Olivia Reitsma (13146042)
GNK 286, Case study 5
Introduction
Burkitt’s Lymphoma was first described by Dr Denis Burkitt, who described the neoplasm in Ugandan children in 1957. 1,2 This report will cover the findings of a patient with a diagnosis of Burkitt Lymphoma, including the clinical presentation, disease history, treatment options and prognosis of the disease.
An overview
Burkitt Lymphoma can be described as a neoplasia of B cells that grows mainly in the jaw and sometimes also in the ovary and testis. Burkitt Lymphoma is a high grade Non-Hodgkin’s Lymphoma, histologically characterised by a mass of diffuse small non-cleaved B cell lymphocytes. 2 It usually presents in extranodal sites and has a very high proliferative rate. 2 It has…show more content… • Extradural lesion causing compression of the spinal cord.
• However, the child’s general condition is usually remarkably good. 2
Diagnosis
To diagnose Burkitt Lymphoma, a fine needle aspiration cytology of the mass or excision biopsy for histology is needed to confirm diagnosis.2
Cytological appearance:
Cells are fairly uniform in size, with rounded nuclei with granular nuclear chromatin, as well as a thin rim of basophilic cytoplasm. Vacuoles usually are present. The nucleus is slightly indented and has 2-5 nucleoli. 2
Histological appearance:
The distinctive ‘Starry Sky’ appearance is seen histologically with hematoxylin and eosin staining. 3 This pattern is made up of sheets of oval, monomorphic cells with large nuclei and multiple small nucleoli. These cells have a dark lilac cytoplasm, and a high mitotic activity. These cell are interspersed in-between macrophage debris, giving the typical ‘Starry Sky’ appearance. 3
The laboratory results of the Case Study shows typical endemic Burkitt’s Lymphoma:
First laboratory…show more content… 2
Supportive therapy will include the active prevention of tumour lysis syndrome, as well as sufficient hydration and Allopurinol at a daily dose of 10-20mg/kg.2
The mainstay of definitive treatment is chemotherapy, as the tumour is highly chemo sensitive. Cyclophosphamide is administered in a dose pf at least 1 g/m2 , together with a high or intermediate dose methotrexate. The chemotherapy is ‘short duration, high intensity’-treatment and, combined with CNS prophylaxis, shows excellent survival rates in children.2
Prognosis
Generally patients have a good prognosis. With modern chemotherapy, 85-100% of patients diagnosed in an early stage of the disease and 75-85% diagnosed in an advanced stage survive for 3 years. If there is no relapse after a year of chemotherapy, the patient has a 90% chance of indefinite survival. Therefore, Burkitt’s Lymphoma is a curable