A very rare blood cancer, this lymphoma may be due to a chromosomal abnormality. Another form, related to breast implant placement, has also been observed. What symptoms? What treatments?
What is anaplastic large cell lymphoma?
Anaplastic large cell lymphoma is a blood cancer that originates from T cells, a type of white blood cells in the lymphatic system. It is a rare lymphoma, about 2% of non-Hodgkin’s lymphomas. According to Erika Brunet, Inserm Research Director at the Imagine Institute, this disease can take several forms:
► The systemic forms, which thus affect several organs such as the lymph nodes, skin, liver or spleen.
► The ALK positive : they are so called because they express the ALK protein. The average age of people affected by this form is 34 years. It is also the form that is found more in children.
► The ALK negatives : these do not express the protein and affect older people (average age 54 years).
Other forms:
What are the symptoms ?
“Symptoms are similar to those of other lymphomas: fever, fatigue, weight loss, sweating and possibly the presence of a mass in the lymph nodes. In the cutaneous form, there are spots on the skin.
What causes ALCL?
The positive ALK form is due to a chromosomal abnormality : two pieces of chromosomes are exchanged and the protein is activated at the junction. “But we still don’t know what drives this in the first place. As for non-ALK forms, the cause is unknown, we don’t have evidence of a specific genetic abnormality or environmental factor.”
What are the risks for women who have breast prostheses?
Breast implant-related anaplastic large cell lymphoma is a very different form of lymphoma. According to the National Cancer Institute, the first case in France appeared in 2009. “This lymphoma generally occurs with micro-textured implants and develops around the prosthesis. Fortunately, this is a very rare form, but in this particular case the cause is clear. One of the hypotheses put forward is that this type of dentures could create chronic inflammation due to the presence of bacteria which would then adhere to the prosthesis to form a kind of bio-film“, explains Erika Brunet. Lymphoma can sometimes be diagnosed 7 to 15 years after the implant is placed. There are two types of anaplastic large cell lymphoma linked to breast implants:
► One in situ form that is limited to the capsulethat is, the membrane around the implant;
► One infiltrating form with a mass adjacent to the peri-prosthetic capsule.
Certain signs should alert and encourage the woman to consult her surgeon: a periprosthetic effusion significant, a breast enlargementpain, inflammation, mass, ulceration, deterioration of general condition.
What life expectancy?
The ALK positive subtype can be treated well with chances of survival up to 90%. ALK negative lymphoma has a prognosis less good by 50 to 60%. With regard to breast implant-related lymphoma, the chance of survival”seems worse in patients with an infiltrative form, that is, a tumor extending beyond the capsule“, explains the National Cancer Institute.
What treatment to treat anaplastic lymphoma?
The treatment corresponds first to a combination of conventional chemotherapies. In the event of a recurrence, more targeted and increasingly effective treatments can be proposed. Finally, if necessary, a graft stem cells may be considered for ALK-positive lymphoma.
Thanks to Erika Brunet, research director at Inserm.