WHAT A HİGH FREQUENCY OF DEFERİPRONE-İNDUCED AGRANULOCYTOSİS İN A TURKİSH POPULATİON OF THALASSEMİA MAJOR

Öz

Background: Deferiprone may cause agranulocytosis.
Methods: All cases of thalassemia major followed by Center of Hereditary Blood Disorders
of the Iskenderun Public Hospital and Hematology Department of the Mustafa Kemal
University were retrospectively reviewed.
Results: Eighteen cases were on desferrioxamine therapy, five on deferiprone, and 21 on both
initially. Three of 26 deferiprone receiving cases developed agranulocytosis in the first year of
therapy, so its frequency was 11.5%. When we compared the ratio with previous reports (0.5-
1%), difference was significant (p<0.01). Milder neutropenia was observed in none of
deferiprone receiving cases, which was significantly lower (0.0% vs 8.5%, p<0.01). Three
agranulocytosis cases were both on desferrioxamine and deferiprone therapies, and only one
case was female. Additionally, two of them were splenectomized, and none was antiHCV
positive.
Conclusion: Hereditary factors and combination with desferrioxamine are probably the
most important factors for higher frequency of deferiprone induced agranulocytosis rather
than female sex, splenectomy operations, or hepatitis infections.  

Anahtar Kelimeler

• Thalassemia major
• iron overload
• desferrioxamine
• deferiprone
• ICL 670

Bir Türk talasemi majör hasta grubunda deferiprona bağlı agranülositozun yüksek sıklığı

Öz

Deferipronun agranülositoz yapma potansiyeli mevcuttur. İskenderun Devlet Hastanesi Kalıtsal Kan Hastalıkları Merkezi ve Mustafa Kemal Üniversitesi Tıp Fakültesi Hematoloji Servisince takip edilen talasemi majör hastalarının kayıtları incelendi. Onsekiz vaka desferoksamin, beş vaka deferipron ve 21 vaka her ikisini de kullanmaktaydı. Tedavinin ilk yılında deferipron kullanan 26 hastadan üçünde (%11.5) agranülositoz gelişti. Bu oran, önceki yayınlarda belirtilen agranülositoz görülme oranı olan %0.5-1 ile karşılaştırıldığında, arada istatistiksel olarak anlamlı bir farkın mevcut olduğu görüldü (p<0.01). Hafif nötropeni hiçbir vakada görülmedi ve bu oran da anlamlı şekilde düşüktü (%0.0’a karşılık %8.5, p<0.01). Agranülositoz görülen vakaların üçü hem desferoksamin hem de deferipron kullanmaktaydı ve vakaların bir tanesi bayandı. Ayrıca, agranülositoz görülen vakaların ikisi splenektomiliydi ve hiçbir vakada antiHCV pozitifliği tespit edilmedi. Sonuç olarak, kalıtımsal faktörler ve desferoksamin ile birlikte kullanımın, deferiprona bağlı agranülositoz için önemli risk faktörleri olduğu ve bayan cinsiyet, geçirilmiş splenektomi veya hepatit enfeksiyonlarının getirdiği risklerin düşük olabileceği düşünüldü.

Anahtar Kelimeler

• Talasemi majör
• artmış demir yükü
• desferoksamin
• deferipron
• ICL

Kaynakça

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