Pediatrik Vakalarda Philadelphia Benzeri Akut Lenfoblastik Lösemi

Yıl 2021, Cilt: 19 Sayı: 1, 141 - 150, 09.04.2021

Ecem Efendi Erdem Gülşah Cecener Havva Tezcan Ünlü Melike Sezgin Evim

Öz

Lösemi, hematopoietik hücrelerin malign transformasyonu sonucu gelişen, heterojen neoplastik hastalıklar grubudur. Philadelphia (Ph) benzeri akut lenfoblastik lösemi (ALL), yüksek risk sınıflamasında yer alan ve kötü prognoz gösteren B- hücreli akut lenfoblastik löseminin (B-ALL) alt grubudur. Gen ekspresyon profili olarak Ph pozitif ALL’ye benzemektedir, ancak bu alt tipte BCRABL1 füzyonu mevcut değildir. Hastalık genetik olarak heterojendir. Ayrıca, etnik kökenleri farklı olan Ph benzeri ALL vakalarının genetik değişimleri de farklılık göstermektedir. Ph benzeri ALL vakalarının B-ALL’li hastalar arasından ayırt edilerek sınıflandırılabilmesi etkin tedavi almaları için önemlidir. Son yıllarda, Ph benzeri ALL alt grubuna ait vakaların tanımlanması için, B-ALL vakalarında genetik ve klinik bulgular değerlendirilmesinin yanı sıra, gen ifade farklılıklarının analiz edildiği panellerin tasarlanması gündemdedir.

Anahtar Kelimeler

akut lenfoblastik lösemi, Philadelphia-benzeri,, pediatrik

Philadelphia-Like Acute Lymphoblastic Leukemia in Pediatric Cases

Öz

Leukemia is a heterogeneous group of neoplastic diseases that develop as a result of the malignant transformation of hematopoietic cells. Philadelphia (Ph)-like
acute lymphoblastic leukemia (ALL) is a subgroup of B-cell acute lymphoblastic leukemia (B-ALL), which is included in the high-risk classification and has a poor
prognosis. It is similar to Ph-positive ALL in gene expression profile, but this subtype does not have BCR-ABL1 fusion. The disease is genetically heterogeneous.
In addition, the genetic changes of Ph-like ALL cases with different ethnic origins also differ. It is important to distinguish and classify Ph-like ALL cases from patients with B-ALL for effective treatment. In recent years, it is on the agenda to design panels in which gene expression differences are analyzed in addition to evaluating genetic and clinical findings in B-ALL cases to identify cases belonging to the Ph-like ALL subgroup.

