Multimodal Endovascular approach in a Case of Multiple Dural Arteriovenous Fistulae.
We describe a rare presentation of DAVF in a patient with multiple DAVF, at 3 separate sites within the cranial cavity; associated with a medical history of two traumatic motor-vehicle accidents, total anomalous pulmonary venous return (TAPVR), and acquired aplastic anemia. We describe our approach in the treatment of this complex condition. PMID: 31536637 [PubMed - in process]
Conditions: Aplastic Anemia; Paroxysmal Nocturnal Hemoglobinuria Interventions: Other: symptom questionnaire; Other: recording of vital signs; Other: European Organisation for Research and Treatment of Cancer (EORTC) quality of life questionnaire (QLQ)-C30 questionnaire; Other: interview Sponsors: University Hospital, Basel, Switzerland; ProPatient foundation of the University Hospital Basel; Novartis Not yet recruiting
Allogeneic hematopoietic stem cell transplantation (HSCT) is an effective and curative treatment option for patients with aplastic anemia (AA). High-dose cyclophosphamide (CY) (200 mg/kg) combined with anti-thymocyte globulin (ATG) is frequently used as a conditioning regimen, and shows excellent outcomes especially in HSCT from a human leukocyte antigen (HLA)-matched sibling . However, the cardiotoxicity of high-dose CY is a major problem , since patients with AA often have cardiac dysfunction due to long-term anemia and iron overload associated with massive transfusion.
ConclusionThe prevalence of PNH clones in MPN is similar to that described in myelodysplastic syndromes. Whether PNH clones influence MPN phenotype and complications should be studied prospectively in larger patient cohorts and over long-term follow-up.
ConclusionsSevere hypercalcemia and aplastic anemia are potential paraneoplastic syndromes of adult T-type lymphoblastic lymphoma, with fatal short-term outcome.
stic Anemia Working Party of the European Society for Blood and Marrow Transplantation Abstract This follow-up study of the randomized prospective trial included 192 patients with newly diagnosed severe aplastic anemia receiving Antithymoglobulin and Cyclosporine, with and without G-CSF. We aimed to evaluate the long-term effect of G-CSF on overall survival, event-free survival, probability of secondary myelodysplastic syndrome or acute myeloid leukemia, clinical paroxysmal nocturnal hemoglobinuria, relapse, avascular osteonecrosis and chronic kidney disease. The median follow-up was 11.7 years (10.9-12.5). Overal...
PMID: 31575670 [PubMed - in process]
Abstract Fanconi anemia, an inherited bone marrow failure syndrome, caused by mutations in DNA repair genes, is characterized by congenital anomalies, aplastic anemia, high risk of malignancies and extreme sensitivity to alkylating agents. We aimed to study the clinical presentation, molecular diagnosis and genotype-phenotype correlation among patients with Fanconi anemia from the Israeli inherited bone marrow failure registry. Overall, 111 patients of Arab (57%) and Jewish (43%) descent were followed for a median of 15 (range 0.1-49) years; sixty-three percent were offspring of consanguineous parents. One-hundred...
Bone Marrow Transplantation, Published online: 25 September 2019; doi:10.1038/s41409-019-0680-4Autoimmune cytopenias (AIC) following allogeneic haematopoietic stem cell transplant for acquired aplastic anaemia: a joint study of the Autoimmune Diseases and Severe Aplastic Anaemia Working Parties (ADWP/SAAWP) of the European Society for Blood and Marrow Transplantation (EBMT)
Bone Marrow Transplantation, Published online: 24 September 2019; doi:10.1038/s41409-019-0696-9Excellent outcomes of hematopoietic stem cell transplantation with total nodal irradiation and antithymocyte globulin conditioning in severe aplastic anemia with advanced age and/or severe comorbidity
Condition: Aplastic Anemia Intervention: Drug: Romiplostim Sponsor: Kyowa Kirin Co., Ltd. Not yet recruiting