CION Cancer Clinics
Anaplastic large cell lymphoma and what ALK status means | CION Cancer Clinics
ALK-positive and ALK-negative are the two main forms of anaplastic large cell lymphoma (ALCL), a rare T-cell lymphoma. ALK-positive ALCL usually affects younger people and tends to respond better to treatment. ALK-negative ALCL is more common in older adults and comes back more often. The result comes from a stain on the biopsy. This page explains both, the other ALCL types, and how treatment is planned. At CION Cancer Clinics, our haematology team plans myeloma and lymphoma care with you, discussed at a tumour board and explained in plain words.
On this page
- What does ALK-positive or ALK-negative mean in ALCL?
- How do ALK-positive and ALK-negative ALCL differ?
- Which kind of ALCL is on your report?
- How is ALCL confirmed and staged?
- How is systemic ALCL usually treated?
- What do people often get wrong about ALCL?
- What do the words on the pathology report mean?
- Common questions about ALCL and ALK status
The short answer
What does ALK-positive or ALK-negative mean in ALCL?
ALK-positive and ALK-negative are the two main forms of anaplastic large cell lymphoma, or ALCL. The label tells you whether the lymphoma cells make an abnormal protein called ALK. ALK-positive ALCL tends to affect children and younger adults and usually responds better to treatment. ALK-negative ALCL tends to affect older adults and needs a more careful plan.
What ALCL is
ALCL is a rare lymphoma that starts in T cells, a type of white blood cell that helps fight infection. Under the microscope the cells are large and carry a marker called CD30 on their surface. It usually shows up as swollen lymph nodes, often with fevers, night sweats or weight loss. It can also affect the skin, bones or other organs.
Why the ALK result matters so much
The ALK protein is made when two genes swap pieces, which switches on growth signals inside the cell. Its presence changes how doctors think about the outlook and, sometimes, the plan after first treatment. The result comes from a stain on your biopsy, so it is on the pathology report rather than on a blood test.
What to do with the result
Read the ALK result together with the rest of the report, not on its own. The stage, where the lymphoma is, your age and your general health all shape the plan. If the report does not mention ALK at all, ask whether the stain was done. It is a routine part of confirming ALCL, and treatment decisions lean on it.
ALCL is not the same as the far commoner B-cell lymphomas. Make sure the report you are reading says T-cell or ALCL.Side by side
How do ALK-positive and ALK-negative ALCL differ?
Not sure whether this applies to you?
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Which kind of ALCL is on your report?
The name ALCL covers four conditions that behave very differently. The word before ALCL on the report matters.
Systemic ALK-positive
Affects lymph nodes and sometimes other organs. The form most often seen in young people. Treated with combination chemotherapy, and often responds well.
Systemic ALK-negative
Looks similar under the microscope but lacks ALK. Treated with combination chemotherapy, with extra thought given to what follows first treatment, because it is more likely to return.
Primary cutaneous ALCL
Stays in the skin as one or a few lumps that may break down. It usually grows slowly and is often treated with local radiotherapy or removal rather than chemotherapy.
Breast implant-associated ALCL
A rare lymphoma in the fluid or scar capsule around a textured breast implant. Often treated by removing the implant and capsule.
Usually shows as
- Late swelling around an implant
- A lump near the implant
Getting the diagnosis right
How is ALCL confirmed and staged?
A proper biopsy
Usually a whole lymph node or a core of tissue, not a fine needle sample. ALCL can be mistaken for other cancers, so enough tissue matters.
Special stains
Immunohistochemistry, meaning stains that show proteins on cells, checks for CD30, ALK and T-cell markers. Some reports add gene tests, which can take a little longer to come back.
Staging scans
A PET-CT shows which areas are involved. Blood tests check the liver, kidneys and blood counts. Some people need a bone marrow test.
Expert review
Because it is rare, a second look at the slides by a lymphoma pathologist is often worthwhile before treatment starts. It rarely delays the plan for long, and it can change the diagnosis.
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Treatment
How is systemic ALCL usually treated?
Most adults with systemic ALCL receive combination chemotherapy. Because the cells carry CD30, many plans now include brentuximab vedotin, an antibody that carries a chemotherapy drug straight to CD30-carrying cells. It is given with other chemotherapy drugs as a drip in day care. Children are usually treated on paediatric protocols.
