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Anaplastic large cell lymphoma

Anaplastic Large Cell Lymphoma

Anaplastic Large Cell Lymphoma (ALCL) is a rare T-cell form of non-Hodgkin’s lymphoma. It generally follows an indolent course and has a favourable prognosis. There are two forms of ALCL.

The cutaneous form primary affects the skin. It can also eventually spread to the lymph nodes. This type has a very good prognosis and follows an indolent course. The systemic type of ALCL affects lymph nodes, extranodal sites and the skin in many patients. It has a more aggressive clinical course and a less favourable outcome. (1) (2) (3)

Diagnosis and prognosis

Diagnosis is usually made when the patient presents with skin lesions, and/or swollen lymph nodes. Extranodal disease is common for systemic ALCL. As with all lymphomas a full work up is required, including blood tests, CT and/or PET scan and of course a surgical biopsy to confirm the diagnosis.

Many studies find that the prognosis is similarly to similarly staged DLBC patients. The International Prognostic Index may be applicable and help guide treatment decisions.

Patients expressing the CD30 antigen generally have a more favourable prognosis, as do patients who express the Anaplastic Lymphoma Kinase protein (ALK+) (1) (2)

Treatment

Cutaneous ALCL can be treated with radiation, methotrexate, surgery or other treatments. (6) Treatment options for systemic ALCL generally include multi-agent chemotherapy similar to diffuse large B-cell lymphoma and may include CHOP or CHOP like chemotherapy.  The regimen DA-EPOCH appears to get superior results due to the addition of Etoposide. (9) Rituximab is not used because it targets the CD20 antigen found only on B-cells. However the monoclonal antibody Brentuximab Vedotin is used for T-cell lymphoma. It targets the CD30 antigen found on most ALCL cells, and has been showing some impressive results in CD30+ lymphomas. (4) (5)

Another option that has shown promising results, particularly in the paediatric population is Vinblastine, whether as a single agent or in combination with other drugs. (7) (8)

Patients with the systemic form of ALCL or relapsed ALCL should consider clinical trials. Clinical trials often offer the opportunity to receive newer more effective therapies, which can be an important consideration for patients with difficult to treat disease.

Click here for clinical trials for any form of ALCL

Click here for clinical trials for relapsed ALCL

Additional reading

Click here to get the quick "5 minute journal" edition of slides showing the results of the Brentuximab study.

Click here to read the 2011 ASH abstract giving the details of the study.

Current Management of Primary Cutaneous CD30+ T-cell Lymphoproliferative Disorders

More information about Anaplastic Large Cell Lymphoma

Visit the ALCL website for more information

References

  1. Anaplastic large cell lymphoma: changes in the World Health Organization classification and perspectives for targeted therapy

  2. Anaplastic Large-Cell Lymphoma, T-/Null-Cell Type

  3. Primary Cutaneous and Systemic Anaplastic Large Cell Lymphoma (free membership required)

  4. Phase 3 trial of brentuximab vedotin and CHP versus CHOP in the frontline treatment of patients (pts) with CD30+ mature T-cell lymphomas (MTCL).

  5. Complete remissions with weekly dosing of SGN-35, a novel antibody-drug conjugate (ADC) targeting CD30, in a phase I dose-escalation study in patients with relapsed or refractory Hodgkin lymphoma (HL) or systemic anaplastic large cell lymphoma (sALCL).

  6. Anaplastic Large Cell Lymphoma (from Medscape)

  7. Single-Drug Vinblastine As Salvage Treatment for Refractory or Relapsed Anaplastic Large-Cell Lymphoma: A Report From the French Society of Pediatric Oncology

  8. Vinblastine in children and adolescents with high-risk anaplastic large-cell lymphoma: results of the randomized ALCL99-vinblastine trial.

  9. The outcome of ALK positive and ALK negative anaplastic large cell lymphoma (ALCL) following DA-EPOCH.


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