Appointment sheet
Philadelphia chromosome-like acute lymphoblastic leukaemia (Ph-like or BCR::ABL1-like ALL)
Prepared with OnCo (onco.cc/prep/all-ph-like/). Orientation, not medical advice; your team knows your case.
My details
Name
Date of appointment
Hospital and clinician
Who is coming with me
What I know, what is unclear, changes to discuss
Saved in this browserWhat I know so far
What is unclear to me
Changes since last time
My questions
16 on the sheetNewly diagnosed
- 1.What is my exact diagnosis, stage, and grade, and which tests established them?
- 2.Which biomarkers have been tested on my tumour (for example Ph-like gene expression signature, CRLF2 overexpression by flow cytometry and CRLF2 rearrangement by FISH, ABL-class, JAK2 and EPOR fusions by FISH or RNA sequencing, JAK1, JAK2, IL7R and SH2B3 mutations, IKZF1 deletion), and what were the results?
- 3.Which subtype is my cancer, and does that change the recommended treatment?
- 4.Is germline (inherited) genetic testing recommended for me or my family?
Diagnosis
- 5.For my situation (diagnosis), which of the standard options do you recommend and why?
ABL-class fusions
- 6.For my situation (abl-class fusions), which of the standard options do you recommend and why?
- 7.Am I a candidate for Dasatinib, Imatinib, and what side effects should I expect?
CRLF2, JAK2 or EPOR lesions
- 8.For my situation (crlf2, jak2 or epor lesions), which of the standard options do you recommend and why?
- 9.Am I a candidate for Ruxolitinib, and what side effects should I expect?
Persistent residual disease
- 10.For my situation (persistent residual disease), which of the standard options do you recommend and why?
- 11.Am I a candidate for Blinatumomab, Tisagenlecleucel, and what side effects should I expect?
Any stage
- 12.Are there clinical trials I could join, for example of Ruxolitinib, Dasatinib, Blinatumomab, Tisagenlecleucel?
- 13.Would a second opinion at a high-volume centre change anything, and can you help arrange it?
- 14.What supportive care (symptom control, nutrition, exercise, mental health, financial help) is available from the start?
- 15.I read that “Whether kinase inhibitors improve cure rather than early response in Ph-like ALL”. How does that affect my plan?
- 16.I read that “Screening is unavailable in most of the world, so most Ph-like patients are never identified”. How does that affect my plan?
The words I may hear
- Ph-like (BCR::ABL1-like) acute lymphoblastic leukaemia: Ph-like leukaemia has the gene-expression fingerprint of Philadelphia-positive leukaemia without the Philadelphia chromosome; behind it are dozens of kinase fusions and JAK pathway lesions, and finding which one is present tells doctors whether an imatinib-type drug or a JAK inhibitor might be added to chemotherapy.
- Philadelphia chromosome (Ph+, BCR::ABL1): A swapped piece between chromosomes 9 and 22 that fuses two genes into BCR::ABL1, a runaway kinase.
- B-ALL risk groups (NCI criteria, ETV6::RUNX1, hyperdiploidy, hypodiploidy, iAMP21, IKZF1, CNS status): Childhood leukaemia treatment is chosen from a risk table built over fifty years: age and white count at diagnosis (the NCI criteria), the chromosome pattern in the blasts (favourable ETV6::RUNX1 and high hyperdiploidy, unfavourable hypodiploidy, iAMP21, KMT2A, Ph-positive and Ph-like), whether leukaemia cells are in the spinal fluid, and above all how fast the leukaemia clears in the first month.
- Minimal / molecular residual disease (MRD): Cancer still present after treatment but too small to see on scans, detected by blood or marrow tests.
Tests and results to bring
Diagnosis: Screen every B-ALL without BCR::ABL1 for the Ph-like signature and identify the kinase lesion; treat as high risk with MRD monitoring.
Biomarker results to ask for: Ph-like gene expression signature (low-density array or RNA sequencing), CRLF2 overexpression by flow cytometry and CRLF2 rearrangement by FISH, ABL-class, JAK2 and EPOR fusions by FISH or RNA sequencing, JAK1, JAK2, IL7R and SH2B3 mutations, IKZF1 deletion, Flow cytometry MRD at end of induction and consolidation.
Scans and tests linked to this cancer: Comprehensive genomic profiling, Multiparameter flow cytometry MRD, NGS-based MRD (clonoSEQ and molecular MRD).
Bring copies of scan reports, pathology and blood results, and a list of every medicine and supplement.
The treatments I may be offered
- ABL-class fusions: Dasatinib or imatinib added to high-risk chemotherapy from induction, as for Ph-positive ALL. (Dasatinib, Imatinib, PDGFRB)
- CRLF2, JAK2 or EPOR lesions: High-risk chemotherapy; ruxolitinib added in trials (AALL1521). (Ruxolitinib, JAK2, Erythropoietin receptor (EPOR))
- Persistent residual disease: Blinatumomab or CD19 CAR T-cells to clear residual disease, then allogeneic transplant. (Blinatumomab, Tisagenlecleucel, Allogeneic stem cell transplantation)
From the standard of care recorded for this cancer; which apply depends on your stage and biomarkers. Ask which the team recommends and why.
Answers and next steps
Saved in this browserWhat I was told
Agreed next steps, dates and who to call