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Blastic plasmacytoid dendritic cell neoplasm BPDCN is a rare and aggressive leukemia for which we developed a nationwide network to collect data from new cases diagnosed in France. In a retrospective, observational study of 86 patients , we described clinical and biological data focusing on morphologies and immunophenotype. Fifty-one percent of cytogenetic abnormalities impact chromosomes 13, 12, 9, and Myelemia was associated with an adverse prognosis.
We categorized chemotherapeutic regimens into 5 groups: acute myeloid leukemia AML -like, acute lymphoid leukemia ALL -like, lymphoma cyclophosphamide, doxorubicin, vincristine, and prednisone [CHOP] -like, high-dose methotrexate with asparaginase Aspa-MTX chemotherapies, and not otherwise specified NOS treatments.
Thirty patients received allogeneic hematopoietic cell transplantation allo-HCT , and 4 patients received autologous hematopoietic cell transplantation. Eleven patients are in persistent complete remission after allo-HCT with a median survival of 49 months vs 8 for other patients. Our series confirms a high response rate with a lower toxicity profile with the Aspa-MTX regimen, offering the best chance of access to hematopoietic cell transplantation and a possible cure.
Abstract Blastic plasmacytoid dendritic cell neoplasm BPDCN is a rare and aggressive leukemia for which we developed a nationwide network to collect data from new cases diagnosed in France.
Publication types Research Support, Non-U. Substances Biomarkers.