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Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare hematologic malignancy with aggressive behavior and poor prognosis. We present the first retrospective analysis mapping its incidence and therapeutic outcomes in patients diagnosed and treated from 2000 to 2017 in the Czech Republic. The cohort comprised 14 patients (10 males, 4 females) with a median age at diagnosis of 39 years (range, 5-68 years). Initially, skin involvement was noted in 10 (71%) patients and bone marrow infiltration was present in 9 (64%). The first complete remission was achieved in 6/14 (43%) patients after acute lymphoblastic leukemia/lymphoma induction therapy and in 3/14 (21%) patients after acute myeloid leukemia regimen. Nine patients underwent allogeneic hematopoietic cell transplantation, with two patients achieving the first complete remission only after allogeneic transplantation. Patients undergoing allogeneic hematopoietic cell transplantation had longer overall survival than those treated without transplantation (the median survival over the period 16.4 vs. 8.1 months). Relapse of the disease was a significant predictor of mortality (p=0.05). Over the study period, patients’ survival ranged from 3.3 to 44.2 months, with a median overall survival of 13 months. Our results revealed an effectivity of allogeneic hematopoietic cell transplantation on complete remission achievement in refractory/relapsed disease. The study aimed to present the actual data from the Czech Republic and thus contribute to a global understanding of BPDCN. [ABSTRACT FROM AUTHOR]

The optimal time for starting G-CSF application after autologous peripheral stem cell transplantation (APSCT) still remains undetermined. All previous studies used 'fixed' days (0 or +1 vs +5 or +7 post-transplant) for this purpose. As many other drugs have individual, patient-dependent criteria (eg antibiotics, blood products, etc), and the discontinuation of G-CSF also has strict patient-dependent criteria (surprisingly absent when starting the drug) we suppose that attempts to find general criteria suitable for every patient may not be successful. In order to also take the patients' individual predispositions into account we designed a randomized clinical trial to compare 'immediate' administration of G-CSF (day +1: group A) vs 'delayed, patient-dependent' (first day when absolute neutrophil count (ANC) was below 0.5 x 10[sup9]/l: group B) therapy with G-CSF (both groups received 10 μg/kg/day i.v.). A total of 70 patients after APSCT suffering from non-Hodgkin's lymphoma (NHL) and Hodgkin's disease (HD) conditioned with BEAM, or from multiple myeloma (MM) after melphalan (L-PAM: 200 mg/m²) were enrolled in this study (35 in each group). Both groups were comparable with regard to age, sex, disease stage and previous therapy as well as the number of CD34+ cells transplanted. In group B, G-CSF administration began on day +4 post-transplant (+2- +5). There were no detectable differences seen in the hematopoietic recovery (time to reach ANC more than 0.5 x 10[sup9]/1:12 days vs 13 days; time to platelet recovery, more than 50 x 10[sup9]/1:24 days in both groups), use of blood products or antibiotics, infections, or days of hospitalization. Delayed GCSF application led to significant cost saving in terms of APSCT (approximately US$1341 for each patient).... [ABSTRACT FROM AUTHOR]

We report a patient who underwent peripheral blood hematopoietic progenitor (PBHP) autologous transplantation for his third SAA relapse. Although his repeated relapses were always successfully reversed by immunosuppression, he developed anaphylaxis with horse, rabbit and mouse immunoglobulins as a side-effect of his previous treatment. He was mobilized with G-CSF and underwent three leukaphereses in the period of hematologic recovery after the second relapse. Fourteen months later, he suffered his third relapse, rescued by autologous PBHP transplantation after cyclophosphamide and melphalan conditioning. At present he is 15 months after transplantation without any treatment, transfusion-independent and in good condition. Others have published the observation that sufficient PBHP can be collected at least in some patients with SAA. Our experience shows that in repeatedly relapsing SAA with no other treatment options autologous transplantation with previously collected PBHP can result in successful hematologic reconstitution. [ABSTRACT FROM AUTHOR]

Reports on an outpatient-based peripheral blood stem cells (PBSC) mobilizing regimen for hematological and non-hematological malignancies. Decsription of patients; Treatment using cyclophosphamide 3 g/m² and G-CSF; Measurment of complete blood counts and CD34[sup+] cells.