Vindesine dexamethasone as a therapeutic option in elderly blastic plasmacytoid dendritic cell neoplasms: a monocentric experience
Poster
Blastic Plasmacytoid Dendritic Cell Neoplasm (BPDCN) is a rare and aggressive hematologic malignancy, characterized by the proliferation of plasmacytoid dendritic cell precursors co-expressing CD4, CD56 and the interleukin-3 receptor subunit alpha (CD123). It preferentially affects elderly males. Whatever the initial cutaneous presentation (typically purplish tumors or ecchymotic nodules or macules), involvement of blood and bone marrow appears rapidly in most patients, and is associated with poor prognosis. Acute leukemia-based multi-agent chemotherapy induction followed by allogeneic hematopoietic stem cell transplantation is the only treatment to achieve sustained remissions. Recently, CD123-directed cytotoxin tagraxofusp, obtained 90% overall response allowing patients to access stem-cell transplantation but at the cost of frequent and sometimes serious toxicity.
Unfortunately, the majority of patients are not eligible for these therapies due to their advanced age or co-morbidities and palliative approaches are then proposed. Vinca-alkaloids are occasionally used in leukemia in certain solid cancers. We report our experience with vindesine and dexamethasone in elderly patients with BPDCN.
Patients were 3 men and 3 women, with an average age of 85 (range: 78 to 93). Half of them presented with diffuse brownish to violaceous nodules or macules and the other half with an isolated violaceous nodule. Performance status according to ECOG was from 1 to 3, and no patient initially presented B symptoms. Of the three patients with bone marrow involvement at diagnosis, two had cytopenia. Any treatment with multidrug therapy was rejected in these elderly patients. Vindesine -dexamethasone was proposed as weekly cycles for a period of 1 month followed by monthly cycle. Patients received an average of 10 cycles (range: 5 to 21). A clinical partial (n=2) or complete response (n=3) was obtained in 5 patients, with a median duration of 9 months (2 to 69). One patient had a complete haematological response. Only two cases, with initial bone marrow infiltration, exhibited grade 1 haematological toxicity and two had peripheral neurological toxicity (grade 2). No patient presented infectious complication related to chemotherapy. The median overall survival was 13 months (5-82 months). All patients finally died of the disease with a bone marrow infiltration.
Our results in elderly compare favorably to more intensive chemotherapies used in younger patients. Median survival of our patients treated by vinca-alkaloids was greater than expected and published in elderly. Moreover, vindesine dexamethasone produced a sustained clinical response in the majority of patients, up to 6 years after discontinuation of treatment. This suggests that this may represent a therapeutic option for BPDCN to be propose even in very advanced age patients because it can lead to complete response and prolonging life expectancy with a favorable toxicity profile in such patients.
Unfortunately, the majority of patients are not eligible for these therapies due to their advanced age or co-morbidities and palliative approaches are then proposed. Vinca-alkaloids are occasionally used in leukemia in certain solid cancers. We report our experience with vindesine and dexamethasone in elderly patients with BPDCN.
Patients were 3 men and 3 women, with an average age of 85 (range: 78 to 93). Half of them presented with diffuse brownish to violaceous nodules or macules and the other half with an isolated violaceous nodule. Performance status according to ECOG was from 1 to 3, and no patient initially presented B symptoms. Of the three patients with bone marrow involvement at diagnosis, two had cytopenia. Any treatment with multidrug therapy was rejected in these elderly patients. Vindesine -dexamethasone was proposed as weekly cycles for a period of 1 month followed by monthly cycle. Patients received an average of 10 cycles (range: 5 to 21). A clinical partial (n=2) or complete response (n=3) was obtained in 5 patients, with a median duration of 9 months (2 to 69). One patient had a complete haematological response. Only two cases, with initial bone marrow infiltration, exhibited grade 1 haematological toxicity and two had peripheral neurological toxicity (grade 2). No patient presented infectious complication related to chemotherapy. The median overall survival was 13 months (5-82 months). All patients finally died of the disease with a bone marrow infiltration.
Our results in elderly compare favorably to more intensive chemotherapies used in younger patients. Median survival of our patients treated by vinca-alkaloids was greater than expected and published in elderly. Moreover, vindesine dexamethasone produced a sustained clinical response in the majority of patients, up to 6 years after discontinuation of treatment. This suggests that this may represent a therapeutic option for BPDCN to be propose even in very advanced age patients because it can lead to complete response and prolonging life expectancy with a favorable toxicity profile in such patients.