Hodgkin's disease or Hodgkin's lymphoma is a type of lymphoma is characterized by the presence of large atypical cells, the cells of Reed-Sternberg. The Sternberg cell is essential for diagnosis, but not completely specific and sometimes found in other types of lymphoma. His real nature is still not known but it appears whether clonal B lymphoid origin.
* Hodgkin's disease-Paltauf-Sternberg,
* Hodgkin's lymphoma,
* Granulomatose malignant,
* Lymphogranulomatose malignant,
* Cancer of the lymph nodes.
It was first described by Thomas Hodgkin in 1832 . Half a century later, Wilms proposes the name Hodgkin's disease. Its mechanism has been discussed for a long time: inflammatory, or infectious disease.
The first attempt chemotherapy date of 1947 with a derivative of mustard gas with a very relative success. The prognosis was terrible because life did not exceed two years after diagnosis. Some progress has been achieved with chemotherapy type MOPP in 1964 (mechlorethamine, vincristine, prednisone and procarbazine) and then type ABVD (doxorubicin, bleomycin, vinblastine and dacarbazine) in the mid-1970.
Unlike other lymphomas whose frequency increases with age, Hodgkin's lymphoma have a frequency curve bimodal: indeed, it occurs more frequently within two age groups, the first age group being the young adults between 20 and 30 years and the second to 70 years. The disease affects men more frequently, except in the case of nodular sclerosis, a variant, which affects women more frequently. According to a recent study by the medical journal Lancet, its incidence, approximately 4 out of 100 000, increase in young adults. This disease represents less than 1% of all cancers known.
Researchers have no certainty as to the origin of this disease, but the Epstein-Barr virus - responsible for mononucleosis - seems to play a role in some forms of Hodgkin's disease as demonstrated recently a team Danish: nearly 50% of cases, cells Reed-Sternberg, "typical" of this cancer (reminder: they sometimes find themselves in non-Hodgkin lymphoma), are infected with the virus.
The swollen lymph nodes (adenopathies) but painless (often located in the neck, or in the axillae) are the most frequent sign of Hodgkin's disease. The thoracic ganglia are also frequently affected, as can be demonstrated in a chest X-ray. There is also the presence of an enlarged spleen.
Approximately one third of patients also faced systemic symptoms (weight loss, feeling of tiredness, fever, night sweats abundant, sometimes pain lymph nodes during ingestion of alcohol or isolated pruritus), which allow them also to guide the diagnosis.
Hodgkin's disease must be clearly distinguished from other causes non-cancerous swelling of the lymph nodes and other types of cancers. The definitive diagnosis is a biopsy (removal of a lymph node for a pathological examination).
The doctor must first ensure that adenopathies not the result of an infection non-cancerous or another type of cancer. The definitive diagnosis will be established once the review by biopsy of a lymph node, examination reveal the presence of large malignant cells (so-called Reed-Sternberg [RS]), binuclées with prominent nucleoli and a immunophénotype + CD15, CD25 + , CD30 + unusual. There is also a levy on the whole cell composed of lymphocytes, histiocytes and eosinophils.
The microscopic examination of the lymph node removed reveals a dispersion of large malignant cells, known as cells Reed-Sternberg, mixed with lymphocytes, histiocytes, eosinophils, and other plasma cells. The cells of Reed-Sternberg are known in the form of large cells often binuclées with prominent nucleoli and a immunophénotype + CD15, CD25 +, CD30 + unusual. In roughly 50% of cases, cells Reed-Sternberg are infected with Epstein-Barr virus.
Hodgkin's disease can be classified into four main types histological. For all the histological classification is not as crucial as in non-Hodgkin's lymphoma: treatment and prognosis of Hodgkin's disease depend more on the stage of the disease as its histotype.
The diagnosis with certainty, the hematologist must determine the stage of the disease and the affected areas and equipment. A radio and a scanner of chest and pelvic areas are made, supplemented by a blood test and a biopsy thrust ostéomédullaire. According to the results, the patient will be classified (the classification of Ann Arbor is the most widespread) in one of four stages.
Hodgkin's disease (also known as Hodgkin's lymphoma) can be divided into four sub-types pathological based on the morphology of cells Reed-Sternberg and the composition of the reactive cell infiltrate detected through biopsy of ganglion. There are clear differences but minimal in the predictions varied forms of the disease.
The histological classification established by Rye in 1965 to sort Hodgkin's disease classic four types:
* 1. type 1 is rich in lymphocytes or predominantly lymphocytic (PL);
* 2. type 2 scléro-nodular (SN) associated with the presence of sclerosis;
* 3. type 3 in mixed cellularity (CM);
* 4. type 4 to lymphocytic depletion (DL).
