ALL Flashcards
The ETV6/RUNX-1 t(12;21) is found in what patient population more so and what kind of prognosis do we see with this?
It is more so found in pediatric patients and it is associated with a favorable prognosis. Only 2% of adult patients have this.
In general how do we treat Ph like B-ALL with ABL translocations?
You treat them with a BCR ABL TKI plus chemo just like you would for typical Ph+ B-ALL. And Allo SCT in CR1.
What is the newest regimen that is approved for Ph+ B-ALL?
Blinatumomab +/- TKI (Ponatinib preferred due to T315I mutation)
What is the mechanism of action of Blinatumomab?
It is a bi-specific antibody that binds to CD19 receptors and CD3 receptors on T cells leading to T-cell activation and lysis of B-cells
Besides BITE therapy as an option for frontline B-ALL what are the other options in those with Ph+ disease?
You can give TKI (Preferably Ponatinib) + multiagent chemotherapy such as Hyper-CVAD
One thing to keep in mind for B-ALL is that there has been a change to the guidelines for front line treatment in patients with Ph+ ALL, what was the old recommendation?
Hyper-CVAD plus dasatinib followed by allo transplant if they achieve CR. This could be the option they want you to pick on the board exam.
When looking at transcripts 210 and 190 which belongs to what disease
p210-CML
p190-B-ALL
What is the mechanism of action of Inotuzumab?
It is a antibody drug conjugate. It binds to CD22 and releases calicheamicin which induces DNA breaks.
What are the side effects you can see with Asparginase toxicity?
Allergic rxn (anaphylaxis), pancreatitis, thrombosis, intracranial hemorrhage, hyperglycemia, hypertriglycerdemia, hepatotoxicity.
What are the second line options for relapsed Ph+ B-ALL? For those with Ph- disease?
TKI alone, TKI+ Blinatumomab, TKI+Steroids, TKI+ chemo (you use regimens that are used in front line setting), TKI+Inotuzumab, CAR-T (Brexi-cel and Tisa cel) all followed by HCT. Ph neg-the options above, but without TKIs (refer to other flashcard regarding when to use CAR-T).
What is the immunophenotype for T-ALL and what is the treatment
Usually positive for CD7, CD3 (surface or cytoplasmic), CD4/8, CD1a, CD2, CD5, CD38
CD1a, sCD3-, My+, due to the poor prognosis these patients require allo SCT.
What is the immunophenotype of T-ALL? What is the tx?
They are CD1-CD8 positive. Tx includes CTX, HD ara-C, asparaginase (now nelarabine included). Maintenance POMP x2-3 years. Mediastinal XRT for Bulky Dx
Which patients absolutely require Allo SCT in B-ALL?
ALL-MLL t(11q23), Precursor T-ALL, Complex cytogenetics with 5 or more abnormalities.
What is the indication of CAR-T in relapsed B-ALL Ph+ and Neg disease?
Tisa-cel can only be given to patients younger than 26 years of age or if a patient has 2 or more relapses that included 2 TKIs
What maintenance therapy can you use in B-ALL Ph+ disease?
Monthly vincristine/prednisone pluses (for 2-3 years). May include weekly methotrexate+daily 6 mercaptopurine. NCCN says you can add a TKI to a regimen.
