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8 slides generated from the cancer page, with a quiz from the open benchmark and speaker notes that cite the sources. Arrow keys move between slides; Print gives one slide per page.
An aggressive T-cell lymphoma that looks like its ALK-positive sibling under the microscope and carries the same CD30 marker, but lacks the broken ALK gene. It affects older people and is cured less often, and several genetic changes inside it predict very different outcomes.
What it is. A lymphoma of T cells that looks anaplastic, carries uniform strong CD30 and has no ALK rearrangement. The definition is therefore partly negative, which is why WHO-HAEM5 describes it as a heterogeneous entity: it is what is left when ALK-positive disease, breast implant-associated disease and the skin lymphomas have been excluded.
How it differs from its ALK-positive sibling. In age, and in outcome. ALK-positive disease affects people in their twenties and thirties; this affects people around 70. Both carry CD30, both are treated with the same first-line regimen, and ALK-positive disease is cured considerably more often. Where the appearance is identical, only the ALK stain tells them apart, which is why it is done on every anaplastic lymphoma.
| Setting | Approach | Guideline |
|---|---|---|
| Making the diagnosis | CD30 and ALK immunohistochemistry on the biopsy. The diagnosis is partly a negative one: uniform strong CD30 with an anaplastic appearance and no ALK, in a lymphoma that is not confined to the skin and is not associated with a breast implant. Those two exclusions matter because both of them are treated very differently. Testing for DUSP22 and TP63 rearrangements is done where it is available, with the caution that WHO-HAEM5 does not regard the resulting groups as established subtypes. | not mapped |
| First-line treatment | Brentuximab vedotin with cyclophosphamide, doxorubicin and prednisone, the same regimen as for ALK-positive disease and on the same trial: in ECHELON-2, five-year progression-free survival was 51.4 per cent against 43.0 with chemotherapy alone and overall survival 70.1 against 61.0. Consolidating a first remission with high-dose therapy and an autologous stem cell transplant is standard practice here, unlike in ALK-positive disease, and it rests on a single-arm study and registry comparisons rather than on a randomised trial; a patient is entitled to be told that. The regimens are on the peripheral T-cell lymphoma page. | not mapped |