After first treatment
For ALK-negative ALCL, some haematologists suggest a stem cell transplant using your own cells once remission is reached. Evidence on who gains most is still being gathered. CION's haematology team assesses the case and, if a transplant is advised, coordinates referral to a qualified centre.
Who a given plan may not suit
Brentuximab can cause numbness or tingling in the hands and feet, so people who already have nerve damage from diabetes may need a different mix. Intensive plans and transplant do not suit everyone with heart, kidney or lung problems.
What this page cannot tell you
It cannot tell you your own outlook, or which plan fits you. Ask your haematologist what your ALK result, stage and health mean together.
Commonly believed
What do people often get wrong about ALCL?
It is a real result. It means the lymphoma cells do not make the ALK protein. It describes a different form of ALCL, not a missing answer.
There are many kinds, and they behave differently, so outlook figures read online for one kind do not apply to another. ALCL, especially the ALK-positive form, often responds better than several other T-cell lymphomas.
Primary cutaneous ALCL usually stays in the skin and is often treated locally. Staging tests show whether it is confined to the skin.
Implant-associated ALCL is rare. Removal is not routinely advised without symptoms. New swelling or a lump near an implant needs a doctor's review.
On your report
What do the words on the pathology report mean?
- Anaplastic
- Cells that look very unlike normal cells under the microscope.
- CD30
- A marker on the cell surface. All ALCL carries it, which is why brentuximab can target it.
- ALK
- Anaplastic lymphoma kinase, a protein made when two genes join abnormally.
- Hallmark cells
- Large cells with horseshoe-shaped nuclei that pathologists look for in ALCL.
- DUSP22 or TP63 rearrangement
- Gene changes sometimes found in ALK-negative ALCL that help predict how it may behave.
Questions we are asked
Common questions about ALCL and ALK status
Is ALK-positive ALCL less serious than ALK-negative?
In general, ALK-positive ALCL responds better to first treatment and has a more favourable outlook. It is still a serious lymphoma that needs prompt treatment. Stage, other gene findings and overall health also matter, so ask your haematologist what your own results mean.
My son is a teenager. Where should he be treated?
Children and teenagers with ALCL are usually treated on protocols designed for young people, by teams experienced in paediatric or young adult lymphoma. CION's haematology team can review the reports and help coordinate care with a suitable centre.
Can the biopsy be checked again?
Yes. Because ALCL is rare and can resemble other conditions, a second pathology review is sensible. Ask the laboratory for the tissue blocks and slides, along with the report. The reviewing team may run extra stains or gene tests on the same tissue.
Will he lose his hair during treatment?
Most combination chemotherapy plans used for ALCL do cause hair loss, which usually grows back after treatment. Other common effects include tiredness, a higher risk of infection and feeling sick. The team will explain what to expect with your plan.
What if ALCL comes back after treatment?
Options may include brentuximab if it was not used before, other chemotherapy, a transplant at a qualified centre, or, for ALK-positive disease, tablets that block ALK. A clinical trial may be worth asking about. The choice depends on earlier treatment and fitness.
Is ALCL caused by something we did?
No. Systemic ALCL is not caused by food, lifestyle or anything a parent did. The gene change behind ALK-positive disease happens inside the cells by chance. Apart from the link with textured breast implants, no clear cause is known.
Which fever needs urgent care during treatment?
Any fever or shivering during chemotherapy needs a same-day call to your team, because blood counts may be low. If the person is confused, breathless or very unwell, go to the nearest emergency department or call 108, and say they are on chemotherapy.
Is treatment covered by Aarogyasri or insurance?
Chemotherapy for lymphoma is often covered under Aarogyasri, PM-JAY, CGHS, ECHS, EHS or cashless insurance. Newer antibody drugs may have different rules, and scheme rules change. Call the helpline with your card details and the team will help you check current cover.
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Sources
- Leukemia & Lymphoma Society — Non-Hodgkin lymphoma
- National Cancer Institute — Adult Non-Hodgkin Lymphoma Treatment (PDQ) - Patient Version
- Cancer Research UK — Types of non-Hodgkin lymphoma
- American Cancer Society — Non-Hodgkin Lymphoma
This page is general information, not a prescription. Do not change or stop any treatment based on what you read here. If anything is worrying you, contact your own treating team — or call our helpline and we will help you reach the right specialist.
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