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Identifier

000441336

https://elocus.lib.uoc.gr//dlib/9/c/c/metadata-dlib-1617880856-18020-10836.tkl

Title

Οξεία Λεμφοβλαστική Λευχαιμία σε Εφήβους και Νεαρούς Ενήλικες. : Θεραπεία και Πρόγνωση σε Παιδοαιματολογική κλινική και Διεθνής Εμπειρία

Alternative Title

Αcute Lymphoblastic Leukaemia in Adoloscents and Young Adults

Author

Μανίκα, Ιωάννα

Thesis advisor

Στειακάκη, Ευτυχία

Reviewer

Παπαδάκη, Ελένη
Ποντίκογλου, Χαράλαμπος

Abstract

Acute Lymphoblastic leukemia (ALL), the most prevalent hematologic malignancy in children, also effects Adolescents and Young Adults (AYA) , variably defined in different studies by an age between 15-18 and 25-39 years. The outstanding therapeutic progress achieved with modern pediatric regimens in childhood ALL led efforts to explore whether a similar treatment approach could be equally effective and safe in AYA , as this population had a survival disadvantage compared with both younger and older patient. Several comparative and non comparative trials in AYA with pediatric-based asparaginase- approaches have been carried out during the last two decades, showed better outcomes compared with traditional adult treatment in B-lineage and T-lineage Philadelphia chromosome negative ALL, while the use of tyrosine kinase inhibitors (TKI) led to comparative progress in Ph+ALL, a former high risk subset more typically observed in older ages. At present, pediatric-based regimens warrant 5-year survival rates of 60-70%. On the other hand, issues of treatment compliance and drug-related toxicity appeared in this group of patients. Advanced diagnostics and minimal residual disease measurements aid in prognosis and have resulted in shifting recommendations regarding allogeneic hematopoietic cell transplantation in first remission. Understanding the genetic basis of ALL leaded to a better risk stratification and a targeted therapy. One of the most remarkable alterations was this of the lymphoid transcription factor gene IKFZ1(IKAROS) which is associated with a high risk of treatment failure in B-ALL and unfortunately is prevalent to the AYA’s group. Recently approved agents like Blinatumomab, Inotuzumab and chimeric antigen receptor T-cell therapy are transforming the treatment of refractory/relapsed B-ALL. It is obvious that we are approaching a new era. An era of precision medicine with immunotherapy and other molecularly targeted treatments that offer unique opportunities to customize treatment intensity with or without hematopoietic stem cell transplantation, reduce the burden of toxicities and combat persistent residual disease. This study summarizes recent scientific developments related to prognosis (ALL biology, MRD) and treatment (frontline management, novel agents, supportive care) specific to this patient population. Finally, exhibits the outcomes of the management of ALL in AYA patients from a Pediatric Hematology Department during the last two decades.

Language

Greek

Subject

Immunotherapy

Pediatric-based regimens

Therapy related toxicity

Ανοσοθεραπεία

Ελάχιστη υπολειπόμενη νόσος

Εφηβοι και νεαροί ενήλικες

Παιδιατρικώς εμπνευσμένα πρωτόκολλα

Τοξικότητα σχετιζόμενη με τη θεραπεία

Issue date