Management of Adult Primary Immune Thrombocytopenia: Delphi- Based Consensus RecommendationsAhmet Muzaffer Demir1, Elif Gülsüm Ümit1, Muhlis Cem Ar2, Mesut Ayer3, Meltem Aylı4, Volkan Karakuş5, Emin Kaya6, Fahir Özkalemkaş7, Nilgün Sayınalp8, Mehmet Sönmez9, Fahri Şahin10, Selami Koçak Toprak11, Tayfur Toptaş12, İrfan Yavaşoğlu13, Ümran Çalış141Trakya University Faculty of Medicine, Department of Internal Medicine, Division of Hematology, Edirne, Türkiye
2İstanbul University-Cerrahpaşa, Cerrahpaşa Faculty of Medicine, Department of Internal Medicine, Division of Hematology, İstanbul, Türkiye
3University of Health Sciences Türkiye, Başakşehir Çam and Sakura City Hospital, Clinic of Hematology, İstanbul, Türkiye
4University of Health Sciences, Gülhane Training and Research Hospital, Clinic of Internal Medicine, Division of Hematology, Ankara, Türkiye
5University of Health Sciences Türkiye, Antalya Training and Research Hospital, Clinic of Hematology, Antalya, Türkiye
6İnönü University Faculty of Medicine, Department of Internal Medicine, Division of Hematology, Malatya, Türkiye
7Bursa Uludağ University Faculty of Medicine, Department of Internal Medicine, Division of Hematology, Bursa, Türkiye
8Hacettepe University Faculty of Medicine, Department of Internal Medicine, Division of Hematology, Ankara, Türkiye
9Karadeniz Technical University Faculty of Medicine, Department of Internal Medicine, Division of Hematology, Trabzon, Türkiye
10Ege University Faculty of Medicine, Department of Internal Medicine, Division of Hematology, İzmir, Türkiye
11Ankara University Faculty of Medicine, Department of Internal Medicine, Division of Hematology, Ankara, Türkiye
12Marmara University Faculty of Medicine, Department of Internal Medicine, Division of Hematology, İstanbul, Türkiye
13Aydın Adnan Menderes University Faculty of Medicine, Department of Internal Medicine, Division of Hematology, Aydın, Türkiye
14Abdi İbrahim İlaç Sanayi ve Tic. A.Ş., Senior Medical Manager, İstanbul, Türkiye
Objective: Primary immune thrombocytopenia (pITP) is an acquired autoimmune disorder related to the increased destruction and/or impaired production of platelets. Its diagnosis and management are challenging and require expertise and the interpretation of international consensus reports and guidelines with national variations in availability. We aimed to assess the agreement of hematologists in Türkiye on certain aspects of both first-line and second-line management of patients with pITP.
Materials and Methods: Applying a modified Delphi method, the Turkish National ITP Working Group (14 steering committee members), founded under the auspices of the Turkish Society of Hematology, developed a 21-item questionnaire consisting of statements regarding the first-line and second-line treatment of pITP. A total of 107 adult hematologists working in either university or state hospitals voted for their agreement or disagreement with the statements in two consecutive rounds.
Results: The participants reached consensus on the use of corticosteroids as first-line treatment and with limited duration. Methylprednisolone was the corticosteroid of choice rather than dexamethasone. Use of intravenous immunoglobulin was not preferred for patients without bleeding. It was also agreed that thrombopoietin receptor antagonists (TPO-RAs) or rituximab should be recommended as second-line treatment and that splenectomy could be considered 12-24 months after diagnosis in patients with chronic pITP.
Conclusion: The optimization of the dose and duration of TPO-RAs in addition to corticosteroids is necessary to improve the management of patients with pITP.
