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Why is Burkitt lymphoma so aggressive? And how do you best detect it?
Burkitt lymphoma grows slowly. The disease has clearly visible symptoms.
Burkitt lymphoma grows at an explosive rate. Pathological diagnostic confirmation is therefore needed as soon as possible.
Treating Burkitt lymphoma is difficult and has a poor success rate.
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Scientific background information
Burkitt lymphoma (BL) is a malignancy of the B cell lymphocytes. Three different variants are known to date:
- Endemic BL; occurs mainly in tropical Africa
- Sporadic BL; occurs mainly in temperate climate zones
- HIV-associated BL; restricted to HIV patients
Burkitt lymphoma is the fastest growing of all known cancers.
Translocation of the MYC gene on chromosome 8 is the fundamental transformative event as it seriously deregulates cell proliferation. The incidence of endemic BL increases significantly if patients have been previously infected with Epstein-Barr virus and malaria. Since this is not the case for the other BL variants, it is believed that a general serious immunodeficiency (e.g. HIV or AIDS) or a somatic mutation increases the probability of developing this disease.
Initially, the symptoms of BL are unspecific (e.g. lack of concentration, tiredness), particularly as the disease usually stays outside the lymph nodes. However, the tumour doubling rate is just 24 hours. Due to this explosive growth, the tumour is usually large when it is detected and diagnosis is required as soon as possible. The diagnosis is confirmed pathologically with fluorescence-in-situ-hybridisation (FISH) and tissue microarrays (TMA), which deliver a cost-efficient and standardised laboratory method for detecting the translocation of the MYC gene.
Due to the strong proliferation rate, BL is highly sensitive to chemotherapy and the chance of a cure is high as long as therapy starts at an early stage.
1. Laurent C et al. (2013): Whole-slide imaging is a robust alternative to traditional fluorescent microscopy for fluorescence in situ hybridization imaging using break-apart DNA probes. Human Pathology (2013) 44, 1544–1555.
2. Pizzo PA, Poplack DG (2001): Principles and Practice of Pediatric Oncology. 4th Edition. Lippincott Williams & Wilkins Publishers, Philadelphia / Baltimore / New York, ISBN 0-7817-2658-1.
3. In: Klöppel G, Stolte M, Rüschoff J (Eds., 2013) Burkitt-Lymphom – ICD-O 09687/3. Pathologie: Verdauungstrakt und Peritoneum. Springer, Heidelberg. DOI 10.1007/978-3-642-02322-4_1 (Reference in German language only)