Skip to main content

Health condition

Anaplastic large cell lymphoma, four diseases in one name - each with its own plan.

A rare CD30-positive T-cell lymphoma. Modern UK care separates systemic ALK-positive and ALK-negative disease, primary cutaneous ALCL, and the implant-associated form - because the treatment ladder looks very different in each.

Jump to treatment
A radiographer guides a patient onto the bed of an advanced 3 Tesla MRI scanner in a London imaging suite

Why trust this guide

  • 01

    Editorial team

    Written by our editorial team from the UK sources listed on each page.

  • 02

    Sourced from guidance

    Checked against NICE, BSH, ESMO and WHO 2022 classification updates you can see at the end.

  • 03

    Current for 2026

    Reflects modern UK practice including brentuximab vedotin combinations and the BIA-ALCL pathway after the 2019 Allergan Biocell recall.

Key facts

ALCL at a glance.

The four WHO 2022 subtypes, what drives each, and where the treatment ladder diverges - in plain English.

  • What it is

    A CD30-positive mature T-cell lymphoma - a group of related diseases with very different behaviour depending on subtype.

  • WHO 2022 subtypes

    Four recognised entities - ALK-positive systemic, ALK-negative systemic, primary cutaneous, and breast implant-associated ALCL.

  • ALK-positive systemic

    Typically young adults and children - carries the t(2;5) NPM1-ALK fusion and cures in around 85 per cent with anthracycline-based chemotherapy.

  • ALK-negative systemic

    Older patients, worse prognosis overall - but the DUSP22-rearranged and TP63-rearranged subsets carry distinct outlooks.

  • Primary cutaneous ALCL

    An indolent skin disease that overlaps with lymphomatoid papulosis - excellent prognosis and often watch-and-wait.

  • BIA-ALCL

    Linked to textured breast implants (Allergan Biocell recalled 2019) - presents as a delayed seroma 8 to 12 years post-implant.

Why this guide matters

One name, four very different diseases.

A general oncology overview misses what matters here. The three points below shape everything that follows.

  • Subtype changes the plan

    ALK status, DUSP22 and TP63 rearrangements, and cutaneous versus systemic biology dictate whether you need chemotherapy at all.

  • BV-CHP is the modern first-line

    ECHELON-2 established brentuximab vedotin plus CHP over CHOP for CD30-positive T-cell lymphomas, including systemic ALCL.

  • BIA-ALCL is often cured by surgery

    Early breast implant-associated ALCL responds to en-bloc capsulectomy and implant removal alone - identifying it early is what saves lives.

How the diagnosis is made

From first symptom to a defined subtype.

A structured UK pathway - biopsy, immunohistochemistry, molecular studies and staging - that ends with a clear plan.

  1. 01

    Assessing

    History and examination

    B symptoms, lymphadenopathy, skin nodules or new unilateral breast swelling in an implant patient all point to different subtypes.

  2. 02

    Assessing

    Excisional lymph node biopsy

    A whole node - not a core - gives the architecture needed to call ALCL and separate it from other CD30-positive lymphomas.

  3. 03

    Assessing

    Immunohistochemistry

    CD30 strong and uniform, EMA positive, ALK positive or negative, CD3 variable, CD4 greater than CD8, cytotoxic markers - granzyme B, perforin and TIA1.

  4. 04

    Confirming

    Molecular and cytogenetics

    FISH or immunostain for ALK, DUSP22 and TP63 rearrangements - each carries prognostic weight in ALK-negative disease.

  5. 05

    Confirming

    PET-CT staging

    Whole-body PET-CT plus a bone marrow biopsy, LDH, LFTs and renal function to stage systemic disease.

  6. 06

    Confirming

    BIA-ALCL pathway

    Breast MRI with implant sequences, ultrasound-guided aspiration of the seroma, cytology and CD30 immunohistochemistry on the fluid.

  7. 07

    Planning

    MDT and referral

    A specialist haemato-oncology MDT decides the plan - with plastic surgery input and a recognised BIA-ALCL centre for implant-associated disease.

Typical timeline: from urgent referral to a confirmed plan in around two to three weeks.

Symptoms

What ALCL can look like.

The subtypes present very differently - systemic B symptoms and nodes, cutaneous ulcerated lesions, or a delayed seroma around a breast implant.