Keywords: Adult primary immune thrombocytopenia, Management, Delphi method
Erişkin Primer İmmün Trombositopeni Yönetimi: Delphi-Temelli Uzlaşı ÖnerileriAhmet Muzaffer Demir1, Elif Gülsüm Ümit1, Muhlis Cem Ar2, Mesut Ayer3, Meltem Aylı4, Volkan Karakuş5, Emin Kaya6, Fahir Özkalemkaş7, Nilgün Sayınalp8, Mehmet Sönmez9, Fahri Şahin10, Selami Koçak Toprak11, Tayfur Toptaş12, İrfan Yavaşoğlu13, Ümran Çalış141Trakya University Faculty of Medicine, Department of Internal Medicine, Division of Hematology, Edirne, Türkiye
2İstanbul University-Cerrahpaşa, Cerrahpaşa Faculty of Medicine, Department of Internal Medicine, Division of Hematology, İstanbul, Türkiye
3University of Health Sciences Türkiye, Başakşehir Çam and Sakura City Hospital, Clinic of Hematology, İstanbul, Türkiye
4University of Health Sciences, Gülhane Training and Research Hospital, Clinic of Internal Medicine, Division of Hematology, Ankara, Türkiye
5University of Health Sciences Türkiye, Antalya Training and Research Hospital, Clinic of Hematology, Antalya, Türkiye
6İnönü University Faculty of Medicine, Department of Internal Medicine, Division of Hematology, Malatya, Türkiye
7Bursa Uludağ University Faculty of Medicine, Department of Internal Medicine, Division of Hematology, Bursa, Türkiye
8Hacettepe University Faculty of Medicine, Department of Internal Medicine, Division of Hematology, Ankara, Türkiye
9Karadeniz Technical University Faculty of Medicine, Department of Internal Medicine, Division of Hematology, Trabzon, Türkiye
10Ege University Faculty of Medicine, Department of Internal Medicine, Division of Hematology, İzmir, Türkiye
11Ankara University Faculty of Medicine, Department of Internal Medicine, Division of Hematology, Ankara, Türkiye
12Marmara University Faculty of Medicine, Department of Internal Medicine, Division of Hematology, İstanbul, Türkiye
13Aydın Adnan Menderes University Faculty of Medicine, Department of Internal Medicine, Division of Hematology, Aydın, Türkiye
14Abdi İbrahim İlaç Sanayi ve Tic. A.Ş., Senior Medical Manager, İstanbul, Türkiye
Amaç: Primer immün trombositopeni (pITP), trombositlerin artan yıkımı ve/veya bozulmuş üretimi ile ilişkili edinsel bir oto-immün hastalıktır. pITP’nin tanı ve yönetimi zorludur; ayrıca hem uzmanlık hem de uluslararası fikir birliği raporları ile kılavuzlarının, ulusal farklılıkları da göz önünde bulundurarak, yorumlanmasını gerektirir. Bu çalışmada Türkiye’deki hematologların pITP hastalarının birinci ve ikinci basamak tedavisinin belirli alanlarındaki fikir birliği düzeylerini değerlendirmeyi amaçladık.
Gereç ve Yöntem: Türk Hematoloji Derneği bünyesinde kurulan Türkiye Ulusal ITP Çalışma Grubu (14 yürütme kurulu üyesi), modifiye Delphi yöntemi ile pITP’nin birinci basamak ve sonraki tedavilerine ilişkin ifadelerden oluşan 21 maddelik bir anket geliştirdi. Üniversite veya devlet hastanelerinde çalışan 107 yetişkin hematolog, ardışık iki tur boyunca ifadelere katılıp katılmama yönünde oy kullandı.
Bulgular: Katılımcılar kortikosteroidlerin birinci basamak tedavi olarak ve sınırlı süreyle kullanılması konusunda fikir birliğine vardı. Metilprednizolonun, dekzametazondan ziyade tercih edilen kortikosteroid olduğu gözlendi. İntravenöz immünoglobulin tedavisinin kanama dışında kullanımı tercih edilmez iken, thrombopoietin reseptör antagonistleri (TPO-RA) veya rituksimab ikinci basamak tedavi olarak önerilebileceği, kronik pITP hastalarında tanıdan 12-24 ay sonra splenektomi düşünülebileceği konusunda da görüş birliğine varıldı.
Sonuç: pITP’li hastaların yönetimini iyileştirmek için kortikosteroidlerin yanı sıra TPO-RA’ların doz ve süresinin optimizasyonu gerekli olduğu konusunda fikir birliği sağlanmıştır.
Anahtar Kelimeler: Erişkin primer immün trombositopeni, Hastalık yönetimi, Delphi metodu
Ahmet Muzaffer Demir, Elif Gülsüm Ümit, Muhlis Cem Ar, Mesut Ayer, Meltem Aylı, Volkan Karakuş, Emin Kaya, Fahir Özkalemkaş, Nilgün Sayınalp, Mehmet Sönmez, Fahri Şahin, Selami Koçak Toprak, Tayfur Toptaş, İrfan Yavaşoğlu, Ümran Çalış. Management of Adult Primary Immune Thrombocytopenia: Delphi- Based Consensus Recommendations. Turk J Hematol. 2024; 41(2): 97-104
Corresponding Author: Ahmet Muzaffer Demir |
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