Duygu KÖSE, Emine ZENGİN, Sema AYLAN GELEN, Nazan SARPER

Çocukluk Çağı Akut Lenfoblastik Lösemilerinde, ALL-BFM Protokolleri ile İnduksiyon Ölümleri ve Tedavi-İlişkili Ölümler

GİRİŞ ve AMAÇ: Çocukluk çağı akut lenfoblastiklösemilerinde (ALL), Berlin-Frankfurt-Münster (BFM) çalışmagrubu çok iyi sonuçlar elde edilmiştir. Burada, BFM-ALLçalışma grubuna göre daha kötü sonuçlar alınmasınınnedenlerinin açıklanması için lösemilerin biyolojik özelliklerive BFM tabanlı protokollerle tedavi edilmiş çocuk veergenlerin tedavi sonuçları değerlendirilmiştir. YÖNTEM ve GEREÇLER: Ocak 2001-Aralık 2014döneminde tanı konmuş hastaların bilgileri hastanekayıtlarından alındı. BFM- Türkiye ALL 2000 (TRALL-2000)(BFM-ALL 95 ten türetilmiş), BFM-ALL 95 ve BFM-ALL Intercontinental-2009 tedavi protokolleri art arda kullanıldı.Down sendromlu hastalar dışındaki 1-18 yaştaki tüm hastalarçalışmaya alındı. BULGULAR: Ortalama yaşı 6,64 ± 4,35 yıl olan 207 hastavardı. Hastaların standart, orta ve yüksek risk grubundakidağılımları sırasıyla %27,5, %54,9 ve %16,2 idi.İmmünofenotip sırasıyla hastaların %84,3, %13,7 ve %1,0 indeB-ALL, T-ALL ve bifenotipik lösemiydi. İndüksiyon ölümü 9hastada (%4,3) saptandı ve 33. günde remisyon oranı %94,2idi. On üç hasta (%6,5) ilk tam remisyonda kaybedildi.İndüksiyon+ tam remisyondaki ölümlerin 16 sı (%7,7)enfeksiyonla ilişkiliydi. Relaps oranı %19,3 tü. Ölüm oranı%20,1 idi. Yedi-yıllık olaysız sağkalım ve genel sağkalımsırasıyla %73,0 ve %79,4 idi. TARTIŞMA ve SONUÇ: BFM-ALL çalışma grubunun BFM ALL 95 çalışmasına kıyasla, bu çalışmada indüksiyon ölümlerive ilk remisyondaki ölümler daha yüksekti. Sağkalım oranlarıTürkiye'deki benzer merkezler gibiydi. Enfeksiyonlara bağlıölümlerin azaltılması için, hematoloji ünitelerinin şartlarınıniyileştirilmesi, daha iyi destek bakım, çalışanların verefakatçilerin eğitilmesi gerekmektedir.

Induction Deaths and Treatment-Related Mortality in ChildhoodAcute Lymphoblastic Leukemia with ALL-BFM Protocols

INTRODUCTION: Favorable results were achieved inchildhood acute lymphoplastic leukemia (ALL) by Berlin Frankfurt-Münster (BFM) study group trials. Here, biologicalfeatures of leukemia and treatment results of children andadolescents treated with BFM-based protocols were analysedto explain relatively inferior outcomes compared to BFM-ALLstudy group METHODS: Data of the patients diagnosed between January2001-December 2014 were collected from hospital records.BFM- Turkey ALL 2000 (TRALL-2000) (modified BFM-95),BFM-ALL 95 and BFM-ALL-Intercontinental -2009 treatmentprotocols were used consecutively. All patients 1-18 year-old,excluding patients with Down syndrome were included in thestudy. RESULTS: There were 207 patients with a mean age of 6.64 ±4.35 years. Distribution of the patients were 27.5%, 54.9% and16.2% in standard, medium and high risk groups, respectively.Immunophenotype was B-ALL, T-ALL and biphenotypicleukemia in 84.3%, 13.7% and 1% of the patients respectively.There were 9 (4.3%) induction deaths and remission rate was94.2% on day 33. Thirteen patients (6.5%) died in the firstcomplete remission. Sixteen of the deaths (7.7%) in inductionplus first complete remission were infection related. Relapserate was 19.3%. Mortality was 20.1%. Seven-year event –freesurvival and overall survival were 73% and %79.4respectively. DISCUSSION AND CONCLUSION: Compared tomulticenter trial BFM-95 of BFM-ALL study group, inductiondeaths and death in first CR were high. Survival rates weresimilar to other Centers of Turkey. Improvement in conditionsof the Hematology Units, better supportive care, training of thestaff and caregivers will be required to decrease infectionrelated mortality.

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