  • B symptoms

    Fevers, drenching night sweats and unexplained weight loss - common in systemic ALK-positive disease.

  • Lymphadenopathy

    Firm, painless enlargement of neck, axillary or groin nodes - the classic systemic presentation.

  • Extranodal involvement

    Skin, bone, soft tissue, gastrointestinal tract, bone marrow or - rarely - the central nervous system.

  • Cutaneous nodules

    Solitary or grouped, often ulcerated skin lesions in primary cutaneous ALCL - sometimes regressing on their own.

  • Delayed breast seroma

    Unilateral breast swelling and fluid around a textured implant, usually 8 to 12 years or more after surgery.

  • Capsular contracture

    Chronic tightening, firmness or distortion around a breast implant can accompany BIA-ALCL.

  • Bulky mediastinal disease

    A large chest mass with cough, breathlessness or superior vena cava pressure can be the first sign in young adults.

  • Red flag - rapid systemic decline

    Fast-growing nodes with high LDH, fevers and weight loss deserve urgent same-week haematology review.

Treatment

How ALCL is treated in the UK.

BV-CHP for systemic disease, transplant consolidation for high-risk ALK-negative cases, watch-and-wait or local therapy for cutaneous disease, and surgery-first for early BIA-ALCL.

  • BV-CHP - first-line systemic

    Brentuximab vedotin with cyclophosphamide, doxorubicin and prednisolone. ECHELON-2 showed a survival advantage over CHOP for CD30-positive T-cell lymphomas.

  • Consolidation stem cell transplant

    Upfront allogeneic transplant is considered for high-risk ALK-negative systemic disease in fit patients after remission.

  • Brentuximab vedotin monotherapy

    For relapsed or refractory systemic ALCL - response rates around 86 per cent, curative in a subset when followed by transplant.

  • Watch and wait - cutaneous

    Solitary primary cutaneous lesions often regress spontaneously - active surveillance is a legitimate first step.

  • Local therapy - cutaneous

    Excision alone or involved-site radiotherapy for localised primary cutaneous ALCL - excellent long-term outcomes.

  • Methotrexate or brentuximab

    Low-dose methotrexate or brentuximab vedotin for multifocal or recurrent primary cutaneous disease.

  • En-bloc capsulectomy - BIA-ALCL

    Early-stage BIA-ALCL is cured in over 90 per cent by complete capsulectomy and implant removal alone.

  • BV-CHP for advanced BIA-ALCL

    Later-stage or invasive BIA-ALCL adds BV-CHP to complete surgery and is coordinated through a specialist sarcoma or lymphoma centre.

What this guide is based on

The sources behind every claim on this page.

WHO classification, UK and European specialist guidance, and the trials that shaped current practice.

Key references

Guidelines and standards we relied on.

A quiet reminder

This guide is for information, not medical advice.

Your haematology or oncology team knows your case in full. If something has changed or you are unsure, contact them - do not wait for the next scheduled appointment.

  • WHO Classification of Haematolymphoid Tumours, 5th edition (2022).

  • British Society for Haematology (BSH). Guidelines on the management of mature T-cell lymphomas.

  • ESMO Clinical Practice Guidelines. Peripheral T-cell lymphomas.

  • Horwitz S et al. ECHELON-2 trial - brentuximab vedotin with CHP versus CHOP.

  • MHRA and FDA. Field safety notices - Allergan Biocell textured implant recall (2019) and BIA-ALCL guidance.

  • NICE. Brentuximab vedotin for CD30-positive lymphoma technology appraisals.

Red flags

When ALCL needs urgent attention.

Situations where you should contact your team the same day - or attend A and E rather than wait for a clinic slot.

  • Superior vena cava obstruction

    Face and neck swelling, distended neck veins and breathlessness from a bulky mediastinal mass - a same-day emergency.

  • Tumour lysis syndrome

    Rapid cell breakdown after starting chemotherapy - biochemistry needs close monitoring in high-burden disease.

  • CNS involvement

    New headache, cranial nerve signs, seizures or altered behaviour in a known ALCL patient warrant urgent imaging and lumbar puncture.

  • New delayed breast seroma

    Any spontaneous swelling around a breast implant more than a year after surgery needs BIA-ALCL exclusion, not simple drainage.

