Background and Objectives: Plasmablastic lymphoma (PBL) is an aggressive high grade non-Hodgkin lymphoma (NHL) occurring predominantly in Human Immunodeficiency Virus (HIV) infected adult patients. Rare cases are seen in children with immunodeficiency and HIV. This is the first paediatric case reported at Dr George Mukhari Academic Hospital (DGMAH).
A 13 year old male, HIV infected who defaulted highly active antiretroviral therapy (HAART). He presented with three week history of right cheek and eye swelling, and recurrent epistaxis. On physical examination, right eye proptosis and a right nasal purple mass were noted.
Methods: Investigations performed were: Imaging studies of the brain, Full Blood Count and Differential Count (FBCD), CD4 count, HIV viral load, bone marrow aspirate (BMA) with flow cytometry, trephine biopsy, sinonasal mass biopsy and immuno-histochemical stains of the biopsies.
Results: FBCD showed bicytopenia (haemoglobin 6.0 g/dl, platelet count 69 × 10^9/l), 12% atypical plasmacytoid lymphocytes and 2% blasts. CD4 count was 162 cells/mm^3 and HIV viral load of 471 000 copies. Sinonasal biopsy revealed infiltration by neoplastic cells of intermediate to large sizes, with eccentric nuclei, prominent nucleoli and high mitotic activity. These cells showed diffuse membranous and nuclear positivity for CD138 and IRF4/MUM1 respectively and were negative for CD20, CD3, BCL2 and BCL 6 suggestive of plasmablastic lymphoma. BMA showed 52% plasmacytoid lymphoid cells admixed with plasma cells. The trephine biopsy showed similar features as seen in the nasal biopsy. The features were consistent with infiltration by plasmablastic lymphoma.
Conclusion: PBL is estimated to be 2% of all HIV-related lymphomas and is rare in paediatric patients. The clinical course is very aggressive with most patients dying in the first year after diagnosis. This patient was treated with chemotherapy, unfortunatley he demised seven months after diagnosis and treatment. The outcome may have improved more with better management of the HIV infection.