Non Hodgkin Lymphoma
fLes lymphomas not hodgkiniens are some malignancies developed from lymphoid tissues.
Lymphoma is a cancer of the lymphatic system at the expense of lymphocytes.
The lymphatic system includes the bone marrow, spleen, thymus, lymph nodes and lymphatic vessels; It ensures the defence of the body against microbes, parasites, toxins, foreign bodies, etc.
Form of lymphoma and classifications
There are malignant lymphomas 'ganglion' and 'extra-ganglionnaires '.
Histology
Aggressive malignant lymphomas (lymphomas ganglion or extra-ganglionnaires with an aggressive presentation) Phenotype B
Lymphoma large cell:
Lymphoma centroblastique
Lymphoma immunoblastique
B-cell lymphoma rich in T cells
B-cell lymphoma rich in histiocytes
Lymphoma large cell anaplastic B
Lymphoma Burkitt-like
lymphomatoide Granulomatosis
Lymphoma associated with a pyothorax
primitive B Lymphoma of the mediastinum (thymic type)
intravascular Lymphoma
Lymphoma of the PEL
Burkitt Lymphoma
Lymphoma of the mantle
Classic shape
with round cells
form blast
with large cells
Phenotype T
anaplastic large cell lymphoma
Peripheral T Lymphoma
Angio-immunoblastique
Lymphoma of T areas without precision
Lymphomas not indolent hodgkiniens (lymphomas ganglion or extra-ganglionnaires having an indolent presentation)
Cells of the marginal zone lymphomas
mucosa-associated lymphoid tissue Lymphoma
splenic (of the spleen) Lymphoma with or without villeux lymphocytes
lymph node Lymphoma with or without B monocytoides cells
disseminated forms
Follicular Lymphoma
grade 1 (< 50% of centroblasts) grade 2 (> 50% of centroblasts)
skin
digestive
Lymphoplasmocytoides (or immunocytomes) lymphomas
Classification of Ann Arbor
Stage I: Achievement of a single ganglion group or of a single body.
Stage II: Achieving more a ganglionic area on the same side of the diaphragm. (lower or upper part of the body)
Stage III: Multiple Lymphadenopathy in both sides of the diaphragm. (lower and upper part of the body)
Stage IV: Infringement broadcasts one or several organs and bone marrow.
To which is added:
E in stages I, II or III: If of a contiguous visceral breach
A: If no sign of B
B:-unexplained weight loss more of 10% of the weight of the body in less than 6 months (or 5% in 1 month) or fever unexplained > 38 ° C more of 8 days or profuse night sweats.
Classification of the general State of the patient (who)
0: there is no breach of the general State of health, the patient to lead a normal life.
1: There is a wariness, but the patient can go about their daily lives and work.
2: A strong tiredness requires the patient to lie down in the day, but less than half of the day. He can no longer work, but is still able to take care of himself.
3: The patient remains bed-ridden or sitting more than half of the day. The patient is dependent for certain acts of daily life: household, races, etc.
4: The patient can get up and cannot take care of himself. Need for assistance for all acts of everyday life.
Classification according to their degree of malignancy
Lymphomas not indolent hodgkiniens (low malignancy)
Follicular Lymphoma
Lymphocytic Lymphoma (LL) (ganglionic location of chronic lymphoid leukemia LLC)
The marginal zone Lymphoma (and MALT Lymphoma)
Macroglobulinemia of Waldenstrom (also called lymphoplasmocytaire or immunocytome Lymphoma)
Cutaneous Lymphoma T
Lymphomas not aggressive hodgkiniens (high malignancy)
The most common large B cell diffuse Lymphoma of lymphomas (it represents 30-40% of non-hodgkiniens Lymphoma). The average age of the patients is 55 to 60, but it can affect children. These aggressive lymphomas can take birth in the lymph nodes or extra-ganglionnaires locations such as the digestive, testes, thyroid, skin, breast, central nervous system or bones.
