burkitt's non hodgkin's lymphoma | Burkitt's lymphoma (Burkitt lymphoma)

Burkitt's lymphoma
(Burkitt lymphoma)



LBurkitt's lymphoma is a B lymphoma that is mainly manifested in children. There are endemic (African), sporadic (non-African) forms and linked to an immune deficiency.

Burkitt's lymphoma is endemic in Central Africa and accounts for 30% of the child's lymphoma in the United States. The endemic African form often begins with an increase in the volume of the jaw or face bones. In sporadic non-African Burkitt lymphoma, abdominal impairment predominates, often localized in the ileocecal-caecal or mesentery valve area. The tumor can cause obstruction of the intestine. kidneys, ovaries, or breasts may also be involved. In adults, the tumor mass can be important and the generalized disease with liver, spleen and bone marrow damage. CNS involvement is often present at diagnosis or at the time of lymphoma relapse.

Burkitt's lymphoma is the fastest growing human tumor whose pathology reveals a high mitotic rate, a monoclonal proliferation of B lymphocytes, and a ' starry sky ' pattern of benign macrophages that have engulfed lymphocytes Clever apoptotic. There is a characteristic genetic translocation involving the C-MYC gene on chromosome 8 and the immunoglobulin heavy chain gene on chromosome 14. The disease is closely associated with Epstein-Barr virus in endemic lymphoma; However, the causal role of Epstein-Barr virus remains uncertain. Burkitt's lymphoma is common during AIDS and is one of the defining conditions.

HIV-associated Burkitt lymphoma Burkitt associated with HIV Burkitt lymphoma associated with HIV
By the publisher's permission. According to Banks P, et al. In Atlas of clinical Hematology. Edited by JO Armitage. Philadelphia, Current Medicine, 2004.

Diagnosis
The diagnosis is based on the biopsy of a ganglion or organ eventually reached. Rarely, a laparotomy can be practiced both for diagnosis and for treatment. The definition of the stage requires the completion of a CT of the thorax, and abdominal-pelvic, a bone marrow biopsy, an CSF cytology and a PET. The classification procedures must be carried out quickly because of the rapid growth of the tumor.

Treatment
Intensive chemotherapy

Treatment should be initiated quickly because tumors develop rapidly. An alternating intensive polychemotherapy of cyclophosphamide, vincristine, doxorubicin, methotrexate, Ifosfamide, etoposide, Cytarabine (CODOX-M/VCI) plus rituximab gives a cure rate of > 90% in children and adults. Other protocols such as Rituximab plus etoposide, prednisone, vincristine (Oncovin), and doxorubicin (R-EPOCH) and rituximab plus cyclophosphamide, vincristine, doxorubicin (Adriamycin), and dexamethasone (R-Hyper CVAD) are also commonly Used successfully. Neuromeningitis prophylaxis is essential. Tumor lysis syndrome (management of adverse effects of cancer treatment: Tumor lysis syndrome) is common during treatment and patients should receive IV hydration, allopurinol often with rasburicase and associated with close monitoring of the Ionogram (including K and Ca).

In case of intestinal occlusion of tumor origin, even when the tumor has been completely resected during the initial therapeutic or diagnostic laparotomy, aggressive treatment remains indicated. Rescue treatment of treatment failures is generally unsuccessful, underlining the importance of a very aggressive initial treatment.