in theblood banking area, were more confident intransfusion medicine r(54) = .67, p <.001 andmanaging interventional procedures r(54) =.85, p <.001.Conclusion: In this study, hematologistsgenerally felt more competent in subjects suchas transfusion and therapeutic apheresis,which they often think of as not having enoughknowledge in the area of blood banking.Hematologists have been more confident inthe field of transfusion medicine as their yearsof expertise increased, but they did not feelbetter equipped in the fields of blood bankingand interventional competence. The currentresults suggested that hematologists who areexpected to be the blood bank supervisors do notinternalize the area of blood banking, are notstrong in their competence, and do not want towork in this area unless they are required.In hematology education curriculum, positiverevisions in education can be achieved byrevising blood banking curriculum and learningobjectives, standardizing blood center rotationswith content and duration, and support fromonline distance education programs.Giriş: Kan ve kan ürünlerinin uygun klinik kullanımı, transfüzyon tıbbı konusunda teorik ve pratik bilgi birikimini gerektirir. Türkiye’de Müfredat Geliştirme ve Standart Belirleme Sistemi Tıbbi Uzmanlık Kurulu tarafından, Hematoloji Uzmanlık Eğitimi Temel Müfredatı hazırlanmıştır. Bu çalışmada hematologların transfüzyon tıbbı müfredatı ile belirlenen öğrenme hedeflerine ulaşımını ve bu durumu etkileyen faktörleri belirlemeyi amaçladık. Metot: 2013 yılından bu yana Türk Hematoloji Derneği üyesi olan hematologlara transfüzyon tıbbı için müfredatı esas alınarak hazırlanan anket “Survey Monkey” uygulaması ile uygulandı. Anket, Likert ölçeği ve teorik çoktan seçmeli bilgi soruları ile öz yeterlilik değerlendirmelerinden oluşuyordu. Sonuçlar: 213 hematologdan 54’ü (%25) çalışmaya katılmıştır. Hematologların yeterlilik algıları klinik yetkinlik alanlarında ortalama 3,65 ± 0,73 (ortanca 3,60) olarak “Biliyorum ama yeterli düzeyde değil”; kan bankacılığı alanında ortalama 3.31 ± 0.84 (ortanca 3.5) puan ile “biliyorum ama yeterli düzeyde değil”; hemaferez ve transfüzyon tıbbı alanında ise ortalama 4.04 ± 0.63 (ortanca 4) “yeterli” olarak ölçüldü. Girişimsel işlemlerde hematologlar mesleki yeterliliklerinin ortalama 2,79± 0,92 (ortanca 2,93) “Bir fikrim var, biliyorum ama yeterli değil” olarak ifade ettiler. 13 teorik sorunun doğru cevabı ortalama 6,96 ± 1,89 idi (ortanca 7). Kan bankası rotasyonu yapan hematologlar yapamayanlara göre kan bankacılığı t(52) = -3.9, p < .001, transfüzyon tıbbı ve girişimsel alanlarda t(52) = -2.2, p = .04 kendilerini çok daha yetkin hissediyordu. Kan bankacılığı alanında yeterli olduğuna inanan hekimler, transfüzyon tıbbında r(54) = .67, p <.001 ve girişimsel işlemlerin r(54) = .85, p <.001yönetiminde de kendilerinden daha eminlerdi. Tartışma: Bu çalışmada, hematologlar genellikle kan bankacılığı alanında yeterli bilgiye sahip olmadığını düşünürken transfüzyon tıbbı ve terapötik aferez gibi konularda kendilerini daha yetkin hissetmistirler. Hematologlar, uzmanlık yılları arttıkça transfüzyon tıbbı alanında kendilerine daha fazla güvenmeye başlarken, kan bankacılığı ve girişimsel yeterlilik alanlarında kendilerini hala yeterli donanımda hissetmiyorlardı. Mevcut sonuçlar, hematologların kan bankacılığı alanını içselleştirmediklerini, yetkinliklerinde güçlü olmadıklarını ve gerekli olmadıkça bu alanda çalışmak istemediklerini göstermiştir. Transfüzyon tıbbı müfredatının ve öğrenme hedeflerinin gözden geçirilmesi, kan merkezi rotasyonlarının içerik ve süresinin standartlaştırılması ve çevrimiçi uzaktan eğitim programları ile desteklenmesi hematoloji eğitimine olumlu katkılar sağlayabilir