ABSTRACT
Despite the development of highly effective, targeted inhibitors of B-cell proliferation and anti-apoptotic pathways in chronic lymphocytic leukemia (CLL), these treatments are not curative, and many patients will develop either intolerance or resistance to these treatments. Transformation of CLL to high-grade lymphoma-the so-called Richter syndrome (RS)-remains a highly chemoimmunotherapy-resistant disease, with the transformation occurring following targeted inhibitors for CLL treatment being particularly adverse. In light of this, cellular therapy in the form of allogenic stem cell transplantation and chimeric antigen receptor T-cell therapy continues to be explored in these entities. We reviewed the current literature assessing these treatment modalities in both high-risk CLL and RS. We also discussed their current limitations and place in treatment algorithms.
ABSTRACT
Histological features of Epstein-Barr virus (EBV) can rarely mimic lymphoma. A 25-year-old presented with a spontaneous splenic rupture following a short illness. Histopathology assessment of the splenic and marrow tissue was highly suggestive of peripheral T-cell lymphoma not-otherwise-specified (PTCL-NOS). T-cell receptor (TCR) PCR clonality studies revealed a monoclonal T-cell population expressing for both TCRß and γ, strongly suggestive of a T-cell clonal disease. EBV IgM was positive and IgG negative. EBV PCR was positive (7.02 ×10(4)/mL). Despite the strong suggestion of PTCL-NOS from histopathology and clonality studies, the decision was made to observe the patient and not start multiagent chemotherapy. The patient remained well, with no signs of PTCL and subsequently seroconverted to IgG+ EBV. We highlight the potential pitfall of acute EBV masquerading as PTCL and show the critical role of the multidisciplinary integration of histopathological, serology, molecular and clinical features to avoid misdiagnosis.
