Adrianna Vlachos
Hofstra University
Publications 57
#1Zora R. Rogers (UTSW: University of Texas Southwestern Medical Center)H-Index: 30
#2Taizo A. Nakano (University of Colorado Denver)H-Index: 4
Last.Akiko Shimamura (Boston Children's Hospital)H-Index: 38
view all 29 authors...
Quality of response to immunosuppressive therapy and long-term outcomes for pediatric severe aplastic anemia remain incompletely characterized. Contemporary evidence to inform treatment of relapsed or refractory severe aplastic anemia are also limited for pediatric patients. The clinical features and outcomes for 314 children treated from 2002-2014 with immunosuppressive therapy for acquired severe aplastic anemia were analyzed retrospectively from 25 institutions in the North American Pediatric...
1 CitationsSource
#1Jacob C. Ulirsch (Broad Institute)H-Index: 18
#2Jeffrey M. Verboon (Broad Institute)H-Index: 2
Last.Hanna T. Gazda (Broad Institute)H-Index: 23
view all 42 authors...
Diamond-Blackfan anemia (DBA) is a rare bone marrow failure disorder that affects 7 out of 1,000,000 live births and has been associated with mutations in components of the ribosome. In order to characterize the genetic landscape of this heterogeneous disorder, we recruited a cohort of 472 individuals with a clinical diagnosis of DBA and performed whole-exome sequencing (WES). We identified relevant rare and predicted damaging mutations for 78% of individuals. The majority of mutations were sing...
14 CitationsSource
#1Adrianna Vlachos (Hofstra University)H-Index: 21
#2Evangelia Atsidaftos (The Feinstein Institute for Medical Research)H-Index: 2
Last.Jeffrey M. Lipton (The Feinstein Institute for Medical Research)H-Index: 35
view all 17 authors...
#1Adrianna Vlachos (Hofstra University)H-Index: 21
#2Philip S. Rosenberg (NIH: National Institutes of Health)H-Index: 60
Last.Jeffrey M. Lipton (Hofstra University)H-Index: 35
view all 8 authors...
TO THE EDITOR: Inherited bone marrow failure syndromes (IBMFSs), characterized by bone marrow failure and developmental anomalies, are among a group of rare cancer predisposition syndromes. As illustrated by Fanconi anemia (FA),[1][1] the unique biology of this IBMFS has provided important insights
6 CitationsSource
#1Jimmy HomH-Index: 3
#2Brian M. DulmovitsH-Index: 2
Last.Lionel BlancH-Index: 19
view all 6 authors...
#1Adrianna VlachosH-Index: 21
#2Diana S. OsorioH-Index: 3
Last.Jeffrey M. LiptonH-Index: 1
view all 16 authors...
#1Kelly A. O'Brien (NIH: National Institutes of Health)H-Index: 2
#2Jason E. Farrar (University of Arkansas for Medical Sciences)H-Index: 13
Last.David M. Bodine (NIH: National Institutes of Health)H-Index: 50
view all 13 authors...
Diamond-Blackfan anemia (DBA) is a congenital bone marrow failure syndrome characterized by erythroid hypoplasia, usually without perturbation of other hematopoietic lineages. Approximately 65% of DBA patients with autosomal dominant inheritance have heterozygous mutations or deletions in ribosomal protein (RP) genes while <1% of patients with X-linked inheritance have been identified with mutations in the transcription factor GATA1 Erythroid cells from patients with DBA have not been well chara...
22 CitationsSource
#1Adrianna Vlachos (Hofstra University)H-Index: 21
#2Philip S. Rosenberg (NIH: National Institutes of Health)H-Index: 60
Last.Jeffrey M. Lipton (Hofstra University)H-Index: 35
view all 6 authors...
Diamond Blackfan anemia (DBA) is an inherited bone marrow failure syndrome (IBMFS) characterized by red cell aplasia and congenital anomalies. In our 2012 report the predisposition to cancer was quantified for the first time by the DBA Registry of North America (DBAR), the largest established DBA patient cohort with prospective follow-up since 1991. Five years from the original analysis, this update of the cancer incidence in patients with DBA reveals additional solid tumors, in particular gastr...
7 CitationsSource
#1Brian M. Dulmovits (Hofstra University)H-Index: 1
#2Abena O. Appiah-Kubi (Hofstra University)H-Index: 2
Last.Lionel Blanc (Hofstra University)H-Index: 19
view all 21 authors...
Current therapeutic strategies for sickle cell anemia are aimed at reactivating fetal hemoglobin. Pomalidomide, a third-generation immunomodulatory drug, was proposed to induce fetal hemoglobin production by an unknown mechanism. Here, we report that pomalidomide induced a fetal-like erythroid differentiation program, leading to a reversion of γ-globin silencing in adult human erythroblasts. Pomalidomide acted early by transiently delaying erythropoiesis at the burst-forming unit-erythroid/colon...
25 CitationsSource
#1Amit Lahoti (Hofstra University)H-Index: 2
#2Yael Harris (Hofstra University)H-Index: 6
Last.Adrianna Vlachos (Hofstra University)H-Index: 21
view all 6 authors...
Background Diamond–Blackfan anemia (DBA) is a rare inherited bone marrow failure syndrome. The mainstays of treatment involve chronic red cell transfusions, long-term glucocorticoid therapy, and stem cell transplantation. Systematic data concerning endocrine function in DBA are limited. We studied patients in the DBA Registry (DBAR) of North America to assess the prevalence of various endocrinopathies. Procedure In a pilot study, retrospective data were collected for 12 patients with DBA. Subseq...
5 CitationsSource