Ayşen YILDIRIM TÜREDİ, Murat SÜRÜCÜ, Ferah GENEL, Yöntem YAMAN, Canan VERGİN, Yeşim OYMAK

Çocukluk çağı akut lenfoblastik lösemili olgularda görülen miyeloid koekspresyonlar ve TEL-AML1 mutasyonunun klinik önemi

Amaç: Bu çalışmada akut lenfoblastik lösemili hastalarda gözlenen koekspresyonlar ve TEL-AML1 mutasyonunun klinik özellikler ve prognoz ile ilişkisinin araştırılmasıamaçlandı.Yöntemler: Çocuk hematoloji kliniğimizde takip edilen 83 ALL hastasının dosyası retrospektif olarak incelendi. Yaş, cinsiyet, beyaz küre sayısı, hemoglobin düzeyi ve trom- bosit sayısı, ALL alt tipi (B veya T ALL), risk, flow sitometriile bakılan yüzey antijenleri, TEL-AML1 mutasyon varlığı, tedavinin 8., 15. ve 33. gün yanıt ve remisyon ve relapsdurum verileri kaydedildi ve analiz edildi.Bulgular: Hastaların 15’inde (%18) bir ya da daha fazla aberan antijen ekspresyonu saptandı. Oniki hastada(%14,4) miyeloid antijen koekspresyonu (CD13 ve/veyaCD33), B hücre ALL’li 2 hastada sırası ile CD2 ve CD7koekspresyonu, T hücre ALL’li bir hastada ise CD19 koekspresyonu gözlendi. Miyeloid koekspresyon olan veolmayan hastalar arasında, hemoglobin, beyaz küre ve trombosit sayıları, sekizinci, onbeşinci ve otuzüçüncü gün tedaviye verdikleri yanıt, risk grupları (düşük, orta ve yük-sek risk) ve relaps gelişimi açısından anlamlı bir farklı- lık bulunmadı (p>0.05). TEL-AML1 mutasyonu saptananonüç hastanın dördünde miyeloid antijen koekspresyonu saptandı. Bu mutasyon ile koekspresyonlar arasında an-lamlı ilişki gösterilemedi. Miyeloid antijen koekspresyonu ve TEL-AML1 mutasyon birlikteliği gözlenen dört hastada relaps yada eksitus gözlenmedi.Sonuç: ALL’li çocuklarda miyeloid ilişkili antijen ekspresyonun varlığı ve/veya TEL-AML mutasyon varlığı prognozla ilişkili bulunmadı. Daha iyi sonuçların alınabilmesi için yüksek sayıda hasta grupları ile yapılacak çalışmalara gereksinim olduğu kanısına varıldı.

The clinical importance of myeloid antigen coexpression and TEL-AML1 mutation in patients with childhood acute lymphoblastic leukemia

Objective: In this study, we aim to investigate the relationship, if any, between clinical features, prognosis, and the coexpressions and TEL-AML1 mutation in patients with acute lymphoblastic leukemia (ALL). Methods: Eigthy-three patients with acute lymphoblastic leukemia were retrospectively examined. Age, gender, White blood cell count, hemoglobin level, platelet count, ALL subtypei (B or T ALL), risk groups, surface antigens deteceted by flow cytometry, existence of TEL-AML1 mu- tations, response, remission and relapse status at 8., 15. ve 33. Days of treatment were recorded and analyzed. Results: 15 (18%) out of 83 were identified with aberrant antigen expression. Of these patients, twelve (14.4%) had myeloid antigen coexpression (CD13 and/or CD33), two with B cell ALL had CD2 and CD7 coexpressions respectively, one with T cell ALL had CD19 coexpression. No significant differences were found between patients with and without myeloid antigen coexpression in terms of hemoglobin levels, white blood cells and platelet counts, responses given on the 8th, 15th, and 30th days on the treatment, risk groups, and relapse (p>0.05). Myeloid antigen coexpression was found in 4 of 13 patients who were iden- tified with TEL-AML1 mutation. No significant relationship was found between this mutation and coexpressions. No relapse and exitus were observed in four patients with co- expression and TEL-AML1. Conclusion: The prognosis and clinical features shows no statistically significant relationship with the presence of neither Myeloid antigen expression nor TEL-AML1 muta- tion. We believe, however, the future studies involving bigger sample sizes will prove to be useful in terms of more convincing results.

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