Burkitt lymphoma is a high-grade B-cell neoplasm. It has 2 major forms, the endemic (African) form and the nonendemic (sporadic) most often involves the maxilla or mandible.
Burkitt lymphoma is a high-grade B-cell neoplasm. It has 2 major forms, the endemic (African) form and the nonendemic (sporadic) most often involves the maxilla or mandible.
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Burkitt lymphoma is a high-grade B-cell neoplasm. It has 2 major forms, the endemic (African) form and the nonendemic (sporadic) most often involves the maxilla or mandible.
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Attribution Non-Commercial (BY-NC)
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Baixe no formato DOC, PDF, TXT ou leia online no Scribd
Definition Burkitt lymphoma is endemic in certain regions of
It is a high-grade B-cell neoplasm and has 2 major forms, the equatorial Africa and other tropical locations between endemic (African) form and the nonendemic (sporadic) form. latitudes 10° south and 10° north. Incidence in these Burkitt lymphoma is a childhood tumor but it is observed in areas of endemic disease is 100 per million children. adult patients. Mortality/Morbidity: Burkitt lymphoma is one of the fastest growing malignancies With combination chemotherapy and CNS in humans, with a very high growth fraction. prophylaxis, the survival rate is now at least 60%. Pathophysiology: Patients with limited disease have a survival rate of Burkitt lymphoma is a monoclonal proliferation of B 90%. lymphocytes characterized by small noncleaved cells that are Patients with bone marrow and CNS involvement uniform in appearance and that produce a diffuse pattern of have a poor prognosis. Adults with the disease, tissue involvement. especially those in the advanced stage, do more -Under the microscope, Burkitt lymphoma is characterized poorly than affected children. by the presence of a "starry sky" appearance (also observed Sex: in other highly proliferative lymphomas), imparted by The male-to-female ratio is 2-3:1. scattered macrophages with phagocytes cell debris. Age: -The African form most often involves the maxilla or -Burkitt lymphoma is most common in children. In mandible. The involvement of abdominal organs, such as the Africa, the mean age is 7 years. kidneys, ovaries, or retroperitoneal structures, is slightly less -Outside Africa, the mean age is 11 years. common. In contrast, the sporadic form usually involves Clinical presentation abdominal organs, with the most common involvement of the History: distal ileum, cecum, or mesentery and less common In the African form involvement of other abdominal organs, pelvic organs, and Most often present with swelling of the facial bones. affected jaw other facial bones, loosening of Most Burkitt lymphomas carry a translocation of the c-myc the teeth oncogene from chromosome 8 to either the immunoglobulin Swelling of the lymph nodes, which are (Ig) heavy-chain region on chromosome 14 [t(8;14)] or one nontender and rapidly growing, in the neck or of the light-chain loci on chromosome 2 (kappa light chain) below the jaw. [t(8;2)] or chromosome 22 (lambda light chain) [t(8;22)]. Abdominal presentation is slightly less The Epstein-Barr virus (EBV) has been implicated strongly common in the African form, while the relationship is less clear in the Wt loss, Night sweat, generalized fatigue. sporadic form. Sporadic form -EBV is associated with about 20% of sporadic cases. Abdominal tumors causing swelling and pain -Rare adult cases are associated with immunodeficiency, in the affected area. particularly AIDS. Some patients present with symptoms of -The lymphocytes have receptors for EBV and are its specific bowel obstruction secondary to an ileal-cecal target. intussusception caused by tumor growth. -In the African form, the hosts are believed to be unable to -Because of the rapid growth of the Burkitt tumor, mount an appropriate immune response to primary EBV patients may quickly manifest significant metabolic infection, possibly because of coexistent malaria or another derangement and renal function impairment infection that is immunosuppressive. Months to years later, excessive B cell proliferation occurs
)The most commonly used staging system for NHL
in children is that of the St. Jude's Children's -Less common presentations of Burkitt lymphoma include an Research Hospital. epidural mass, skin nodules, CNS symptoms, and bone (Stage I) marrow involvement. It separates patients with a single tumor or diseased -Rare cases of Burkitt lymphoma can present as acute lymph node leukemia (L3-ALL) with fever, anemia, bleeding, and (Stage II) adenopathy. or two or more tumors or diseased lymph nodes on Physical: the same side of the diaphragm Major signs of Burkitt lymphoma include a soft tissue mass (Stage III) associated with the involvement of the jaw or other facial from those with a large chest or abdominal tumor or bones, enlarged cervical lymph nodes, abdominal masses, (Stage IV) and ascites. Involvement of the bone marrow and central nervous system Staging The most common system of staging for non-Hodgkin's Investigations lymphomas in adults, including Burkitt's lymphoma, is the Lab Studies: Ann Arbor system. 1.FBC and ESR Stage I: 2.Electrolytes-serum levels of sodium, potassium, - Limited to one group of lymph nodes either above or calcium, phosphorus, magnesium below the diaphragm, 3.Urea, and creatinine - Organ or part of the body other than the lymph nodes, 4.Uric acid levels but has not spread to other organs or lymph nodes. 5.Liver function tests. Stage II: 6.Biopsy of suspected lymph nodes or other disease - The lymphoma in two or more lymph node groups on sites, including bone marrow, is essential for the the same side of the diaphragm diagnosis. - only one organ involved and has spread to the lymph 7.FNA of the lymph node may also be done. nodes near that organ 8.Cerebrospinal fluid (CSF) also should be evaluated Stage III: in all cases. - The lymphoma is present in groups of lymph nodes 8.Cytogenetic studies of peritoneal or pleural fluid, on both sides of the diaphragm. when appropriate, should be performed for diagnostic - It may involve an organ or site outside the lymph or staging purposes. nodes, the spleen, or both. Imaging Studies: Stage IV: 1. CXR -rule out lung metastasis and mediastinal - The lymphoma is disseminated (spread) throughout involvement. Chest CT should be performed if chest one or more organs outside the lymph nodes. There x-ray is abnormal may or may not be involvement of lymph nodes that 2.Abdominal U/S are remote from the affected organs. 3. Abdominal CT scan -retroperitoneal and For each of stages mesenteric lymph nodes, liver, kidneys, ovaries, and A -No general symptoms other structures. B- Is used for patients with any of the following: 4.Head or spinal CT scan or MRI is indicated if Unexplained loss of more than 10% of body weight in neurological signs and symptoms are present. the last six months 5.Bone scan and plain bone radiographs are needed Unexplained fever for patients with symptoms of bone involvement. Drenching night sweats E - malignancies outside the lymph nodes Other sites in the body are identified with additional letters, such as D for the skin or H for the liver.
Burkitt cells are homogeneous in size and shape, with round to oval nuclei and slightly coarse chromatin, with multiple nucleoli, and with intensely basophilic vacuolated cytoplasm that contains neutral fat. Frequent mitotic figures usually are observed. A starry sky appearance is imparted by scattered macrophages with phagocyte cell debris.