Blood Cancers: lymphomas
Sharing nearly half of the blood cancers are lymphomas, cancer of the lymphatic system, with about 80% non-Hodgkin's lymphoma (NHL) and 20% Hodgkin lymphoma (LH).
Lymphomas are characterized by the excessive proliferation of lymphocytes (B most often or T) at the level of the lymph nodes, liver, spleen, more rarely other organs. They cause an increase in their size. The main symptoms therefore consist of an increase in the size of the lymph nodes.
The diagnosis is based on the biopsy analysis of the affected ganglia. Imaging tests – Scanners and PET scanners – will be conducted to assess the spread of the disease in the body.
Since lymphocytes are blood cells, they can develop in the whole body: unlike solid tumors, the notion of metastasis does not exist in this kind of cancer.
The therapeutic decisions are then taken at the multidisciplinary consultation meetings (RCP) bringing together all the specialists concerned.
Hodgkin's lymphoma
With 1880 people diagnosed in 2012, Hodgkin's lymphoma (LH) is a relatively rare cancer. It is also one of the most well-treated cancers. It develops according to 2 peaks of frequency: In young adults, between 20 and 35 years, or after 70 years.
The treatment of LH depends on the stage at which it was diagnosed and its subtype. It is most often based on a combination of chemotherapy (to treat the organism as a whole) and radiotherapy (to treat lymph nodes locally).
In the event of relapse, another chemotherapy, often associated with a bone marrow transplant, may be proposed. Recently, the first targeted therapies – in combination with chemotherapy or alone – have appeared. They should make it possible to further improve the prognosis of this cancer.
Non-Hodgkin's lymphoma
With more than 11 000 new cases diagnosed in France each year, non-Hodgkin lymphomas (NHL) are the 5th cancer in terms of frequency. They are observed at any age, including children and adolescents, but their frequency increases after 65 years.
Other Forms
Follicular lymphoma, diffuse large cell lymphoma B, marginal zone lymphoma, mantle lymphoma, macroglobulinemia disease, lymphoblastic lymphoma, Burkitt lymphoma.... There are many forms of NHL. The precise diagnosis of the NHL can only be established from the analysis of a ganglionic sampling.
Some NHL are very slow-changing, sometimes defined as indolent. These include follicular lymphoma, lymphocytic lymphoma (LL), chronic lymphoid leukemia (LLC), marginal zone lymphomas, Lymphoplasmocytaire or waldenstrom lymphoma of macroglobulinemia, cutaneous T-cell lymphoma or B.
Mantle cell lymphomas, large B-cell diffuse lymphomas, Burkitt lymphomas are generally more aggressive.
The prognosis and treatment of NHL varies depending on the subtypes, the grade of the disease, the age of the patient and the extension (number of nodes affected) of the disease. Some forms indolent are monitored and will only receive treatment if an evolution of their disease is found.
The treatment is based on the association of several chemotherapy: for a long time this polychemotherapy was composed of cyclophosphamide, doxorubicin, vincristine and prednisone, a protocol called CHOP. The addition of targeted therapy, rituximab, as part of the R-CHOP protocol, has increased the healing rate, lengthened the time before possible relapses and the survival of NHL patients, particularly in the most aggressive forms . Since other targeted therapies have been born and should still improve the prognosis of the NHL.
In rare cases, the NHL develops exclusively in the central nervous system which includes the brain, the eye, the brains and the spinal cord. It is referred to as primitive cerebral lymphoma (LCP) or primitive intra-ocular lymphoma (piols). In order to improve the management of patients with these forms of lymphoma, a network of French expert centres was established in 2011. It revolves around a national expert center in Ile-de-France, on two sites, the hospital of pity-Salpêtrière in Paris and the hospital René Hayward-Institut Curie in Saint Cloud.
Blood Cancers: MULTIPLE Myeloma
Share nearly 5000 people were diagnosed in France in 2012 for multiple Myeloma (MM). In more than 50% of cases, the disease develops in people over 70 years of age.
Multiple myeloma develops to the detriment of a particular type of white blood cells, the plasma. These cells correspond to the final stage of specialization of B lymphocytes. Their main property is to produce antibodies – also called immunoglobulins –, molecules capable of organizing an immune response targeted against an intruder (antigen).
When a plasmocyte becomes tumoral, it multiplies in excess, produces, in large quantities, a single type of immunoglobulin, the "monoclonal peak " and causes deregulation of other plasma with restriction of the synthesis of normal antibodies. One of the consequences is the decrease in the effectiveness of the immune response to an infection. Tumor plasma can also invade the bone marrow and reduce the formation of other blood and immune cells.
All these deregulations cause symptoms – fatigue, anemia, renal failure – revealing the disease. The most specific symptoms of MM remain, however, pain and fragility at the bone level. They result from the destruction of the bone by tumor plasma.
These symptoms will lead to the prescription of a blood test in search of a monoclonal peak suggestive of the existence of abnormal plasma. However it is the analysis of the composition of the bone marrow – the Myelogram – following a puncture that will establish the definitive diagnosis of MM.
In patients whose disease is detected early or have no symptoms, a simple surveillance is sometimes offered. For others, treatment is mainly based on various chemotherapy-based associations: most often melphalan (the intensity of which varies depending on the age of the patient) and more or less targeted therapies: immunomodulator ( Thalidomide or derivatives) or bortezomib (or derivatives: Kyprolis, Ixazomib).
Today, new targeted or immunotherapeutics therapies are being evaluated, particularly in the case of relapse. Among these new therapies are molecules that act on the immune system (monoclonal antibodies: Daratumomab, Eculizumab).
For the majority of patients, there is currently a very important lengthening of survival. New therapies under evaluation should further improve the management of patients with multiple myeloma.
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