The first 60 days: Philadelphia chromosome-like acute lymphoblastic leukaemia (Ph-like or BCR::ABL1-like ALL)
Ph-like leukaemia behaves like Philadelphia chromosome-positive leukaemia, with the same kind of overactive growth signalling, but lacks the BCR::ABL1 gene itself. It is caused by a scattered set of gene fusions and mutations, many of them blockable by existing kinase pills such as dasatinib or ruxolitinib, and it is now screened for at diagnosis so those drugs can be tried. Below, week by week, is what OnCo's record of Philadelphia chromosome-like acute lymphoblastic leukaemia (Ph-like or BCR::ABL1-like ALL) says about the first two months: the order is typical, the timing is yours to ask about. Sections appear only where the record has something to say. Orientation, not medical advice.
What happens now
Staging tests establish exactly what and where the cancer is. Everything else follows from the answers.
Screen every B-ALL without BCR::ABL1 for the Ph-like signature and identify the kinase lesion; treat as high risk with MRD monitoring.
Ask which of these were tested and what the results were; see the report reader for what each value means.
These specialties appear in the standard of care for this cancer. In most centres they meet weekly as a tumour board to agree each plan; you can ask when yours was discussed and what was decided.
- PathologistNamed in the standard of care for: Diagnosis.
- Medical oncologistNamed in the standard of care for: ABL-class fusions, CRLF2, JAK2 or EPOR lesions, Persistent residual disease.
- Transplant and cell therapy teamNamed in the standard of care for: CRLF2, JAK2 or EPOR lesions, Persistent residual disease.
A second opinion from a centre that treats many similar cases is normal, not rude; the standard of care tells you what to compare it against.
Each row is a setting from the standard of care, in the order it usually arises. Not all will apply to you; your stage and biomarkers decide which do. The guideline grade, where recorded, says how strong the evidence is.
- 1.ABL-class fusionsNCI PDQ: Childhood Acute Lymphoblastic Leukemia Treatment (health professional version)
Dasatinib or imatinib added to high-risk chemotherapy from induction, as for Ph-positive ALL.
- 2.CRLF2, JAK2 or EPOR lesionsNCI PDQ: Childhood Acute Lymphoblastic Leukemia Treatment (health professional version)
High-risk chemotherapy; ruxolitinib added in trials (AALL1521).
- 3.Persistent residual diseaseNCI PDQ: Childhood Acute Lymphoblastic Leukemia Treatment (health professional version)
Blinatumomab or CD19 CAR T-cells to clear residual disease, then allogeneic transplant.
Generated from this cancer's standard of care, biomarkers and open problems. Take the group that matches where you are. To tick, add your own and print, open the one-page appointment sheet.
Newly diagnosed
- What is my exact diagnosis, stage, and grade, and which tests established them?Everything else follows from an accurate stage and subtype.
- 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?These results decide eligibility for targeted therapy, immunotherapy, and trials.
- Which subtype is my cancer, and does that change the recommended treatment?Recognised subtypes for this cancer include Ph-like ALL with CRLF2 rearrangement, Ph-like ALL with ABL-class fusions, Ph-like ALL with JAK2 or EPOR rearrangements.
- Is germline (inherited) genetic testing recommended for me or my family?Inherited variants can change treatment and matter for relatives.
Diagnosis
- For my situation (diagnosis), which of the standard options do you recommend and why?Guideline options include: Screen every B-ALL without BCR::ABL1 for the Ph-like signature and identify the kinase lesion; treat as high risk with MRD monitoring.
ABL-class fusions
- For my situation (abl-class fusions), which of the standard options do you recommend and why?Guideline options include: Dasatinib or imatinib added to high-risk chemotherapy from induction, as for Ph-positive ALL.
- Am I a candidate for Dasatinib, Imatinib, and what side effects should I expect?Knowing the expected toxicities helps you plan work, family, and supportive care.
CRLF2, JAK2 or EPOR lesions
- For my situation (crlf2, jak2 or epor lesions), which of the standard options do you recommend and why?Guideline options include: High-risk chemotherapy; ruxolitinib added in trials (AALL1521).
- Am I a candidate for Ruxolitinib, and what side effects should I expect?Knowing the expected toxicities helps you plan work, family, and supportive care.
Persistent residual disease
- For my situation (persistent residual disease), which of the standard options do you recommend and why?Guideline options include: Blinatumomab or CD19 CAR T-cells to clear residual disease, then allogeneic transplant.
- Am I a candidate for Blinatumomab, Tisagenlecleucel, and what side effects should I expect?Knowing the expected toxicities helps you plan work, family, and supportive care.
Any stage
- Are there clinical trials I could join, for example of Ruxolitinib, Dasatinib, Blinatumomab, Tisagenlecleucel?Trials are how the next standard of care is set; asking early keeps options open.
- Would a second opinion at a high-volume centre change anything, and can you help arrange it?Rare or high-stakes decisions benefit from a centre that treats many similar patients.
- What supportive care (symptom control, nutrition, exercise, mental health, financial help) is available from the start?Supportive care improves quality of life and helps patients complete treatment.
- I read that “Whether kinase inhibitors improve cure rather than early response in Ph-like ALL”. How does that affect my plan?Open problems are where trials and second opinions matter most.
- 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?Open problems are where trials and second opinions matter most.
Trials open now for this cancer in OnCo, largest phase first. Joining a trial is a decision like any other: ask what the comparison arm is, whether a placebo is used, and what happens if you leave. The cancer page searches ClinicalTrials.gov live for more.
Much of what helps in the first weeks is free if you know to ask: testing, helplines, rides and lodging, second opinions, trial travel.
- Philadelphia chromosome-like acute lymphoblastic leukaemia (Ph-like or BCR::ABL1-like ALL): the full pagePh-like leukaemia behaves like Philadelphia chromosome-positive leukaemia, with the same kind of overactive growth signalling, but lacks the BCR::ABL1 gene itself. It is caused by a scattered set of gene fusions and mutations, many of them blockable by existing kinase pills such as dasatinib or ruxolitinib, and it is now screened for at diagnosis so those drugs can be tried.
- One-page appointment sheetYour questions, the words you may hear, what to bring, and space for the answers. Print it.
- Treatment sequencingWhich treatment tends to follow which, line by line.
- NavigatorStandard of care for your stage, what you have tried, and trials near you.
- 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.
Every term links to the glossary.