![]() MRI brain showed multifocal white matter changes affecting the occipital and parietal lobes ( Figure 1e). Coincident with the end of chemotherapy he developed rapid visual deterioration without any associated field defects, extra-ocular muscle weakness or retinal pathology. He subsequently received 4 cycles of R-GCVP chemotherapy followed by 2 cycles of Rituximab and made a good response with striking regression of his nodal and bony disease ( Figure 1d). Treatment with R-CHOP chemotherapy was limited to 2 cycles due to cumulative doxorubicin dose. In situ hybridization for EBV (EBER) was negative, however the sample was necrotic and it was therefore not clear whether this was an accurate result. PET-CT revealed enlarged FDG-avid nodes on both sides of the diaphragm with bone involvement ( Figure 1d) diffuse large B-cell lymphoma was diagnosed on lymph node histology ( Figure 1c). ![]() e MRI brain showing multifocal white matter changes in the occipital and parietal lobesĪt the age of 57 symptoms suggestive of lymphoproliferative disease recurred, this time associated with worsening of his intermittent thrombocytopenia, with platelet counts ranging from 33–237 ×10 9/L, intermittent neutropenia, severe CD4 and B-cell lymphopenia (CD4 count 0.09×10 9/L B cell count 0.02 ×10 9/L) and an EBV viral load of a million copies/ml. d PET-CT images showing widespread adenopathy with avid FDG uptake, hepatosplenomegaly and bony uptake in the pelvis and spine (top images) with regression of nodal and bony disease post-chemotherapy (bottom images). c Lymph node histology showing diffuse large B-cell lymphoma with extensive necrosis and fibrosis (top image) and ghostly outlines of very large CD20+ cells (bottom image). b MRI brain showing high T-2 signal in right occipital lobe (top image), which persisted and was associated with underlying atrophy 4 years later (bottom images). Although his neurological symptoms resolved over 4–6 months, 4 years later the MRI changes were persistent and associated with underlying atrophy ( Figure 1b).Ī Lymph node histology showing EBV-driven lymphoproliferative disease with atypical Reed-Sternberg-like cells (arrow, top image), staining for CD20 (middle image) and EBV LMP1 (bottom image). Shortly afterwards he developed left-sided homonymous hemianopia and pupillary mydriasis MRI brain showed features thought to be suggestive of posterior reversible encephalopathic syndrome (PRES) secondary to chemotherapy ( Figure 1b) lumbar puncture was not performed. Despite this, he made a good clinical and radiological response, his EBV viral load became undetectable and serum IgA and M recovered to normal levels. Treatment was curtailed after 5 out of 6 planned cycles due to Pneumocystis pneumonia (PCP). He was initially treated with 4 cycles of single agent Rituximab, however full R-CHOP chemotherapy was later instituted as his B-symptoms persisted. PET-CT showed widespread adenopathy with avid fluorodeoxyglucose (FDG) uptake and lymph node histology showed an EBV-driven lymphoproliferative disorder ( Figure 1a). Investigations demonstrated fluctuating EBV viremia up to 18,000 copies/ml and a decline of his previously normal serum IgA and IgM to undetectable levels. Although his platelet count was normal at presentation, a mild intermittent thrombocytopenia was noted during follow-up with counts ranging from 104–256×10 9/L.Īt the age of 52 he began to suffer from increased respiratory tract infections, associated with waxing and waning lymphadenopathy, hepatosplenomegaly and B-symptoms. He was therefore diagnosed with specific polysaccharide antibody deficiency and, in view of co-existing bronchiectasis, was commenced on immunoglobulin infusions with a good response over the next 16 years. He was able to mount responses to protein vaccines however, failed to respond to pneumococcal polysaccharide. Initial investigations revealed normal immunoglobulins, serum protein electrophoresis, and absolute counts of peripheral T-, B- and NK-cells, although his CD4:CD8 ratio was reversed at 0.76. In childhood he had 3 episodes of pneumonia requiring hospital admission, and family history was remarkable for unexplained neonatal death of a male sibling. The patient was originally referred to immunology at the age of 36 years, with a long history of sinopulmonary infections and left-sided bronchiectasis.
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