The first 60 days: Hairy cell leukaemia
Hairy cell leukaemia is a rare, slow B-cell leukaemia with a single defining mutation (BRAF V600E) that is unusually curable: one week of a purine analogue puts most people into remission for years, and BRAF drugs rescue those who relapse. Below, week by week, is what OnCo's record of Hairy cell 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.
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.First line, symptomaticNCCN category Category 1 (cladribine ± rituximab), NCCN Guidelines: Hairy Cell Leukemia
Cladribine (5-7 days) or pentostatin, with rituximab concurrent or delayed (improves MRD-negative CR).
Repeat purine analogue + rituximab.
Vemurafenib + rituximab (or dabrafenib-trametinib); ibrutinib; clinical trial. Moxetumomab pasudotox produced durable remissions but was withdrawn from sale in 2023 and is no longer available.
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 BRAF V600E, Flow cytometry: CD11c, CD25, CD103, CD123, MRD by flow or BRAF ddPCR, IGHV4-34 usage, MAP2K1 mutations), 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 Classic HCL, HCL variant, IGHV4-34 HCL.
- Is germline (inherited) genetic testing recommended for me or my family?Inherited variants can change treatment and matter for relatives.
First line, symptomatic
- For my situation (first line, symptomatic), which of the standard options do you recommend and why?Guideline options include: Cladribine (5-7 days) or pentostatin, with rituximab concurrent or delayed (improves MRD-negative CR).
- Am I a candidate for Cladribine, Rituximab, and what side effects should I expect?Knowing the expected toxicities helps you plan work, family, and supportive care.
Relapse after >2 years
- For my situation (relapse after >2 years), which of the standard options do you recommend and why?Guideline options include: Repeat purine analogue + rituximab.
- Am I a candidate for Cladribine, Rituximab, and what side effects should I expect?Knowing the expected toxicities helps you plan work, family, and supportive care.
Early relapse or refractory
- For my situation (early relapse or refractory), which of the standard options do you recommend and why?Guideline options include: Vemurafenib + rituximab (or dabrafenib-trametinib); ibrutinib; clinical trial. Moxetumomab pasudotox produced durable remissions but was withdrawn from sale in 2023 and is no longer available.
- Am I a candidate for Vemurafenib, Rituximab, Dabrafenib + trametinib or related drugs, 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 Vemurafenib, Dabrafenib + trametinib?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 “Variant HCL and IGHV4-34 disease respond poorly to purine analogues”. How does that affect my plan?Open problems are where trials and second opinions matter most.
- I read that “Infection risk during induction neutropenia”. 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.
- Hairy cell leukaemia: the full pageHairy cell leukaemia is a rare, slow B-cell leukaemia with a single defining mutation (BRAF V600E) that is unusually curable: one week of a purine analogue puts most people into remission for years, and BRAF drugs rescue those who relapse.
- 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.
- 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.