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View article: Orthopaedic Surgery Outcomes in Patients With Haemophilia A or B Treated With Extended Half‐Life Recombinant Factor VIII and IX Fc Fusion Proteins: A Multicentre Prospective Study
Orthopaedic Surgery Outcomes in Patients With Haemophilia A or B Treated With Extended Half‐Life Recombinant Factor VIII and IX Fc Fusion Proteins: A Multicentre Prospective Study Open
Introduction Haemophilia A and B are hereditary bleeding disorders that require multidisciplinary perioperative management. Data on orthopaedic surgery outcomes with extended‐half‐life (EHL) recombinant Fc‐fusion factor VIII (rFVIIIFc) and…
View article: Impaired platelet function in Hermansky-Pudlak syndrome associated with novel mutations in <i>HPS3</i>, <i>HPS6</i> and <i>HPS8</i> genes
Impaired platelet function in Hermansky-Pudlak syndrome associated with novel mutations in <i>HPS3</i>, <i>HPS6</i> and <i>HPS8</i> genes Open
We report three unrelated cases of Hermansky-Pudlak (HP) characterized by novel mutations in HPS3 (compound heterozygosity for c.1457G>A and c.1813G>T), HPS6 (homozygous c.210_211insGGGCC), and HPS8 (homozygous c.299dupC in BLOC1S3) genes.…
View article: Major Orthopaedic Surgery in Persons with Haemophilia A with and without Inhibitors Treated by Emicizumab: A Mid-Term, Large, and Successful Series at a Single Center
Major Orthopaedic Surgery in Persons with Haemophilia A with and without Inhibitors Treated by Emicizumab: A Mid-Term, Large, and Successful Series at a Single Center Open
Introduction: Patients with Haemophilia (PWH) need orthopaedic treatments and often they undergo surgery. Classically, PWH with inhibitors have to face such procedures earlier than other patients. Major orthopaedic surgery is not easy and …
View article: Real-World Efficacy and Safety of Plasma-Derived Von Willebrand Factor-Containing Factor VIII Concentrates in Patients With Von Willebrand Disease in Italy
Real-World Efficacy and Safety of Plasma-Derived Von Willebrand Factor-Containing Factor VIII Concentrates in Patients With Von Willebrand Disease in Italy Open
Plasma-derived von Willebrand factor-containing factor VIII concentrates (pd-VWF/FVIII-C) are the mainstay of treatment in von Willebrand disease (VWD). Real-world data on efficacy and safety of these pd-VWF/FVIII-C are required. To retros…
View article: Supplemental Table S1 from Osteogenic Potential of Mesenchymal Stromal Cells Contributes to Primary Myelofibrosis
Supplemental Table S1 from Osteogenic Potential of Mesenchymal Stromal Cells Contributes to Primary Myelofibrosis Open
Primers used for RQ-PCR.
View article: Supplemental Table S1 from Osteogenic Potential of Mesenchymal Stromal Cells Contributes to Primary Myelofibrosis
Supplemental Table S1 from Osteogenic Potential of Mesenchymal Stromal Cells Contributes to Primary Myelofibrosis Open
Primers used for RQ-PCR.
View article: Data from Osteogenic Potential of Mesenchymal Stromal Cells Contributes to Primary Myelofibrosis
Data from Osteogenic Potential of Mesenchymal Stromal Cells Contributes to Primary Myelofibrosis Open
Primary myelofibrosis is a myeloproliferative neoplasm that is a precursor to myeloid leukemia. Dysmegakaryopoiesis and extramedullary hematopoiesis characterize primary myelofibrosis, which is also associated with bone marrow stromal alte…
View article: Data from Osteogenic Potential of Mesenchymal Stromal Cells Contributes to Primary Myelofibrosis
Data from Osteogenic Potential of Mesenchymal Stromal Cells Contributes to Primary Myelofibrosis Open
Primary myelofibrosis is a myeloproliferative neoplasm that is a precursor to myeloid leukemia. Dysmegakaryopoiesis and extramedullary hematopoiesis characterize primary myelofibrosis, which is also associated with bone marrow stromal alte…
View article: Supplemental Table S2 from Osteogenic Potential of Mesenchymal Stromal Cells Contributes to Primary Myelofibrosis
Supplemental Table S2 from Osteogenic Potential of Mesenchymal Stromal Cells Contributes to Primary Myelofibrosis Open
Hematopoiesis support by MSCs in LTBMC BFU-E, CFU-G, CFU-M and CFU-GM by PMF (n=3) and healthy donors (HD, n=3) expressed as number of colonies for 104 bone marrow mononuclear cells.
