follicular b cell lymphoma | Follicular lymphoma

Follicular lymphoma

 Follicular lymphoma
It's a low-GRADE lymphoma
How is the disease?

With about a quarter of the lymphomas diagnosed in France, this is the second most common form of lymphoma. It affects adults, rarely before 45 years, most often after 60 years.

The majority of patients present, on examination, superficial lymph nodes affecting most ganglion areas.

These lymphomas are disseminated from the outset but are of low malignancy. They are characterized by a slow evolution marked by phases of stagnation, even spontaneous involution and few general signs.

The characteristics phenotypes are a clonal proliferation of B cells of the germ center (CD5-, CD20 +, and CD10 +), of the lymph node follicle. Phenotypes are defined as: the presence of surface antigens associated with B + cells: CD19 +, CD20 +, CD22 +, CD24 +, CD79a +, CD9 +
A positive surface immunoglobulin; BCL-2 +; BCL-6 + CD5-, CD43-, CD23-/+, CD11c-; HLA-DR +, CD35 +, CD21 +
Positive adhesion molecules

Cytogenetics the early chromosomal anomaly, found in more than 70% of patients, is a translocation t (14; 18) (Q32; 21) on the Karyotype and a Bcl2/IgH rearrangement in molecular biology.
This rearrangement resulted in a constitutive stimulation of the Bcl2, causing the blockade of apoptosis and the accumulation of lymphocytes in the lymph nodes.
The expression of the marker of proliferation, Ki67 is rarely found, at the onset of the disease.

The different grades according to the WHO classification, follicular lymphoma is "graded" based on the proportion of centroblasts *, correlated with clinical evolution. Three grades are distinguished: Grade 1, where small cells predominate
Grade 2, mixed, with small and large cells
Grade 3 where large cells predominate (follicular lymphoma with large cells), the latter corresponding to Centroblasts *. This type of NHL is disseminated at blood level in 10 to 20% of cases

In situ follicular lymphoma (follicular neoplasia in situ)

It's lymphoma that has a low risk of progression to lymphoma. However, it may be associated with previous or synchronous follicular lymphoma, * centroblasts: B lymphocytes being proliferated it is a chronic disease

The evolution is slow (form called indolent) it is done over several years, with a well preserved general state. Thus, some patients may very well present fluctuating adenopathies for 5 years, without requiring any special treatment. On the other hand, more rarely, some patients have a more progressive disease. The interval between the onset of lymph nodes and the diagnosis can give an idea of the scalability of the disease. 

The transformation to high-grade large cell lymphoma is quite common after a few years. 

Recent work has shown that changes in the tumor micro-environment could help to refine the prognosis and decisions regarding the management of the disease, that is, treatment or active surveillance. 

The prognosis it is linked to the tumor mass and is specified thanks to the use of the FLIPI score (see box). 

The 5-year survival rate of follicular lymphoma increased from 66% to 84% in 2015.

His treatment...

Treatment options range from simple, untreated monitoring to intensive treatment with stem cell grafting. Schematically, specialists consider that: low-tumor-mass lymphomas deserve simple surveillance

Tumor-intensive lymphomas should be treated by an association comprising a monoclonal antibody (Rituximab (MabThéra ™) and chop (R-chop) chemotherapy

The evolution is usually characterized by a good response to the treatments but also by frequent relapses.