Case Summary – Burkitts lymphoma

Our patients initially presented with abdominal pain, which may have had many underlying aetiologies.  With a fairly acute onset there were many diagnoses that needed to be considered.  The key learning points in this case were the possibility of spontaneous tumour lysis, and the need for immediate treatment of the high grade aggressive lymphoma.   In this case, there were two causes for the acute renal failure – obstruction secondary to bulky disease, and the tumour lysis.  Another important aspect to consider is the possibility of thrombosis, given the significant lower limb swelling and underlying malignancy.

Burkitts lymphoma is a highly aggressive mature B-cell neoplasm often presenting in extranodal sites or as an acute leukemia.

Three different clinical variants of BL have been described:

• Endemic – common in Africa, children aged 4 to 7 years
• Sporadic – accounts for 1-2% of all adult lymphomas in western europe
• Immunodeficiency – patients with HIV.

Morphology:

Medium-sized cells with abundant, basophilic cytoplasm  – containing lipid vacuoles

Round nuclei with clumped chromatin and multiple nucleoli;

Diffuse, monotonous pattern of infiltration

These cells express surface IgM, Bcl-6, CD19, CD20, CD22, CD10, and CD79a, and are negative for CD5, CD23, and TdT.  The expression of Bcl-6 and CD10 suggest a germinal center origin.

Genetics

Eighty percent of burkitts lymphoma cases are positive for t(8;14), resulting in the juxtaposition of the c-myc gene on chromosome 8 with IgH enhancer elements on chromosome 14.

Treatment regimes

Short-duration, intensive regimens that minimize treatment delays and maintain serum drug concentrations over at least 48 to 72 hours have the greatest efficacy in Burkitts.  This is due to the high-growth fraction of burkitts cells (doubling time of approximately 25 hours) favoring re-entry of remaining viable malignant cells into the cell cycle and rapid growth between chemotherapy cycles.

Possible treatment regimes:

• Intensive, short-duration regimens like CODOX-M/IVAC
• Long-duration chemotherapy similar to acute lymphoblastic leukemia (ALL) treatment, like hyper-CVAD
• Combination regimens followed by autologous stem cell transplantation (SCT)
• Most current regimens have added rituximab to previously established chemotherapy regimens

Lower intensity regimes may be considered for our patient given his current clinical state, he may not tolerate such intensive treatment initially.

In cases in which the patient is found to be HIV positive, it is also essential to commence HAART therapy, in combination with the chemotherapy.

CNS involvement is common in cases of Burkitts lymphoma, therefore CNS prophylaxis is given in combination with systemic therapy.  This often involves intrathecal methotrexate or cytarabine.

Poor prognostic features:

• advanced age,
• advanced stage,
• poor performance status,
• CNS or bone marrow involvement,
• anemia,
• the presence of circulating blasts,
• an elevated LDH

references

http://bloodjournal.hematologylibrary.org/content/104/10/3009.long Adult Burkitt leukemia and lymphoma. Kristie A. Blum, Gerard Lozanski, and John C. Byrd