In addition, the absence of general signs (fever, weight loss, night sweats) is marked A, while the presence of one or more signs the disease in class B. In the past, patients in Group B were unfavorable prognosis. Thanks to new treatments, the predictive nature of these two groups seems gradually abandoned. Finally, an international study in 1996 has identified seven factors important prognosis on patients with a zone that extends sick or well being at an advanced stage of the disease. One patient had none of these factors to be any chance of survival 5 years of 84%. The presence of each factor reducing the percentage chance of survival 5 years falls to 72% for patients with 5 of these factors. Which are:
* Age ≥ 45 years
* Stage IV of the disease,
* Hemoglobin <10.5 mg / dl,
* Number of lymphocytes <600 / l or μ <8%
* Albumin <4.0 mg / dl,
* Number of white blood cells ≥ 15 000 / μ l.
After a Hodgkin's disease was diagnosed, we will determine the level of progress of the disease: the patient will undergo a series of tests and procedures that will help determine which parts of the body are affected. These procedures include always histology, a physical examination, blood taken, radio chest, CT scans or MRI (magnetic resonance imaging) of the chest, abdomen and pelvis, a PET scan (CT ' positron emission) and a puncture bone marrow. Some patients will need a liver biopsy, lymphangiogrammes, gallium scans, bone scan, and laparotomy surgical operation that involves opening the chest and a visual inspection of tumors. The laparotomy surgery is now mainly of historical significance.
Based on this cut, the patient will be categorized as a certain classification (classification of Ann Arbor is a common example):
* The Stage I is the involvement of an area with a single node (I) or a single region extralymphatique (Ie);
* The Stage II is the involvement of several areas lymph node located on the same side of the diaphragm (II) or another lymph node region, and with a contiguous zone extralymphatique (II);
* Stage III is the involvement of a lymph node region located on both sides of the diaphragm, which may include the spleen (IIIs) and / or be limited to one region or body extralymphatique contiguous (III, IIIes);
* The stage IV is the infection of one or more organs extralymphatiques.
The lack of systemic symptoms is notified by adding an "A" on behalf of the stadium and the presence of systemic symptoms it is notified by adding a "B". The biological inflammatory syndromes that they will be rated by an 'a' if absent and a "b" if present. Finally, the flooding of a non-contiguous lymphatic is rated by an "E" (for stage I, II and III only), while the flooding of the rate will be more specifically noted by an "s".
In 1996, an international study has identified 7 factors that can accurately predict the success rate of traditional treatment on patients with an area stretching sick or are at an advanced stage of the disease. The period of remission of 5 years is directly correlated with the number of factors that showed the patient. This period of 5 years represents 84% success rate for patients showing no factor. Each factor lowers the success rate of 7%, so much so that the rate drops to 72% for patients with at least 5 factors.
The prognostic factors identified by this international study are:
* Age ³ 45 years
* Stage IV of the disease
* Hemoglobin <10.5 mg / dl
* Number of lymphocytes <600 / l or μ <8%
* Albumin <4.0 mg / dl
* Number of white blood cells ≥ ³ 15 000 / μ l
With proper treatment, over 90% of Hodgkin disease is curable.
According to the extension, a hematologist decide cancer chemotherapy (association of molecules ABVD (Adriamycine, Bleomycin, medicines Vinblastine and Dacarbazine) or, more rarely now, MOPP), or BEACOPP (experimental) and / or radiotherapy , According to the state of progress of the disease. With proper treatment, 90% of patients heal. In the case of initial treatment failure, it is not uncommon that patients recover, however after a second treatment (IVA75 + autologous bone marrow ...), even after a third (marrow transplantation).
Patients whose disease was detected early (stage I and II) are directly treated with radiotherapy. Patients whose disease was diagnosed later (stage III, IVA, or IVB) are processed through chemotherapy. Patients with an advanced stage with many nodules in the chest have a combined treatment: chemotherapy and radiotherapy.
Currently, chemotherapy ABVD (for 4 substances used: adriamycine, bleomycine, vinblastine, and dacarbazine) is the most appropriate treatment for Hodgkin's disease. Developed in Italy in the years 1970, the treatment ABVD usually takes between 6 and 8 months, although some times longer treatments are needed.
Hodgkin's disease is very radiosensible, ie it responds well to radiotherapy. It calls for external doses of radiation between 20 and 40 in Gray irradiation extended (eg mantelet; lumbo-aortic; low splenic).
But this exposes them to radiation side effects like thyroid disorders including hypothyroidism, stenosis of coronary arteries, acute leukemia and cancers of the breast side ... Thus, the attitude of choice is the association with chemotherapy called radio-chemotherapy.
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