View article: Supplemental Table S2 from Osteogenic Potential of Mesenchymal Stromal Cells Contributes to Primary Myelofibrosis
Supplemental Table S2 from Osteogenic Potential of Mesenchymal Stromal Cells Contributes to Primary Myelofibrosis Open
Hematopoiesis support by MSCs in LTBMC BFU-E, CFU-G, CFU-M and CFU-GM by PMF (n=3) and healthy donors (HD, n=3) expressed as number of colonies for 104 bone marrow mononuclear cells.
View article: Safe and Successful Surgical Outcome in Persons with Hemophilia A with and without Inhibitors Treated with Emicizumab: A Large, Single Center, Real-World Experience
Safe and Successful Surgical Outcome in Persons with Hemophilia A with and without Inhibitors Treated with Emicizumab: A Large, Single Center, Real-World Experience Open
Emicizumab is a humanized recombinant bispecific antibody, bridging together activated factor IX (FIXa) and factor X (FX), thus mimicking the activity of FVIII in vivo. Emicizumab is designed for long-term prophylaxis in patients with seve…
View article: Fractures needing orthopaedic surgery in haemophilic patients: long-term experience of a dedicated team at a single institution
Fractures needing orthopaedic surgery in haemophilic patients: long-term experience of a dedicated team at a single institution Open
Purpose: Haemophilia is a rare inherited X-linked bleeding disorder associated with various levels of coagulative factor VIII (type A) or IX (type B) deficit. Persons with haemophilia (PWH) can be affected by trauma and fractures just like…
View article: Splanchnic vein thromboses associated with myeloproliferative neoplasms: An international, retrospective study on 518 cases
Splanchnic vein thromboses associated with myeloproliferative neoplasms: An international, retrospective study on 518 cases Open
Myeloproliferative Neoplasms (MPN) course can be complicated by thrombosis involving unusual sites as the splanchnic veins (SVT). Their management is challenging, given their composite vascular risk. We performed a retrospective, cohort st…
View article: Myelodysplasia as assessed by multiparameter flow cytometry refines prognostic stratification provided by genotypic risk in systemic mastocytosis
Myelodysplasia as assessed by multiparameter flow cytometry refines prognostic stratification provided by genotypic risk in systemic mastocytosis Open
Systemic mastocytosis (SM) is characterized by extreme heterogeneity of manifestations and prognosis. Several disease‐related biomarkers, including clinical, hematological and molecular variables, have been correlated with prognosis. Altho…
View article: Validation of the Mayo alliance prognostic system for mastocytosis
Validation of the Mayo alliance prognostic system for mastocytosis Open
this analysis allowed to confirm the general validity of risk stratification of patients with systemic mastocytosis according to the newly developed Mayo models; furthermore, we revealed its performance in predicting prognosis of intermedi…
View article: Ruxolitinib for essential thrombocythemia refractory to or intolerant of hydroxyurea: long-term phase 2 study results
Ruxolitinib for essential thrombocythemia refractory to or intolerant of hydroxyurea: long-term phase 2 study results Open
Ruxolitinib for Essential Thrombocythemia Refractory to or Intolerant of
\nHydroxyurea: Long-Term Phase 2 Study Results
View article: del(12)(q24q24) SETD1B/GTF2H3
del(12)(q24q24) SETD1B/GTF2H3 Open
We identified a novel SETD1B/GTF2H3 fusion gene in a polycythemia vera (PV) patient with complex karyotype, harboring a cryptic deletion involving chromosome band 12q24.31. This rearrangement led to the juxtaposition of the SETD1B (SET dom…
