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Journals / Turkish Journal of Hematology / 2014 / Cilt: 31 - Sayı: 2

Wilms Tumor 1 Gene Mutations in Patients with Cytogenetically Normal Acute Myeloid Leukemia

Normal Sitogenetiğe Sahip Akut Miyeloid Lösemi Hastalarında Wilms Tümör 1 Geni Mutasyonları

Salah AREF
Solafa El SHARAWY
Mohamed SABRY
Emad AZMY
Dalia Abdel RAOUF
Nadia El MENSHAWY
Pages
143–148
DOI
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AI Summary & Key Takeaways

AI Insight
⚡ TL;DR(Too Long; Didn't Read)

Patients with WT1 mutations had overall survival lower than patients with the wild type (HR=1.38; 95% CI 4.79-6.86; p=0.004).

📌 3 Key Takeaways

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Objective & Hypothesis:Objective: This study aimed to assess the prognostic impact of Wilms tumor 1 (WT1) mutations in cytogenetically normal acute myeloid leukemia (CN-AML) among Egyptian patients.Materials and Methods: Exons 1, 2, 3, 7, 8, and 9 of WT1 were screened for mutations in samples from 82 CNAML patients out of 203 newly diagnosed AML patients, of age ranging from 21 to 74 years, using high-resolution capillary electrophoresis.
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Key Findings & Data:Results:Eleven patients out of 82 (13.41%) harbored WT1 mutations.
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Conclusion & Impact:Patients with WT1 mutations had overall survival lower than patients with the wild type (HR=1.38; 95% CI 4.79-6.86; p=0.004).
🔬Design:Research Article
📍Scope:Comprehensive Sample
⚙️Variables:Domain Parameters

Abstract

Objective: This study aimed to assess the prognostic impact of Wilms tumor 1 (WT1) mutations in cytogenetically normal acute myeloid leukemia (CN-AML) among Egyptian patients.Materials and Methods: Exons 1, 2, 3, 7, 8, and 9 of WT1 were screened for mutations in samples from 82 CNAML patients out of 203 newly diagnosed AML patients, of age ranging from 21 to 74 years, using high-resolution capillary electrophoresis. Results:Eleven patients out of 82 (13.41%) harbored WT1 mutations. Mutations were detected in exon 7 (n=7), exon 9 (n=2), exon 8 (n=1), and exon 3 (n=1), but not in exons 1 or 2. There was no statistically significant difference between the WT1 mutants and wild types as regards age, sex, French-American-British subtypes, and the prevalence of success of induction remission therapy (p=0.966; 28.6% vs. 29.3%). Patients with WT1 mutations had overall survival lower than patients with the wild type (HR=1.38; 95% CI 4.79-6.86; p=0.004). Conclusion:CN-AML patients with WT1 mutations have poor clinical outcome. We recommend molecular testing for WT1 mutations in patients with CN-AML at diagnosis in order to improve risk stratification of those patients.

Özet

Amaç:Bu çalışma sitogenetiği normal akut miyeloid lösemi (SN-AML) tanısı olan Mısırlı hastalarda Wilms tümör 1 (WT1) mutasyonlarının prognoz üzerine etkilerini incelemeyi amaçlamaktadır. Gereç ve Yöntemler:İki yüz üç yeni tanılı AML hastası arasından yaşları 21 ile 74 arasında değişen 82 SN-AML hastasına ait örneklerde exon 1, 2, 3, 7, 8 ve 9 WT1'e ait mutasyonlar açısından yüksek çözünürlüklü kapiller elektroforez kullanılarak tarandı. Bulgular: Seksen iki hastanın 11'i (%13,41) WT1 mutasyonlarını taşımaktaydı. Mutasyonlar exon 7 (n=7), exon 9 (n=2), exon 8 (n=1) ve exon 3'te (n=1) tespit edildi, ancak exon 1 veya 2'de tespit edilmedi. WT1 mutasyonunu taşıyanlar ile taşımayanlar arasında yaş, cinsiyet, French-American-British (FAB) alt tipleri ve indüksiyon tedavisindeki başarı açısından istatistiksel anlamlı farklılık saptanmadı (p=0.966; 2%8.6 vs. %29.3). WT1 mutasyonu olan hastalarda genel sağkalım mutasyonu olmayanlara göre daha düşük bulundu (HR=1.38; %95 CI 4.79-6.86; p=0.004). Sonuç: WT1 mutasyonu olan SN-AML hastalarında klinik gidiş kötüdür. SN-AML hastalarında tanı anında daha iyi bir risk sınıflaması yapmak için WT1 mutasyonlarının moleküler testlerle araştırılmasını önermekteyiz.

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Cite This Article

AREF, S., SHARAWY, S. E., SABRY, M., AZMY, E., RAOUF, D. A., MENSHAWY, N. E. (2014). Wilms Tumor 1 Gene Mutations in Patients with Cytogenetically Normal Acute Myeloid Leukemia. Turkish Journal of Hematology, Cilt: 31 - Sayı: 2, 143–148. https://ajindex.com/articles/412825

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