Dr Ian Hewson Director Dental Services The Alfred Hospital PO Box 315 Prahran VIC 3181 Email: firstname.lastname@example.org
Oral plasmablastic lymphoma is a rare malignancy that is associated with patients with HIV or other immunosuppression. This article describes a case of a patient with severe haemophilia A (<1% factor VIII) who had medically acquired HIV and hepatitis C, a CD4+ count of 192 cells/μL and a viral load of 33 200 copies/mL. The patient presented with a two-month history of a firm swelling around a lower molar. The tooth was removed and the surrounding tissue biopsied. The importance of obtaining an early definitive diagnosis and seeking adequate medical treatment is discussed.
Oral plasmablastic lymphoma (OPL), a form of non-Hodgkin lymphoma, is a rare aggressive malignancy that can occur in the oral cavity of immunodeficient patients, most particularly those with HIV infection. It was first described in 1997.1 Pre-highly active anti-retroviral therapy (HAART) data indicate that these tumours are aggressive, frequently resistant to therapy and often rapidly fatal,2 with a median survival of approximately five months.3 In the setting of HIV infection, plasmablastic lymphoma can evolve into plasmablastic leukaemia.4
The median age of presentation is 39 years, median CD4+ count of 173 cells/μL and an average of six years after diagnosis of HIV.5 Epstein-Barr virus (EBV) is detected in 75% and human herpes virus 8 in 16% of cases.5 OPL is an aggressive B-cell lymphoma that is likely to affect young HIV positive males who have a CD4+ cell count of less than 200 cells/μL and chronic HIV infection.5
Plasmablastic lymphoma accounts for 2.6% of all HIV-related non-Hodgkin lymphomas.6 There are three categories. The first type is of the oral mucosa, the second is extraoral including GI tract, lung and bones, and the third is associated with Castleman’s disease.
A male with severe haemophilia A (<1% Factor VIII) presented to the Dental Unit at The Alfred Hospital complaining of a tender and mobile 36. This had a firm swelling associated with the surrounding gingival tissues (Fig 1) and had been getting progressively worse over a two-month period. The patient’s local dentist had been adjusting the occlusion so all the occlusal porcelain had been removed from porcelain fused to metal (PFM) crown. A radiograph indicated sound endodontic therapy and no apparent bony destruction (Fig 2).
The patient had medically acquired HIV and hepatitis C, a CD4+ count of 192 cells/μL and a viral load of 33 200 copies/mL.
As the 36 was tender and mobile and slowly getting more symptomatic, the decision was made to remove it and biopsy the surrounding tissues. Under appropriate factor VIII cover, the tooth was removed and biopsy of the surrounding tissue taken. The biopsy confirmed the suspicion of a lymphoma, a definitive diagnosis of plasmablastic lymphoma with strong immunoreactivity with CD38, but no immunoreactivity with EBV was made.
The patient was contacted and advised to attend The Alfred Haematology/Oncology Unit for appropriate treatment. Unfortunately, he was advised by his local holistic dentist to seek alternative therapy and did not attend the hospital for another two weeks. By this stage the lymphoma had rapidly increased in size (Fig 3) and it was a further two weeks until he consented to conventional oncology treatment (Fig 4).
The lymphoma was at stage 1AE. The patient was commenced on HAART and a chemotherapy regime of Cyclophosphamide, Doxorubicin, Vincristine (Oncovin) and Prednisolone (CHOP) for five days every 14 days. Initially, there was a good response but a local relapse occurred after the fifth cycle. Salvage radiotherapy had a minimal effect and salvage chemotherapy of IVAC6 was administered with a good partial response.
The patient then underwent an autologous bone marrow transplant and maintenance of HAART. The HAART gave good HIV viral control with the viral load reduced to <50 copies/mL. Unfortunately, the patient discontinued his HAART after four months. His viral load increased to >100 000 copies/mL and his CD4+ count dropped to 7 cells/μL. He remained in remission for a further four months then suffered a systemic relapse and expired some 14 months after initial diagnosis.
Although in this case the patient developed an aggressive malignancy with a poor prognosis, considerable time was lost before initial diagnosis was made and then a further delay before anti-tumour treatment could be commenced.
If the dental practitioner is presented with an unusual clinical situation of which they are unsure, the patient should be referred to an appropriate specialist as soon as possible to enable a definitive diagnosis to be made. Life-threatening malignancies should be managed in the appropriate hospital setting.