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Anaplastic Large Cell Lymphoma
DescriptionAnaplastic Large Cell Lymphoma (ALCL) is a T-cell form of non-hodgkin's lymphoma. It generally follows an indolent course and has a favourable prognosis. It is considered to be one of the Cutaneous T-cell Lymphoma's (CTCL) when it primarily affects the skin. But there is also a systemic type which is quite different in its course. On this page we will try to present information on each type.
Here is an excellent link which gives extensive information about ALCL in all its forms. It discusses epidemiology, presentation, treatment and more. Unfortunately this article used to be free, but now the journal The Oncologist requires membership to read it, or your can purchase it for $25 USD. If you have ALCL it might be worth the cost. ALCL, an in-depth discussion of all types from the journal The Oncologist
Another comprehensive review of primary cutaneous anaplastic large cell lymphoma from The CancerNetwork Current Management of Primary Cutaneous CD30+ T-cell Lymphoproliferative Disorders
The Cancer network's T-cell lymphoma series. Primary Cutaneous and Systemic Anaplastic Large Cell Lymphoma
Single agent Vinblastine has shown some remarkable results. Below is the link to a small study in which 83% of patients being treated for relapsed ALCL achieved a complete remission with Vinblastine.
CTCL Large cell, CD30 Positive / Anaplastic Large Cell Lymphoma (ALCL)Cutaneous Anaplastic Large Cell Lymphoma (ALCL) falls under the category of Cutaneous T-cell lymphomas. There is also another form of Anaplastic Large Cell Lymphoma (ALCL) which is systemic in nature meaning that it arose somewhere other than the skin, although it too can spread to the skin. The cutaneous form has a very favourable prognosis, while the systemic form is less favourable. In both cases being CD30+ (CD30 is also called Ki-1) improves the prognosis and response to treatment. More information about Cutaneous Anaplastic Large Cell Lymphoma
More information about general Anaplastic Large Cell Lymphoma
Systemic Anaplastic Large Cell LymphomaSystemic ALCL is a T-cell lymphoma that is quite rare. It represents only about 2-8% of all lymphomas. It often expresses the Anaplastic Lymphoma Kinase (ALK) protein on the surface of the cells. Patients who are ALK+ have a more favourable prognosis than those who are ALK-
Diagnosis and prognosisDiagnosis is usually made when the patient presents with swollen lymph nodes. Extranodal disease is very 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.
Most studies find that the prognosis is similarly to similarly staged DLBC patients. The International Prognostic Index may be applicable and help guide treatment decisions.
TreatmentTreatment options are similar as for diffuse large B-cell lymphoma and may include CHOP or CHOP like chemotherapy. Rituximab is not used because it targets the CD20 antigen found only on B-cells. However there are monoclonal antibodies in development for T-cell lymphoma which target the CD30 antigen found on ALCL cells. Here is the early results from one clinical trial..
Recent phase II clinical trials of Brentuximab vedotin (SGN-35) have been showing very positive results. This drug is a combination of a monoclonal antibody and a drug. Initial studies of the monoclonal antibody (directed against the CD30 antigen) showed modest results, but the antibody did not kill enough of the cancer cells to be a huge success. But when it was conjugated to a drug it was found that the antibody could attach to the cell and the drug could kill it.
Click here to read the 2011 ASH abstract giving the details of the study.
Visit the ALCL website for more information
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