Lymphomas in cells of the lymphomas coat in B cells, the mantle cell lymphomas represent 5-10% of cases of lymphomas not hodgkiniens and most often affect men more of 50 years and the disease is often advanced at the time of the diag nosis. Patients generally present multiple ganglion damage. One or several organs (particularly the gut) and bone marrow may also be affected. This lymphoma is often aggressive. However, new therapies showed an efficacy on the disease.
Burkitt Lymphoma, lymphoma 'type Burkitt' (small cell non traditional) Lymphoma malignancy high B-cell, it concerns adults and children. It is one of the lymphomas diagnosed in patients infected with HIV. All forms of Burkitt lymphomas are most often men, they behave in the same way and are all spontaneously very aggressive. However, the prognosis after treatment is generally favourable.
Peripheral T lymphomas they represent 10-15% of lymphomas not hodgkiniens in adults. The term peripheral T lymphoma is based on the fact that it comes to tumors composed of cells T (and not B) and that these cells are mature. Most peripheral T lymphomas have a malignancy high with the exception of the mycosis fungoides.
Other lymphomas
HIV-associated lymphomas estimated that almost 10% of HIV-positive patients develop lymphoma. It is usually of higher malignancy.
Primitive Lymphoma of the central nervous system Cancer that affects only the brain and the spinal cord. Apart from HIV infection, it mainly affects people in more than 60 years.
Lymphomas of the child they represent about 5 percent of non-hodgkiniens lymphoma.
Etiology
Usually indefinite
Risk factors:
HIV
Virus Epstein-Barr and Burkitt Lymphoma
HTLV-1 (Caribbean and Japan)
Autoimmune disease
Immunosuppression
Viral chronic hepatitis C1
A francaise2 study has highlighted, at the molecular level, a relationship between occupational exposure to pesticides and the acquisition of a chromosomal abnormality known as one of the initial steps in the development of lymphomas not hodgkiniens. Radiation or radioactivity exposure is one of the triggering factors.
Symptomatology
In addition to impairment of the general condition, we find a low-grade fever (38 ° C), weight loss, night sweats (++), etc.
Ganglion forms
Presence of superficial Lymphadenopathy palpable and deep visible in prints.
Extra-ganglionnaires forms
There is no specific symptom, but target the expression of invasion of the body: the stomach, thyroid, brain, testis, the parotid, the eye, the bone marrow.
Prognostic index
International prognostic index (IPI) large cell lymphomas
According to the classification of Ann Arbor
According to the histology of the malignant lymphoma
Based on rates of LDH (lactate dehydrogenase)
According to the age of the patient (more or less 60 years old)
According to its general condition (0, 1, 2, 3, 4)
According to the number of locations extra-ganglionnaires (0 or 1 versus > 1)
International prognostic index in follicular lymphomas (FLIPI)
According to the classification of Ann Arbor
According to the histology of the malignant lymphoma
According to the rate of lactate dehydrogenase
According to the age of the patient (more or less 60 years old)
According to hemoglobin
Diagnosis
Laboratory tests of blood
Bone marrow biopsy
Ganglionic biopsy
CT scan (scan) of the chest and abdomen
Positron emission tomography
Which are sometimes associated:
Lumbar puncture
Gastric or colon Fibroscopy
Bone scan
Immunophenotyping of lymphocytes
Treatment
Radiotherapy
Chemotherapy
Immunotherapy:
Treatment method to change the natural defense means of the body, either by injection of serum or immunoglobulin that brings specific antibodies (passive immunotherapy), or vaccinotherapie which raises the production of these antibodies (active immunotherapy).
Monoclonal antibodies: Rituximab (MabThera)
Interferon
Gene therapy
Anti-angiogenic therapy
Radio-immunotherapy
Vaccines
Proteasome inhibitors
Antisense molecules
Surgery
Splenectomy
Allograft
Autograft
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