Thank you for all your input this week!This case looked at a 32 year old gentleman who was diagnosed with burkitt lymphoma (BL) following a 3 week history of sweats, weight loss and an increasing abdominal mass and splenomegally. He was also found to be HIV positive.
He was commenced on RCODOX-M/IVAC chemotherapy and was given rasburicase and aggressive fluid hydration to try and prevent tumour lysis. Following review by the ID team HAART was also started. His first lumbar puncture did not reveal any CNS involvement.
BL is a very aggressive B cell NHL which is associated with MYC translocations. Most commonly involving translocations between the MYC gene locus on chromosome 8 and Immunoglobulin heavy chain locus on chromosome 14.
There are 3 variants of BL. Endemic variant which is associated with EBV infection, which tends to be found in regions where malaria is endemic. Sporadic BL is found in areas where malaria is not endemic and only 10-20% of these are EBV positive. The 3rd variant is immunodeficiency associated BL. This is associated with HIV infection but can be found in people with other immuno deficient states eg post transplant. The incidence of BL among HIV positive patients has not declined despite the increase in antiretrovirals, and can develop regardless of the CD4 count.
Typical histological finding are of replacement of normal architecture by medium sized monomorphic lymphocytes, which have prominent nucleoli. Cytoplasm is basophilic with prominent vacuoles. Macrophages are also present and give the classical ‘starry sky’ appearance.
Cells express surface Ig and CD19/CD20/CD22/CD79a/BCL6/HLADR and are negative for CD5/CD23/Tdt/BCL2. They have a high proliferation fraction with Ki67 nearing 100%.
Patients often present with rapidly enlarging masses b symptoms and high LDH and can have spontaneous tumour lysis.
At diagnosis all patients should be screen for HIV and hepatitis viruses. And staging of disease should involved CT staging scan +/- PET CT, bone marrow and lumbar puncture. Base line ECHO/cardiac assessment should also be performed due to the use of anthracycline chemotherapy.
Patients diagnosed with BL are at risk of tumour lysis and treatment/monitoring for this are important when treating patients. LDH and Uric acid should be checked in all patients, and rasburicase should be considered.
BL tends to be poorly responsive to CHOP chemotherapy, however intensive chemotherapy regimes such as RCODOX-M/IVAC are potentialy curative. CNS involvement is not uncommon and varies between different subtypes of BL. Treatments should involve CNS directed therapy to treat disease/prevent CNS relapse. Due to the intensive nature of the chemotherapy patients will have significant myelosupression and will require blood product support with close monitoring for infections.
Following 1 cycle of RCODOX-M/IVAC our patients mid point scan showed excellent response to treatment. He required extended inpatient admissions with neutropenic sepsis. But went on to complete treatment and end of treatment ct showed complete remission.
Do you have any questions about this weeks case? Is there anything you would have done differently?
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