large cell lymphoma | Anaplastic lymphoma

Anaplastic lymphoma




 Ooreka anaplastic lymphoma, or large cell anaplastic lymphoma, is a rather rare T-type lymphoma. Indeed, it does not represent more than 2% of all cases of non-Hodgkin lymphomas. Like Burkitt's lymphoma, mantle lymphoma, or large B-cell diffuse lymphoma, this cancer is aggressive.

Anaplastic lymphoma: the ALK gene involved
Although it can affect people of any age, anaplastic lymphoma mainly concerns teenagers and little boys. It alone accounts for 14% of the cases of children's lymphomas.

Anaplastic lymphomas are generally associated with chromosomal translocation (chromosomes exchange some of their genetic material). These reshuffles affect the ALK gene, a form of tyrosine kinase, an enzyme that activates or blocks many cellular functions.

Most of the time, lymphomas that express the ALK (ALK positive) gene concern young people. Negative ALK anaplastic lymphoma is found in older people. It is also more aggressive and less good prognosis.

Two types of anaplastic lymphomas: cutaneous and systemic
Two types of anaplastic lymphomas are distinguished:

Primary cutaneous anaplastic lymphoma, which affects only the skin (25% of cutaneous T-lymphomas);
Systemic primitive anaplastic lymphoma that affects the lymph nodes and several organs.
Primitive cutaneous anaplastic lymphoma
The primary cutaneous anaplastic lymphoma is a lymphoma that originates at the skin level (dermis and subcutaneous cell tissue) and never comes out. In only 10% of cases, it can extend to nearby lymph nodes.

This results in a cutaneous tumor visible under the skin (in 80% of cases it is a single or localized attack). Sometimes ulcerated (60% of cases), it can take the form:

of nodules (30%)
Rarely have papules (7%);
of plates (3%).
The primary cutaneous anaplastic lymphoma is less aggressive than the systemic and therefore it is a good prognosis. Generally the 5-year survival rate exceeds 90% (even if lymph nodes are reached).

Good to know: Some people with primitive cutaneous anaplastic lymphoma can heal spontaneously.

Systemic primitive anaplastic lymphoma
Systemic primitive anaplastic lymphoma first develops at the level of the lymph nodes. It will then propagate in other organs and tissues among which we find:

The liver
Bone marrow;
the lungs;
The bones;
the skin;
the digestive tract;
Soft tissue.
Very aggressive, it evolves rapidly and its diagnosis is usually relatively late (lymphoma has already reached a stage III or IV when it intervenes).

Anaplastic lymphoma Treatment
Patients with positive ALK lymphoma respond better to treatment and chemotherapy. So their prognosis is favorable. Conversely, patients with negative ALK anaplastic lymphoma are less likely to have a better prognosis and the risk of relapse is higher. This is why they undergo more intensive chemotherapy and an autograft can be envisaged.

Nevertheless, anaplastic lymphomas are generally quite sensitive to chemotherapy, which makes it possible to achieve good results. It can be followed by radiation therapy, including:

If the primary cutaneous anaplastic lymphoma is localized;
If systemic primitive anaplastic lymphoma is stage I and therefore affects only one ganglionic area.

Note: If you perform a surgical procedure that allows you to remove the entire tumor, you can do without other treatments.-