The first 60 days: Acute promyelocytic leukaemia
Acute promyelocytic leukaemia is caused by a single fused gene that freezes blood cells at an immature stage and triggers dangerous bleeding. Two non-chemotherapy drugs, a vitamin A derivative and arsenic trioxide, make the cells mature and cure more than nine in ten patients. Below, week by week, is what OnCo's record of Acute promyelocytic leukaemia 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.
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: Molecular relapse.
- Medical oncologistNamed in the standard of care for: Suspected APL, first hours, Low- and intermediate-risk, High-risk, Molecular relapse.
- Transplant and cell therapy teamNamed in the standard of care for: Molecular relapse.
- Palliative and supportive care teamNamed in the standard of care for: Suspected APL, first hours, Low- and intermediate-risk.
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.
All-trans retinoic acid plus arsenic trioxide induction and consolidation without chemotherapy (APL0406); dexamethasone prophylaxis or treatment for differentiation syndrome.
All-trans retinoic acid plus arsenic trioxide with idarubicin or gemtuzumab ozogamicin added during induction to control the white count.
Start all-trans retinoic acid on morphological suspicion; transfuse platelets and fibrinogen to targets; avoid invasive procedures until coagulopathy is controlled.
Arsenic-based salvage to molecular remission, then autologous transplant if PML::RARA negative, allogeneic transplant if not.
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 PML::RARA fusion by PCR or FISH, tkaryotype, White cell count at presentation, Fibrinogen, D-dimer and platelet count, PML::RARA transcript MRD after consolidation), 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 Low- and intermediate-risk APL, High-risk APL, Variant APL with RARA fusions other than PML.
- Is germline (inherited) genetic testing recommended for me or my family?Inherited variants can change treatment and matter for relatives.
Suspected APL, first hours
- For my situation (suspected apl, first hours), which of the standard options do you recommend and why?Guideline options include: Start all-trans retinoic acid on morphological suspicion; transfuse platelets and fibrinogen to targets; avoid invasive procedures until coagulopathy is controlled.
- Am I a candidate for Tretinoin (all-trans retinoic acid, ATRA), and what side effects should I expect?Knowing the expected toxicities helps you plan work, family, and supportive care.
Low- and intermediate-risk
- For my situation (low- and intermediate-risk), which of the standard options do you recommend and why?Guideline options include: All-trans retinoic acid plus arsenic trioxide induction and consolidation without chemotherapy (APL0406); dexamethasone prophylaxis or treatment for differentiation syndrome.
- Am I a candidate for Tretinoin (all-trans retinoic acid, ATRA), Arsenic trioxide, Dexamethasone, and what side effects should I expect?Knowing the expected toxicities helps you plan work, family, and supportive care.
High-risk
- For my situation (high-risk), which of the standard options do you recommend and why?Guideline options include: All-trans retinoic acid plus arsenic trioxide with idarubicin or gemtuzumab ozogamicin added during induction to control the white count.
- Am I a candidate for Tretinoin (all-trans retinoic acid, ATRA), Arsenic trioxide, Idarubicin or related drugs, and what side effects should I expect?Knowing the expected toxicities helps you plan work, family, and supportive care.
Molecular relapse
- For my situation (molecular relapse), which of the standard options do you recommend and why?Guideline options include: Arsenic-based salvage to molecular remission, then autologous transplant if PML::RARA negative, allogeneic transplant if not.
- Am I a candidate for Arsenic trioxide, 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 Arsenic trioxide, Tretinoin (all-trans retinoic acid, ATRA)?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 “Early haemorrhagic death before treatment starts, especially where diagnosis is slow”. How does that affect my plan?Open problems are where trials and second opinions matter most.
- I read that “Treatment of variant RARA fusions that do not respond to arsenic or retinoic acid”. How does that affect my plan?Open problems are where trials and second opinions matter most.
Much of what helps in the first weeks is free if you know to ask: testing, helplines, rides and lodging, second opinions, trial travel.
- Acute promyelocytic leukaemia: the full pageAcute promyelocytic leukaemia is caused by a single fused gene that freezes blood cells at an immature stage and triggers dangerous bleeding. Two non-chemotherapy drugs, a vitamin A derivative and arsenic trioxide, make the cells mature and cure more than nine in ten patients.
- 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.
- Differentiation syndrome: When a targeted drug makes leukaemia cells mature all at once, causing fever, fluid in the lungs, and weight gain.
- Cytogenetics and karyotype: Looking at a cancer's chromosomes under the microscope to find missing, extra, broken or swapped pieces.
- 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.