hodgkin's lymphoma

What does a Hodgkin's lymphoma?

Hodgkin's lymphoma is characterized by the proliferation of abnormal cells developed from a B lymphocyte, identified as Reed-Sternberg cells. The tumor cells, unlike other cancers tend to be few in number, and the environment is very polymorphic and inflammatory. The LH translates into an increase in volume of the lymph nodes. It evolves slowly, usually over several months.

He most often manifests as the Lymphadenopathy neck, visible and palpable, generally painful, sometimes associated with General signs such as fever, night sweats, weight loss, asthenia, pruritus. He trained rarely symptomatic compression of neighbouring bodies responsible for respiratory discomfort or a superior cellar syndrome.

All superficial and deep lymph node sites can be affected, in particular the mediastinum. Other bodies, such as the spleen (considered a lymphoid organ), but also by decreasing frequency lung, bone, bone marrow, liver, kidneys are sometimes achieved.

We are 4 stages of development from 1 to 4 depending on whether the disease is spread on an or 2 sides of the diaphragm, and a non-lymphoid organ is affected or not.

Surgical ganglionic biopsy or needle, under local or general anesthesia is necessary for diagnosis.

Tomography in show position, coupled with a scanner (PET-scan) is now reviewing key to the assessment of the extent of disease at diagnosis and response to treatment. After a first phase of only Visual analysis of fasteners, the score of Deauville, in 5 points, on Visual and quantitative data are now used.

The support of the Institut Curie Hodgkin Lymphoma
For children, adolescents and young adults (AJA), treatment protocols have been developed at European level. They are essentially based on chemotherapy. The duration of the unit depends on the tumor volume (< or > 200 ml), the stage of extension of the disease, the existence of an inflammatory syndrome and the general impact. It also depends on the response of disease treatment, evaluated on the volumetric by a scanner plan and the metabolic plan by a pet scan.

Radiotherapy on the initially invaded lymph is now used for a quarter of the patients about.

(Between 2 and 6 cycles of chemotherapy) treatments are administered through a central catheter to implantable, most often in day hospital. The drugs used are anthracyclines, alkylating to low-dose, Vincristine, Etoposide and a corticosteroid.

The survival of children and adolescents currently treated for Hodgkin lymphoma is over 90%, all stages combined.

In case of early relapse or refractory disease, chemotherapy of 2nd line, often associated with autologous peripheral stem cell transplant is available. Recently, the first therapies targeted - in combination with chemotherapy or alone - have emerged. They should help to further improve the prognosis of this cancer.

Translational and basic research on Hodgkin's lymphoma

Targeted treatments

Targeted therapies are now part of the treatment of adult patients not responding to conventional treatment.

Brentuximab - Vetodin (BV) is an antibody (a complex protein used by the immune system to detect and neutralize pathogens) is administered coupled with a molecule the auristatine. After a test phase 2 which concluded that very good answers and acceptable toxicity (peripheral neuropathy mainly - disorders that affect the peripheral nerves), the molecule has obtained approval for placing on the market in some adult patients whom conventional treatments have no results.

The BV is also indicated in patients with unfavorable relapse and preventive treatment after intensive chemotherapy. In adults, the association of the BV to conventional chemotherapy (ABVD) was also tested in phase 1, but resulted in an unacceptable lung toxicity. Another trial with a molecule combined, containing also the Brentuximab-Vetodin, is ongoing in patients with at least one risk factor.

Other targeted treatments have been tested. Rituximab is effective in patients with a certain type of rare tumor (15% of cases). Other treatments, such as everolimus (an inhibitor of mTOR), was recently tested in patients in relapse and has presented good results.

The inhibitors 'immune checkpoint. "

The Nivolumab and the Pembrolizumab are antibodies tested in patients in relapse after several different treatments. These drugs is administered on an outpatient basis, with few side effects, and can be given over a long period.