Hodgkin’s disease or “Hodgkin’s lymphoma” is a blood cancer more common in young adults and after the age of 70.
Definition: what is Hodgkin’s disease?
Hodgkin’s lymphoma (HL) or “Hodgkin’s disease” is a form of cancer of the lymphatic system (blood cancer). THE English doctor Thomas Hodgkin had already described this disease in 1832 and was the first to identify this form of lymphoma. It is notably characterized by the presence of particular abnormal cells (THE Sternberg cells). This is what differentiates Hodgkin lymphoma from all other lymphomas which are given the generic name “non-Hodgkin lymphomas”. In Hodgkin’s disease, the proliferation of abnormal cells leads to increase in lymph node volume lymphatics. Presumably, the disease initially appears in a specific location, usually a lymph node. Locations outside the lymph nodes are rare. Some affected lymph nodes may be inside the thorax and therefore not accessible to palpation by the doctor. We distinguish 4 stages of development from 1 to 4 depending on whether the disease extends to one or both sides of the diaphragm, and whether or not a non-lymphoid organ is affected.
What are the symptoms of Hodgkin’s disease?
“The most common symptom of HL isenlarged lymph nodesnotably those located in the neckabove the collarbone, in the armpit, more rarely in the groin”, explains Dr Julien Lenglet, hematologist at Antony Private Hospital. Swollen lymph nodes are always painless. The lymph nodes inside the thorax can give cough or compression of the large vessels inside the thorax, giving a facial swelling : this is what we call a superior cava syndrome. Very often the diagnosis is made during a routine physical examination. “It’s important to keep in mind that most people who see their doctor because they have enlarged lymph nodes do not have Hodgkin’s lymphoma.”continues the doctor. Having swollen glands is often the result of an infection, however, in this case the glands in the neck are usually painful. The nodes of Hodgkin’s lymphoma are usually larger than 2 centimeters, hard, adherent to the deep plane.
Symptoms may also include:
- Recurrent episodes of fever, especially in the form affecting the elderly,
- Excessive sweating at night, soaking the sheets,
- Unintentional weight loss.
- A persistent fatigue and a lack of energy.
- Of the generalized itching all over the body.
What causes Hodgkin’s disease?
The causes of Hodgkin’s disease remain largely unknown. However, some research has shown that people who have already contracted the Epstein-Barr virus (responsible for infectious mononucleosis) appear to have a higher risk of developing this type of cancer. Heredity apparently plays no determining role. Hodgkin’s disease, like all other types of cancer, is not not contagious.
What are the stages?
There are 4 stages of Hodgkin’s disease. Stage I corresponds to the mildest form and stage IV to the most advanced form of the disease. Each stage is divided into (A) or (B), (A) meaning there are no general symptoms and (B) meaning there are general symptoms.
Stage I. The cancer is still confined within a single group of lymph nodes on one side of the thoracic diaphragm.
Stage II. The cancer has spread through the lymphatic system, remaining on only one side of the diaphragm.
Stage III. The cancer has spread into the lymphatic system, on both sides of the diaphragm.
Stage IV. The cancer has spread beyond the lymphatic system, into one or more organs (lung, bones, liver, bone marrow).
A biopsy to confirm the diagnosis of cancer.
“It’s about surgically remove a piece of lymph node (or the entire ganglion) in order to study it under the microscope. The biopsy is generally carried out under local anesthesia for superficial lymph nodes (neck, armpits, groin). In cases where the lymph node is deep, the biopsy can be guided by imaging using the scanner. explains the specialist.
Carried out by a specialist doctor called an “anatomopathologist”, the anatomopathological examination allows in particular toobserve Reed Sternberg cells characteristics of Hodgkin lymphoma. These are giant cells of specific appearance. Their environment which shows signs of inflammation can also be seen under a microscope. In addition, it is possible to properly characterize Reed Sternberg cells using antibodies that recognize molecules present on their membrane (CD30 expression is mandatory for diagnosis). In general, it is necessary to count about eight days between the biopsy and the definitive diagnosis. Certain simple examinations make it possible to guide the diagnosis such as acceleration of sedimentation rate or a increased C-reactive protein. Often carried out at the same time as the diagnosis, the extension assessment is essentially based on imaging examinations which make it possible to determine the number of sites affected by the disease. Different imaging techniques are used, including scanner and PET (positron emission tomography) which makes it possible to find all the locations of the lymphoma and to estimate the response to available treatments early.
Targeted therapies should improve the prognosis of this cancer.
What are the treatments for Hodgkin’s disease?
The treatment is based on the chemotherapy and the radiotherapyeven for early stages. It’s about doing rapidly reduce tumor massthen complete with radiotherapy on the residual tumor masses. Chemotherapy is therefore essential at all stages. For early stages the chemotherapy cycles are reduced (between 3 and 4) for more advanced stages they are more numerous (up to 6). Two chemotherapy protocols are used in the management of this lymphoma: the protocol Beacopp and the ABVD (a little less heavy). Likewise, radiotherapy doses vary depending on the stage. It is sometimes no longer carried out at the early stage by certain teams.
Furthermore, for several years now, other treatments have been used in adults, in patients with unsatisfactory responses to standard treatments for Hodgkin’s lymphoma. It is mainly a targeted treatment against Hodgkin lymphoma tumor cells and immunotherapies allowing the body’s immune defenses to be stimulated in order to fight through the patient’s own immunity. These two treatments have demonstrated their effectiveness in adult hematology and are now commonly used in the management of adult Hodgkin lymphoma. “For patients whose disease is not responsive to first treatments or returns after having been in remission, a new chemotherapy will be offered, combining high-dose chemotherapy, followed by a autologous stem cell transplant, says Dr Lenglet.
Prognosis: what is the life expectancy for Hodgkin’s disease?
The prognosis for Hodgkin’s disease is overall very good with overall survival, in children and adults under 20 years of age, of more than 90%.
Produced in collaboration with Dr Julien Lenglet, hematologist at Antony Private Hospital.