sporadic burkitt lymphoma | Sporadic Burkitt lymphoma

Sporadic Burkitt lymphoma

Main epidemiological characteristics
The clinical variant of Burkitt lymphoma has no specific geographic or climatic distribution. It accounts for about 1-2% of adult lymphomas and up to 40% of the child's lymphomas in the United States and Europe (Ferry J. A)

A particular variety is represented by the occurrence in the patient immunocompromised by the AIDS virus, where, unlike other AIDS-related cancers, Burkitt's lymphoma occurs in subjects with CD4 cell counts greater than 200 cells/μ L.

Clinical presentation
Adult patients usually have extra-ganglionic disease most often affecting the abdomen. (cf. K.A. Blum et al)

Revealing symptoms include abdominal pain, nausea, hail occlusion, digestive hemorrhage, or appendicular syndrome.

The tumor masses are most often enormous affecting the intra-abdominal ganglia, the intestine but sometimes also the liver, the pancreas, the spleen, the kidneys or the ovaries. Rates of dehydrogenase lactate and uric acid are high.

An invasion of the bone marrow is present in about 35% of cases and a central nervous system is affected in 15% of cases.

Histology
Many denominations have existed describing both the lymphomateux B aspect of high grade and even the leukemia acute lymphoblastic aspect.

The main cytological features, but especially immunohistotypiques, are highlighted by immunohistochemistry, B lymphocytes are CD20 +, CD10 +, Ki67 + (in 100% of cells), TdT-(terminal deoxynucleotidyl transferase Transdon) and CD34-.

The translocation T (8; 14) (q24; q32) interesting chromosome 14q32 (immunoglobulin gene) and 8q24 (oncogene MYC) is highlighted by a FISH analysis.

An essential differential diagnosis is diffuse lymphoma with large cells B: Modern studies of transcriptional and genomic profiles make it possible to distinguish Burkitt lymphoma. (cf. M. Hummel et al or S.S. Dave et al.)

Treatment
Due to the rapidity of proliferation, treatment uses short and intensive treatment that can be repeated without delay of cures.

Various complex therapeutic associations have been developed. Prophylactic treatment in the spinal cord is often undertaken. The observed toxicity is major and can be lethal. The non-recurrence survival obtained in adult forms would be in the order of 60% for patients with poor prognosis (significant tumor mass, neurological impairment), higher (80-90%) in subjects with good factors Prognosis.

Because of the positivity to the surface Antigen CD 20, a treatment by rituximab seems to increase the number of complete remissions necessary to obtain the cure.