View article: Safety and efficacy of ruxolitinib in splanchnic vein thrombosis associated with myeloproliferative neoplasms
Safety and efficacy of ruxolitinib in splanchnic vein thrombosis associated with myeloproliferative neoplasms Open
Splanchnic vein thrombosis (SVT) is one of the vascular complications of myeloproliferative neoplasms (MPN). We designed a phase 2 clinical trial to evaluate safety and efficacy of ruxolitinib in reducing splenomegaly and improving disease…
View article: The effect of arterial hypertension on thrombosis in low‐risk polycythemia vera
The effect of arterial hypertension on thrombosis in low‐risk polycythemia vera Open
low-risk PV patients with arterial hypertension represent a subgroup in which a more intensive therapy should be explored in prospective studies designed to examine whether the addition of cytoreductive drugs to phlebotomy and aspirin and …
View article: Improving prognostic tools in systemic mastocytosis: Insights from mutations
Improving prognostic tools in systemic mastocytosis: Insights from mutations Open
Systemic mastocytosis (SM) is a chronic myeloproliferative neoplasm that is highly heterogeneous in terms of clinical manifestations whilst apparently homogeneous in terms of molecular pathogenesis. As a matter of fact, in at least 90% of …
View article: Prognostic impact of bone marrow fibrosis in primary myelofibrosis. A study of the AGIMM group on 490 patients
Prognostic impact of bone marrow fibrosis in primary myelofibrosis. A study of the AGIMM group on 490 patients Open
The prognostic significance of bone marrow (BM) fibrosis grade in patients with primary myelofibrosis (PMF) is still debated. A fibrosis grade greater than 1 was shown to associate with higher risk of death, and addition of fibrosis grade …
View article: MR Imaging in non‐hepatosplenic extramedullary hematopoiesis in primary myelofibrosis
MR Imaging in non‐hepatosplenic extramedullary hematopoiesis in primary myelofibrosis Open
A 49-year-old man, diagnosed 2 years earlier with primary myelofibrosis (PMF), presented with a complaint of progressive dysesthesias involving the left leg and foot. When PMF was diagnosed, grade 3 fibrosis at bone marrow examination, an …
View article: Clinical presentation and management practice of systemic mastocytosis. A survey on 460 Italian patients
Clinical presentation and management practice of systemic mastocytosis. A survey on 460 Italian patients Open
Systemic mastocytosis is a rare heterogeneous myeloproliferative neoplasm characterized by abnormal proliferation and activation of mast cells. We describe a large multicentre series of 460 adult patients with systemic mastocytosis, with a…
View article: Epidemiology and clinical relevance of mutations in postpolycythemia vera and postessential thrombocythemia myelofibrosis: A study on 359 patients of the AGIMM group
Epidemiology and clinical relevance of mutations in postpolycythemia vera and postessential thrombocythemia myelofibrosis: A study on 359 patients of the AGIMM group Open
Transformation to secondary myelofibrosis (MF) occurs as part of the natural history of polycythemia vera (PPV‐MF) and essential thrombocythemia (PET‐MF). Although primary (PMF) and secondary MF are considered similar diseases and managed …
View article: JAK2V617F complete molecular remission in polycythemia vera/essential thrombocythemia patients treated with ruxolitinib
JAK2V617F complete molecular remission in polycythemia vera/essential thrombocythemia patients treated with ruxolitinib Open
this study reports on the occurence of JAK2V617F complete molecular remission in polycythemia vera/essential thrombocythemia patients treated with ruxolitinib.