Two broad classification of lymphoma • Hodgkin lymphoma (HL): also known as Hodgkin disease (HD) which is named after Dr. Thomas Hodgkin who first described it. • Non-Hodgkin lymphoma (NHL): NHL tends to occurs in younger children, whereas HL is more likely to affects older children and teens. • The WHO classification of NHLs is based on B-cell malignancies T- cell malignancies 23-Aug-23 BURKITT LYMPHOMA 7
Three Classification of NHL : 1 . B-cell NHL: it originated from B-cells. Burkitt lymphoma/leukemia and diffuse large B-cell lymphoma 2 . Lymphoblastic lymphoma: It develops from T cells and usually starts from thymus (thymus contains T lymphocytes). It is rarely seen in tonsil too. 3.Large cell lymphoma: Large cell lymphomas arise from mature T and B cells and develop in any part of the body where lymphocytes are there . • Two main subtypes of large cell lymphoma are: • 1. Anaplastic Large cell lymphoma: it usually develops from mature T cells. It usually lymph nodes in the neck or other areas and may be found in the skin, lungs, bone, digestive tract or other organs. 23-Aug-23 BURKITT LYMPHOMA 8
Two main subtypes of large cell lymphoma are: 1. Anaplastic Large cell lymphoma: it usually develops from mature T cells. It usually lymph nodes in the neck or other areas and may be found in the skin, lungs, bone, digestive tract or other organs. 2. Diffuse large B-cell lymphoma: it starts in B cells. Also called primary mediastinal B-cell lymphoma. These lymphomas sometimes grow as large masses in the mediastinum. 23-Aug-23 BURKITT LYMPHOMA 9
Burkitt Lymphoma • Burkitt lymphoma is a form of non-Hodgkin's lymphoma. It is highly aggressive form of lymphoma resulting from translocation from chromosome 8 to chromosome 14 • It is named after Denis Parsons Burkitt, the Irish surgeon who first described the disease in 1958 while working in equatorial Africa. 23-Aug-23 BURKITT LYMPHOMA 10
Definition: • Brukitt lymphoma is also as small noncleaved cell lymphoma, it is a Non-Hodgkin Lymphoma of the high grade exclusively B- cell origin. • The disease may jaw, central nervous system, bowel, kidneys, ovaries or other organs. It also called Burkitt leukemia when it presents as tumour cells in bone marrow and the bloodstream. 23-Aug-23 BURKITT LYMPHOMA 11
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Endemic Burkitt lymphoma Endemic (African) form: the most common form, found mainly in central Africa, where it is associated with the Epstein Barr virus (EBV). It is most common in children. This form often manifests as enlargement of the jaw or facial bones Endemic Burkitt lymphoma primarily affects African children ages 4 to 7 and is twice as common in boys. 23-Aug-23 BURKITT LYMPHOMA 14
Sporadic Burkitt lymphoma • Sporadic (non-African) form: Sporadic Burkitt lymphoma occurs worldwide. • Globally, it accounts for 1% to 2% of adult lymphoma cases. • In the U.S. and Western Europe, it accounts for up to 40% of pediatric lymphoma cases. • That often develops in the abdomen with bone marrow involvement. The kidneys, ovaries, breasts or other organs may also be involved. • This form commonly affects children and young adults 23-Aug-23 BURKITT LYMPHOMA 15
Immunodeficiency-associated • This variant of Burkitt lymphoma is most common in people with HIV/AIDS. • It accounts for 30% to 40% of Non-Hodgkin lymphoma in HIV patients and may be an AIDS-defining disease. • It also can occur in people with congenital conditions that cause immune deficiency and in organ transplant patients who take immunosuppressive drugs 23-Aug-23 BURKITT LYMPHOMA 16
Etiology of Burkitt lymphoma Idiopathic Epstein-Barr Virus (EBV) Malaria Chromosomal abnormalities: translocation of C myc - gene HIV Infection Oncogenes 23-Aug-23 BURKITT LYMPHOMA 17
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CLINICAL MANIFESTATION 23-Aug-23 BURKITT LYMPHOMA 22 Swollen, painless lymph nodes in neck, armpit or groin Abdominal pain, swelling Earliest sign is the loosening of child’s molar or premolar. Intra-abdominal tumours , especially retroperitoneal lymph nodes or ovaries. Extradural lesion causing spinal cord compression and paraplegia. Enlargement of the parotid glands, breasts (usually both), testis, thyroid and kidneys (all are uncommon). Proptosis (bulging eyes) Weight loss Loss of appetite Fatigue Unexplained fever Night sweats
STAGING OF NON-HODGKIN LYMPHOMA 23-Aug-23 BURKITT LYMPHOMA 23
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History collection Physical Examination Complete Blood Cell Count (CBC) Count Serum Chemistry: LDH (Lactate Dehydrogenase) is a tumour marker to monitor response to treatment relapse. Uric Acid is a measure of tumour lysis . Computed tomographic (CT) imaging of the chest, abdomen, and pelvis Chest X-ray Testing for HIV disease CSF Examination for finding brain metastasis Cellular fluid cytology PET or gallium scan Bone marrow biopsy Cellular Biopsy 23-Aug-23 BURKITT LYMPHOMA 27
MANAGEMENT OF BURKITT LYMPHOMA Medical Management Intensive intravenous chemotherapy : Commonly drugs used are: Cyclophosphamide Vincristine Prednisone Doxorubicin 23-Aug-23 BURKITT LYMPHOMA 28
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References Nelson Essential of paediatrics Step up to Pediatrics Ghai Essential of paediatrics 23-Aug-23 BURKITT LYMPHOMA 48