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Related Subjects: | Hodgkin Lymphoma | Non Hodgkin Lymphoma | Diffuse large B-cell lymphoma | Intravascular large B-cell lymphoma | Mantle cell lymphoma | Marginal Zone Lymphoma | Gastric (MALT) Lymphoma | Primary CNS Lymphoma (PCNSL) | Burkitt's lymphoma | Follicular Lymphoma

Patients achieving complete remission must be followed carefully during the first year to detect recurrence of the disease. More than 80% of patients sustain their remission one year following initial treatment and are considered cured.

About

• Monoclonal proliferation of B lymphocytes
• Very Aggressive B-cell form of non-Hodgkin lymphoma
• Accounting for less than 1% of all B-cell lymphomas
• Described by Denis Burkitt an Irish Surgeon working in Africa in the 1950s

Aetiology

• It is the first human tumour to be causally associated with a virus (EBV)
• Burkitt lymphoma is a cancer of the lymphatic system.
• Most due to translocations that involve the Myc gene on chromosome 8.

Translocations

• t(8;14) (q24;q32) in 70%
• t(2;8)(p12; q24)
• t(8;22)(q24; q11).

Types

• Sporadic: throughout most of the world often involves abdomen and bone marrow as well as kidneys, ovaries, breasts. Affects children and young adults.
• Endemic: mostly seen in Equatorial Africa associated with the Epstein-Barr virus (EBV) and possibly co-infection with malaria. 4 to 7 years old, with a male: female ratio of 2:1
• Immunodeficiency-related disease: HIV/AIDS x1000 the risk of developing BL. Usually EBV negative also affects the recipients of organ transplants

Clinical

• Most commonly in children and young adults.
• Affects jaw, central nervous system, bone marrow
• Hepatomegaly and adenopathy
• bowel, kidneys, ovaries or other organs.
• Leukaemia: ALL, L3 type

Differentials

• Diffuse large B-cell lymphoma

Investigations

• FBC, U&E, LFTs, ESR, Ca, LDH: elevated with bulky disease
• Fine-needle aspirate is performed, it should not delay an excisional biopsy necessary for definitive diagnosis.
• Flow cytometric analysis provides independent confirmation of the immunophenotype
• CT Chest abdomen and pelvis for staging
• PET scan: initial staging and to evaluate response to treatment
• Histology: diffuse infiltrate of atypical lymphoid cells with numerous mitoses and a prominent starry-sky pattern because of the presence of multiple tangible body macrophages. High-power magnification shows that the neoplastic cells are medium-sized, round, and uniform, with nuclei that are similar in size to or slightly smaller than the nuclei of the tangible body macrophages.

Management

• Staging must be completed rapidly in order to avoid delay of therapy, which must start within 48 hours after diagnosis. Therapy is toxic and results in significant myelosuppression and potentially life-threatening complications. In general children with BL have better survival rates than adults with BL
• Resection of large abdominal masses appears to be beneficial.
• Potentially curable with high-intensity, short-duration combination chemotherapy. However, import to ensure the prevention of tumour lysis syndrome
• Chemotherapy: Vincristine, Cyclophosphamide, 6 Mercaptopurine, Doxorubicin and Prednisolone. it is recommended to add rituximab to chemotherapy.
• CODOX-M and BFM regimens are most successful
• Radiotherapy is not indicated except in case of CNS involvement
• In the case of partial remission or with chemosensitive relapse, autologous stem cell transplantation is recommended following re-induction with non-cross-resistant polychemotherapy. Monitoring FBC and cognitive functions are important to detect the late toxicity of the applied therapies.

References

• Burkitt Lymphoma at statpearls
• BHS Guidelines for the treatment of Burkitt's lymphoma
• Burkitt Lymphoma: Clinicopathologic Features and Differential Diagnosis Judith A. Ferry