Disease of Hodgkin

The disease of Hodgkin or lymphoma of Hodgkin is a type of Lymphome characterized by the presence of large atypical cells, the Cellules of Reed-Sternberg. The cell of STERNBERG is essential to the diagnosis, but attention it is not completely specific and is found sometimes in other types of lymphomas. Its real nature is still little known but it would seem that it is of lymphoid origin B clonale.

Synonyms

disease of Hodgkin-Paltauf-Sternberg ,
Hodgkin (very short),
lymphoma hodgkinien ,
granulomatose malignant ,
malignant lymphogranulomatosis ,
cancer of the ganglia (to some extent one can speak about cancer).

History

It was described for the first time Thomas Hodgkin in 1832. A half century later, Wilms proposes name disease of Hodgkin . Its mechanism was discussed a long time of it: inflammatory, infectious or tumoral.

The first attempt at chemotherapy goes back to 1947 with a derivative of the Mustard gas with a very relative success. The forecast was appalling since the lifespan did not exceed two years after the diagnosis. Very significant progress was reached with the chemotherapy of the type MOPP in 1964 (mechlorethamine, vincristine, procarbazine and prednisone) then of type ABVD (doxorubicine, bléomycine, vinblastine and dacarbazine) in the middle of the years 1970.

Epidemiology

Contrary to the other lymphomas of which the frequency augmente with the age, the lymphomas hodgkiniens have a bimodal frequency curve: indeed, it more frequently intervenes within two distinct groups of age, the first group of age being that of the young adults between 20 and 30 years et the second towards 70 ans. This disease more frequently touches the men, except in the case of the nodular Sclérose, an alternative, which more frequently touches the women. According to a recent study of the medical check Lancet , its incidence, of approximately 4 cases on 100 000, would increase in the young adults. This disease represents however less than 1% of the totality of known cancers.

Etiology

The researchers do not have any certainty as for the origin of this disease but the Virus of Epstein-Barr - responsible for the Mononucléose - seems to play a part in certain forms of the disease of Hodgkin like showed it recently a Danish team: in nearly 50% of the cases, cells of Reed-Sterberg, “typical” of this cancer (recall: sometimes they find in lymphomas not hodgkinien), are infected by the virus.

Symptoms

lymphatic ganglia inflated (Adenopathy S) but painless (often localized in the neck, or the hollows axillaires) are the most frequent sign of the disease of Hodgkin. The thoracic ganglion S are them also frequently touched, as can show it a Radiographie chest.

Approximately a third of the patients meets also systemic symptoms (slimming, feeling of Fatigue, Fièvre, Sueur S night abundant, sometimes pains ganglionic at the time of ingestion of alcohol or Prurit insulated) which also make it possible them to direct the diagnosis.

Diagnosis

the disease of Hodgkin must be distinguished well from the other noncancerous causes of swelling of the ganglia and other types of cancers. The final diagnosis is the Biopsie (ablation of a ganglion for a pathological examination).

The doctor must first of all make sure that the Adénopathie S do not result from a not-cancerous infection or another type of cancer. The final diagnosis will be posed after the examination by Biopsie of the one of the ganglia, examination which will reveal the presence of large malignant (known as of Reed-Sternberg), binuclées cells, with prominent nucleoles and a immuniphénotype CD15+, CD25+, CD30+ not very common. One also finds in the taking away a cellular unit composed of lymphocytes, histiocytes and the eosinophilic ones. cells of Reed-Sternberg , mixed with a reaction (???) cellular made up of lymphocytes, histiocytes, eosinophilic, and cells of plasma. The cells of Reed-Sternberg are known in the shape of large cells often binuclées with prominent nucleoles and a immunophénotype CD15+, CD25+, CD30+ not very common. In about 50% of the cases, the cells of Reed-Sternberg are infected by the Virus of Epstein-Barr. -->

The disease of Hodgkin can be classified in four great histological groups:

with lymphocytary prevalence (< 10%),
with scléro-nodular form (most common), composed of large tumors with lacunar cells of RS concealments surrounded by fibrituc bands
with mixed cellularity,
with lymphocytary dépletion (reclassified recently with the lymphomas anaplasic).

For as much histological classification is not as determining as in the lymphomas non-hodgkiniens: the treatment and the forecast of the disease of Hodgkin depend indeed more of the stage on the disease that of sound histotype .

Assessment of extension

The diagnosis posed with certainty, the hematologist must determine the stage of the disease and the touched body surfaces. A radio and a scanner of the zones thoracic and pelvic are carried out, supplemented by a pushed analysis of Sang and a biopsy ostéomédullaire. According to the results, the patient will be classified (the classification of Ann Arbor being most widespread) in one of the four stages:

Stage I: invasion of a ganglion or a ganglionic group.
Stage II: two ganglionic groups - but on only one side of the diaphragm - are touched.
Stage III: ganglia reaches diaphragm on both sides.
Stage IV: attack of bodies extralymphatic: internal organs, Liver, osseous marrow.

Types The disease of Hodgkin (also known under the name of lymphoma hodgkinien) can be cut out in four pathological sub-types based on the morphology of the cells of Reed-Sternberg and on the composition of the concealment infiltrate reactivates detected thanks to the biopsy of a ganglion. There are clear but tiny differences in the forecasts of the varied forms of the disease.

