Clinical and Biological significance of MYC/BCL6 dual gene rearrangements and protein co-expression in de novo diffuse large B-cell lymphoma: a report from the International DLBCL Rituximab-CHOP Consortium Program
2015
S228 diagnosis was 57 with a range of 21 to 80 years. Stages at initial diagnosis were stage Ib (n1⁄42), stage III (n1⁄42), stage IVA (n1⁄44), and stage IVB (n1⁄49). The median duration of CRs is 6.65 years (range 2 to 13 years). The average time between diagnosis and CR was 2 years (range 1 to 11 years). Three achieved CR on combined immunomodulatory therapy (extracorporeal photopheresis (ECP), alphainterferon and/or oral retinoids), plus antibiotics, and topical steroids. One patient achieved CR on alemtuzumab, another on mogamulizumab. The other 11 failed various therapies, but achieved CR via total skin electron beam therapy and allogeneic stem cell transplantation (SCT). Conclusion: Sezary Syndrome is a rare and aggressive type of CTCL that is refractory to current therapies. We report that CR may be achieved in 3 ways: combined immunomodulatory therapy, targeted biological agents, or total skin electron beam followed by non-ablative allogenic SCT. Further investigation of SCT as a potential curative treatment for SS is warranted.
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