  • Capsular mass or skin change

    A palpable mass, skin ulceration or nodal swelling around an implant is a red flag for invasive BIA-ALCL.

  • Rapidly enlarging cutaneous lesion

    A skin nodule that does not regress or that grows quickly can signal transformation - biopsy rather than watch.

  • High LDH and B symptoms

    Rising LDH with fevers and weight loss points to aggressive systemic disease that needs urgent staging.

  • Neutropenic sepsis

    Any fever above 38 degrees on chemotherapy is a medical emergency - go straight to A and E with your alert card.

  • Post-transplant complications

    Graft-versus-host disease, infection or new neurology after allogeneic transplant needs specialist review.

Living with it

A rare cancer, with a real chance of cure.

Four things that make the biggest difference through treatment and beyond - a specialist team, structured follow-up, awareness of your implant history, and support for the head as well as the body.

A quiet reminder

Rare does not mean untreatable.

ALK-positive systemic ALCL cures in around 85 per cent of patients, and early BIA-ALCL is often cured by surgery alone. Getting to the right team is half of the work.

  1. 01 Team

    A specialist MDT matters

    ALCL is uncommon - care in a centre familiar with T-cell lymphomas gives you access to trials and the right transplant pathway.

  2. 02 Follow-up

    Structured surveillance

    PET-CT and clinic reviews on a set cadence pick up relapse early - most relapses happen in the first two years.

  3. 03 Implants

    Know your device

    If you have breast implants, keep the make, model and surgery date on file - and report any new swelling or firmness promptly.

  4. 04 Support

    Mind as well as body

    A rare-cancer diagnosis is heavy - Lymphoma Action, Macmillan and a clinical psychologist can help you carry it.

Frequently asked

Everything we get asked about ALCL.

Quick answers on subtypes, ALK status, first-line therapy, breast implants, cutaneous disease and relapse.

  • What is anaplastic large cell lymphoma?

    ALCL is a mature T-cell lymphoma defined by strong, uniform expression of the CD30 protein. The WHO 2022 classification recognises four distinct diseases - ALK-positive systemic ALCL, ALK-negative systemic ALCL, primary cutaneous ALCL and breast implant-associated ALCL - each with different behaviour and treatment.

  • What does ALK positive or negative mean?

    ALK is a gene on chromosome 2. In ALK-positive systemic ALCL, a translocation - most often t(2;5)(p23;q35) creating NPM1-ALK - drives the disease and predicts a good response to anthracycline-based chemotherapy. ALK-negative disease is genetically more mixed, though DUSP22-rearranged tumours tend to do better and TP63-rearranged tumours worse.

  • How is systemic ALCL treated in 2026?

    First-line therapy is BV-CHP - brentuximab vedotin combined with cyclophosphamide, doxorubicin and prednisolone. The ECHELON-2 trial showed a survival advantage over CHOP for CD30-positive T-cell lymphomas. High-risk ALK-negative disease is often consolidated with an allogeneic stem cell transplant in fit patients.

  • What is BIA-ALCL and how common is it?

    Breast implant-associated ALCL is a rare, ALK-negative CD30-positive lymphoma that develops around a textured breast implant - most commonly the Allergan Biocell design that was recalled in 2019. It typically appears as a delayed seroma 8 to 12 years or more after implant surgery. Early-stage disease is cured in over 90 per cent by complete capsulectomy and implant removal alone.

  • What are the symptoms of primary cutaneous ALCL?

    Solitary or grouped, sometimes ulcerated skin nodules - part of the CD30-positive lymphoproliferative disease spectrum that overlaps with lymphomatoid papulosis. Lesions often regress spontaneously, and even without treatment the long-term prognosis is excellent. Widespread or persistent disease is treated with excision, radiotherapy, low-dose methotrexate or brentuximab vedotin.

  • What happens if ALCL comes back?

    Relapsed or refractory systemic ALCL responds well to brentuximab vedotin monotherapy - around 86 per cent of patients respond, and a proportion can be cured when the response is consolidated with an autologous or allogeneic stem cell transplant. Clinical trials of newer targeted agents and bispecific antibodies are worth discussing with your MDT.

Related content

Keep reading.