0000000000535111

AUTHOR

Jörg Chromik

showing 4 related works from this author

Clinical Presentation of Patients with Adult Late-Onset Telomere Biology Disorders - Results from the Aachen Telomeropathy Registry

2021

Abstract Introduction: Telomere biology disorders (TBD) are caused by mutations affecting proper telomere maintenance resulting in premature telomere shortening. Telomere length (TL) assessment is currently being used for screening and diagnosis of TBD of which Dyskeratosis congenita (DKC) is the most prominent TBD subtype typically found in children and adolescents. In adults, TBDs are characterized by a broad spectrum of more "cryptic" diverging mono- or oligosymptomatic clinical manifestations such as bone marrow failure (BMF), hepatopathy or interstitial lung disease (ILD). However, despite growing general clinical awareness and exertion of improved TL screening strategies, insufficient…

Pediatricsmedicine.medical_specialtyTelomere biologybusiness.industryImmunologymedicineLate onsetCell BiologyHematologyPresentation (obstetrics)businessBiochemistryBlood
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Erythropoietic cellular analyses in luspatercept-treated lower-risk myelodysplastic syndromes (MDS): Phase 2 PACE-MDS study.

2018

7018Background: Luspatercept (ACE-536) is a TGF-β family ligand trap promoting late-stage erythroid (E) differentiation and increases in hemoglobin. Endpoints of the ongoing, phase 2, open-label st...

Cancer ResearchOncologybusiness.industryPhase (matter)Myelodysplastic syndromesLuspaterceptCancer researchMedicineHemoglobinbusinessLigand (biochemistry)medicine.diseaseLower riskJournal of Clinical Oncology
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Luspatercept for the treatment of anaemia in patients with lower-risk myelodysplastic syndromes (PACE-MDS): a multicentre, open-label phase 2 dose-fi…

2017

Myelodysplastic syndromes are characterised by ineffective erythropoiesis. Luspatercept (ACE-536) is a novel fusion protein that blocks transforming growth factor beta (TGF β) superfamily inhibitors of erythropoiesis, giving rise to a promising new investigative therapy. We aimed to assess the safety and efficacy of luspatercept in patients with anaemia due to lower-risk myelodysplastic syndromes.In this phase 2, multicentre, open-label, dose-finding study (PACE-MDS), with long-term extension, eligible patients were aged 18 years or older, had International Prognostic Scoring System-defined low or intermediate 1 risk myelodysplastic syndromes or non-proliferative chronic myelomonocytic leuk…

AdultMaleIneffective erythropoiesismyalgiamedicine.medical_specialtyPediatricsTime FactorsMaximum Tolerated DoseAnemiaActivin Receptors Type IIRecombinant Fusion ProteinsKaplan-Meier EstimateLower riskmedicine.disease_causeRisk AssessmentSeverity of Illness IndexDisease-Free SurvivalDrug Administration Schedule03 medical and health sciences0302 clinical medicineGermanyInternal medicineSeverity of illnessmedicineHumansProspective StudiesProspective cohort studyAdverse effectAgedProportional Hazards ModelsDose-Response Relationship Drugbusiness.industryMyelodysplastic syndromesAnemiaMiddle AgedPrognosismedicine.diseaseSurvival AnalysisActivinsImmunoglobulin Fc FragmentsTreatment OutcomeOncologyMyelodysplastic Syndromes030220 oncology & carcinogenesisFemalemedicine.symptombusiness030215 immunologyThe Lancet Oncology
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Proteasome-inhibited dendritic cells demonstrate improved presentation of exogenous synthetic and natural HLA-class I peptide epitopes.

2004

The design and successful clinical implementation of cancer vaccines targeting the induction of T-cell mediated immunity is a rapidly evolving field that is hampered by an empirical selection of antigen and adjuvant. In particular, vaccines using defined tumor-associated peptide epitopes elicit only a restricted T-cell repertoire in a minority of patients. In this regard, vaccines comprising the whole spectrum of antigens presented by individual autologous tumors would be advantageous. In an in vitro model, we evaluated the capacity of naturally processed Epstein-Barr virus-transformed B-lymphoblastoid-cell line (LCL)-derived peptides to activate virus-specific CD8+ T cells of seropositive …

AntigenicityHerpesvirus 4 HumanT cellImmunologyHuman leukocyte antigenBiologyCD8-Positive T-LymphocytesIn Vitro TechniquesLymphocyte ActivationCancer VaccinesEpitopeMonocytesEpitopesAntigenHLA AntigensmedicineImmunology and AllergyHumansProtease InhibitorsAntigen PresentationImmunogenicityHistocompatibility Antigens Class IDendritic cellDendritic CellsCell Transformation ViralMolecular biologyCell biologyClone Cellsmedicine.anatomical_structureProteasome inhibitorLymphocyte Culture Test MixedProteasome Inhibitorsmedicine.drugJournal of immunological methods
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