1. The sub-type of the nodular sclerosis is most current and is composed of large tumors with lacunar RS concealments surrounded by fibrotic bands .

2. Prevalent lymphocyte subtype is year uncommon subtype and is composed off vague nodules with numerous reactivates lymphocytes. Unlike the other forms off Hodgkin lymphoma, the non-classic RS concealments are CD15 negative and CD30 while positive for the B concealment marker CD20.

3. Lymphocyte depleted subtype is also year uncommon subtype and is composed off numerous classic RS concealments with only few reactivates lymphocytes.

4. Mixed-cellularity subtype has common subtype and is composed off numerous classic RS concealments admixed with numerous inflammatory concealments including lymphocytes, histiocytes, eosinophils, and plasma concealments. -->

In addition, the absence of general signs (night fever, slimming, sweats) is marked has, while the presence of one or more signs classifies the disease out of B Formerly., the patients concerning the group B were of forecast unfavourable. Thanks to the new treatments, the predictive character of these two groups seems little by little abandoned. Lastly, an international study identified in 1996 seven big factors of forecast on patients having a sick zone which extends or being at a advanced stage of the disease. A patient not having any of these factors to a chance of survival at 5 years of 84%. The presence of each factor decreasing the percentage, the chance of survival at 5 years falls to 72% for the patients having 5 from these factors. Which are:

Age ≥ 45 years,
Stage IV of the disease,
Hemoglobin < 10,5 mg/dl,
Many lymphocytes < 600/μl or < 8%,
Male sex,
Albumin < 4,0 mg/dl,
Many white globules ≥ 15 000/μl.

Stages

After a disease of Hodgkin was diagnosed, one will determine the stage of advance of the disease: the patient will undergo series of tests and procedures which will help to determine which parts of the body are touched. These procedures always comprise the histology , a physical examination, blood tests, radio of the chest, CT scan S or IRM (imagery with magnetic resonance) of the chest, abdomen and basin, Mtoe scan (tomodensitometry of emission by positrons) and a puncture of osseous marrow. Certain patients will need a biopsy of the liver, Lymphangiogramme S, Gallium scan S, osseous Scan, and surgical laparotomy, operation which implies an opening of the rib cage and a visual inspection of the tumors. The surgical laparotomy is at present especially of historical importance.

On the basis of this cutting, the patient will be categorized according to a certain classification (the Classification of Ann Arbor in is a widespread example):

stage I is the implication of a zone with only one ganglion (I) or only one extralymphatic area (IE);
stage II is the implication of several ganglionic zones located on the same side of the diaphragm (II) or of another ganglionic area, and with a contiguous extralymphatic zone (IIe);
stage III is the implication of a ganglionic area located on the two sides of the diaphragm, which can include the spleen (IIIs) and/or be limited to an area or a contiguous body extralymphatic (IIIe, IIIes);
stage IV is the infection of one or more bodies extralymphatic.

The absence of systemic symptoms is notified while adding “has” in the name of the stage; the presence of systemic symptoms is it notified by adding a “B”.

In 1996, an international study identified 7 factors which make it possible to envisage with precision the rate of success of a traditional treatment on the patients having a sick zone which extends or being at a advanced stage of the disease. The period of 5 years remission is directly correlated with the number of factors which the patient presented. This 5 years period accounts for 84% of rate of success for the patients not presenting any factor. Each factor lowers the rate of success by 7%, so much so that this rate falls to 72% for patients presenting at least 5 factors.

The factors of forecast identified by this international study are:

Age ³ 45 years
Stage IV of the disease
Hemoglobin < 10,5 mg/dl
Many lymphocytes < 600/µl or < 8%
Male sex
Albumin < 4,0 mg/dl
Many white globules ≥ ³ 15 000/µl

Treatment

According to the extension, the hematologist will decide on a anti-cancer Chimiothérapie (association of molecules ABVD (Adriamycine, Bléomycine, Vinblastine and Dacarbazine) or, more rarely now, MOPP), or BEACOPP (experimental) and/or of a Radiothérapie, according to the stage of advance of the disease. With an adapted treatment, 90% of the patients cure. In the event of initial therapeutic failure, it is not rare that the patients cure nevertheless after a second treatment (IVA75 + Autogreffe of osseous marrow…), even a third (Transplantation of marrow).

Currently, chemotherapy ABVD (for the 4 substances used: Adriamycine, Bleomycine, Vinblastine, and Dacarbazine) is the treatment more adapted for the disease of Hodgkin. Developed in Italy in the years 1970, treatment ABVD takes in general between 6 and 8 months, even if certain times of the longer treatments are necessary.

With an adapted treatment, more than 90% of the diseases of Hodgkin are curable. -->

See too

Hematology

External bonds

lymphoma on the site of the Group of Study of the Lymphoma of the Adult
France Lymphoma Hope: The association of patients of the lymphoma
www.leucemie-unle.org
Hodgkin' S Lymphoma Home Page on the site of the American national institute on cancer.
Historical of the disease of Hodgkin

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