Bifenotipik Akut Lösemili Hastalarda Uygulanan İndüksiyon Tedavilerinin Etkinliği

Lösemi tanı ve tedavisindeki ilerlemelere rağmen BAL yönetimi zorlu kalmaya devam etmektedir. Bu çalışmanın amacı BAL tanısı alan 13 hastanın klinokopatolojik özelliklerini ve tedavi sonuçlarını analiz edip literatüre katkıda bulunmaktır. Merkezimizde 2017-2022 yılları arasında EGIL veya WHO 2016 kriterlerine göre BAL tanısı alan 13 hastanın tıbbi kayıtları geriye dönük olarak incelendi. Ortalama yaşları 36 olan 13 BAL hastasının 6’sı (%46) kadın 7’si (%54) erkekti. Olguların 11’i (%84,6) myeloid/B, 2’si (%15,6) myeloid/T hücre ekspresyon paterni sergiliyordu. Sitogenetik incelemede olguların 3’ünde (%23,1) t(v;11q23) MLL, 1’inde (%7,7) t(9;22) BCR-ABL1 ve 1’inde (%7,7) FLT-ITD anomalisi mevcuttu. Olguların 7’si (%53) ALL ve 6’sı (%47) AML indüksiyon tedavisi aldı ve 8’inde (%61,5) tedaviye yanıt vardı. Tedaviye yanıt oranının en fazla olduğu protokoller sırasıyla ALLOLD07 (%100), S-HAM (%75) ve HYPER-CVAD (%66) idi. Tanı anından itibaren medyan takip süresi 32 ay (aralık: 2-71), medyan OS 9 ay (%95 CI: 2,71-15,94) ve medyan PFS ise 4.5 ay (%95 CI: 3,32-5,67) idi. Olguların 8’ine allojenik hematopoietik kök hücre transplantasyonu (AKİT) yapıldı. AKİT sonrası 1 hastada relaps hastalık gelişti ve hastalık progresyonundan öldü. Olguların 4’ü BAL progresyonu, 2’si enfeksiyon sebebiyle öldü ve genel sağkalım oranı %53.8 idi. BAL nadir görülen bir hastalık olduğundan optimal tedavinin ne olduğu konusunda ortak bir görüş yoktur. BAL tedavisinde ALL tabanlı indüksiyon rejimleri daha üstün gibi görünmekle beraber, bu nadir hastalığın heterojen doğasını anlamak için daha fazla olguyla yapılacak çok merkezli klinik çalışmalara ihtiyaç vardır.

Anahtar Kelimeler:

Bifenotipik lösemi, Sağkalım, Prognoz

Efficacy of Induction Treatments in Patients with Biphenotypic Acute Leukemia

Despite advances in leukemia diagnosis and treatment, BAL management remains challenging. The aim of this study is to contribute to the literature by analyzing the clino-pathological characteristics and treatment outcome of 13 patients diagnosed as BAL. Medical charts of 13 patients who were diagnosed as BAL according to EGIL or WHO 2016 criteria in our center between 2017- 2022 were retrospectively reviewed. Of the 13 BAL patients with a mean age of 36, 6 (46%) were female and 7 (54%) were male. Eleven (84.6%) of the cases showed B/myeloid, 2 (15.6%) T/myeloid cell expression patterns. In cytogenetic examination, 3 (23.1%) of the cases had t(v; 11q23) MLL, 1 (7.7%) t(9;22) BCR-ABL1 and 1 (7.7%) FLT- ITD abnormality. Eight of the cases (61.5%) received ALL induction therapy and 5 (38.5%) of them received AML induction therapy, and 8 (61.5%) of them responded to the treatment. The treatment protocols with the highest rate of response to treatment were ALLOLD07 (100%), S-HAM (75%) and HYPER-CVAD (66%), respectively. Median follow-up from the time of diagnosis was 32 months (range: 2-71), median OS 9 months (95%CI: 2.71-15.94) and the median PFS 4.5 months (95%CI: 3.32-5.67). Allo-Hematopoietic stem cell transplantation (HSCT) was performed in 8 of the cases. After HSCT, 1 patient developed relapsed disease and died from disease progression. Four of the cases died due to BAL progression, 2 died due to infection, and the overall survival rate was 53.8%. Since BAL is a rare disease, there is no consensus on which treatment is optimal. Although ALL-based induction regimens seem to be superior in the treatment of BAL, multicenter clinical studies with more cases are needed to better understand the heterogeneous nature of this rare disease.

Keywords:

Biphenotypic leukemia, Survival, Prognosis,

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