Kaynakça

• 1. Ching-Hon Pui, Jun J. Yang, Stephen P. Hunger et. al.(2015), Childhood Acute Lymphoblastic Leukemia: Progress Through Collaboration, August/24, Journal Of Clınıcal Oncology.
• 2. Arceci RJ, Hann IM, Smith OP. Pediatric Hematology 3th ed Malden, Massechusetts Blackwell, 2006 pp 450-81.
• 3. Pui CH. Acute lymphoblastic leukemia in children. Curr. Opin. Oncol. 2000;12: 3-12.
• 4. Chessels JM, Bailey C, Richards SM. Intensification of teratment and survival in all children with lymphoblastic leukemia: results of UK Medical Research Council trial UKALL X. Medical Research Council Working Party onChildhood Leukemia. Lancet 1995; 345: 143-8.
• 5. Schorin MA, Blattner S, Gelber RD, Tarbell NJ, Donnelly M, Dalton V, Cohen HJ, Sallan SE. Treatment of childhood acute lymphoblastic leukemia: results of Dana-Farber Cancer Institute/ Children’s Hospital Acute Lymphoblastic Leukemia Consortium Protocol 85-01. J. Clin. Oncol. 1994; 12: 740-7.
• 6. Cosma G, Crofts F, Taioli E, Toniolo P, Garte S. Relationship between genotype and function of the human CYP1A1 gene. J. Toxicol. Environ. Health 1993; 40: 309-16. 7. Wells J, Jain N, Konopleva M (2017) Philadelphia Chromosome- Like Acute Lymphoblastic Leukemia: Progress in a New Cancer Subtype. Clin Adv Hematol Oncol 15(7):554-61.
• 8. Ching-Hon Pui, Kathryn G. Roberts, Jun J. Yang, Charles G. Mullighan; Philadelphia Chromosomeelike Acute Lymphoblastic Leukemia,Clinical Lymphoma, Myeloma & Leukemia, Vol. 17, No. 8, 464-70. 2017 Elsevier Inc. All rights reserved.
• 9. Pui CH, Roberts KG, Yang JJ et al (2017) Philadelphia Chromosome-like Acute Lymphoblastic Leukemia. Clin Lymphoma Myeloma Leukemia 17(8):464-70.
• 10. Pui CH, Yang JJ, Hunger SP et al (2015) Childhood Acute Lymphoblastic Leukemia: Progress Through Collaboration. J Clin Oncol 2015 Sep 20;33(27):2938-48.
• 11. Tran TH, Loh ML (2016) Ph-like acute lymphoblastic leukemia. Hematology Am Soc Hematol Educ Program (1):561-6.
• 12. Boer JM, Den Boer ML (2017) BCR-ABL1-like acute lymphoblastic leukaemia: From bench to bedside. Eur J Cancer 82:203-18.
• 13. Woo JS, Alberti MO, Tirado CA (2014) Childhood B-acute lymphoblastic leukemia: a genetic update. Exp Hematol Oncol 13;3:16.
• 14. Roberts KG, Payne-Turner Y. Li, D., Harvey R.C., et al (2015) Targetable Kinase-Activating Lesions in Ph-like Acute Lymphoblastic Leukemia. N Engl J Med 11; 371(11):1005-15.
• 15. Eric S. Schafer, Judith Margolin, David G. Poplack, and Karen R. Rabin. (2015) Molecular Genetics of Acute Lymphoblastic Leukemia. The molecular basis of cancer. Philadelphia, PA: Elsevier/Saunders, Print book: English: Edition 4.
• 16. Frisch A, Ofran Y. (2019). How I diagnose and manage Philadelphia chromosome-like acute lymphoblastic leukemia, Vol. 104 No. 11 (2019): November, 2019 https://doi.org/10.3324/ haematol.2018.207506.
• 17. Nordgren A, Schoumans J, Söderhall S, et.al. (2001) Interphase fluorescence in situ hybridization and spectral karyotyping reveals hidden genetic aberrations in children with acute lymphoblastic leukaemia and a normal banded karyotype. Br J Haematol. 2001 Sep;114(4):786-93.
• 18. Siegele BJ, Nardi V (2018) Laboratory testing in BCR-ABL1- like (Philadelphia-like) B-lymphoblastic leukemia/lymphoma. Am J Hematol 93(7):971-7.
• 19. Dalva K. Hematoloji’de Akım Sitometri Kullanımı. Moleküler Hematoloji ve Sitogenetik Alt Komitesi, Temel Moleküler Hematoloji Kursu. Ankara Üniversitesi Tıp Fakültesi,Hematoloji.
• 20. Revers-Transkriptaz Polimeraz Zincir Reaksiyonu (RT-PCR) ve Uygulama Alanları Araş.Gör.Burcu Okutucu* Yrd. Doç.Dr. Sacide Pehlivan.
• 21. Sayitoğlu-Aydın M. Hematoloji’de Real Time Pcr. Moleküler Hematoloji Ve Sitogenetik Alt Komitesi, Temel Moleküler Hematoloji Kursu. İstanbul Üniversitesi, Deneysel Tıp Araştırma Enstitüsü, Genetik Anabilim Dalı, İstanbul.
• 22. Cemaliye Boylu Akyerli. Hematoloji’de Mikroarray Kullanımı. Temel Moleküler Hematoloji Kursu, 12-13 Mart 2005. Ankara
• 23. Harvey R.C., Tasian S.K (2020) Clinical diagnostics and treatment strategies for Philadelphia chromosome-like acute lymphoblastic leukemia Affiliations. Blood Adv. 2020 Jan 14;4(1):218-28. doi: 10.1182/bloodadvances.2019000163.
• 24. Prescott K., Jacobs M., Stock W., Wynne J. (2020) New Approaches to Treating Challenging Subtypes of ALL in AYA Patients Current. Hematologic Malignancy Reports https://doi. org/10.1007/s11899-020-00597-y. 2020 Sep 12.
• 25. Cazzaniga G, d’Aniello E, Corral L et al. (2003) Results of minimal residual disease (MRD) evaluation and MRD based treatment stratification in childhood ALL. Best Pract Res Clin Haematol 2003; 15:623-38.
• 26. Flohr T, Schrauder A, Cazzaniga G et al. (2008) Minimal residual disease-directed risk stratification using realtime quantitative PCR analysis of immunoglobulin and T-cell receptor gene rearrangements in the international multicenter trial AIEOPBFM ALL 2000 for childhood acute lymphoblastic leukemia. Leukemia 2008; 31.
• 27. Tasian SK, Loh LM, Hunger SP (2017) Philadelphia chromosomelike acute lymphoblastic leukemia. Blood 130:2064-72.
• 28. Roberts KG, Li Y, Payne-Turner D et al (2014) Targetable kinaseactivating lesions in Ph-like acute lymphoblastic leukemia. N Engl J Med 371(11):1005-15.