Pediatrik Vakalarda Philadelphia Benzeri Akut Lenfoblastik Lösemi

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

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