Matches in SemOpenAlex for { <https://semopenalex.org/work/W2892057736> ?p ?o ?g. }
Showing items 1 to 58 of
58
with 100 items per page.
- W2892057736 abstract "7055 Background: Inherited marrow failure syndromes (IMFS) are considered diseases of childhood, but testing adults with atypical presentations of diseases associated with IMFS is critical for establishing an optimal treatment protocol. The most common IMFS, Fanconi Anemia and Telomere Biology Diseases, are associated with MDS and aplastic anemia (AA), head and neck cancers, skin, and cervical cancer. Such patients can have profound toxicity with standard chemotherapy regimens. Methods: We implemented a screening protocol in patients at highest risk of an undiagnosed IMFS (MDS under age 60, AA patients under age 65, and head and neck cancers under age 60) with chromosome breakage analysis and telomere length testing. Results: Our protocol diagnosed nine patients (estimated ~10% of patients < age 60 with these diseases) with IMFS. The features of these patients are described in the table. Only three (33%) patients had the classic physical characteristics of IMFS. Three patients were over age 50 when diagnosed with an inherited disorder. Most importantly, in all patients the treatment approach was modified significantly, including minimizing conditioning for BMT, utilizing danazol as first line treatment for AA, as well as aggressive cancer and endocrinopathy screening. Outcomes with modified treatment have been favorable, and occult malignancies were detected through screening in two patients. Conclusions: Inherited marrow failure syndromes are uncommon yet under recognized disorders that significantly impact treatment decisions in addition to implementation of surveillance programs for both the affected individual and their relatives alike. Through this approach of thoughtful screening of patients presenting to our institution with marrow failure we identified an unrecognized IMFS in an estimated 10% of patients. [Table: see text]" @default.
- W2892057736 created "2018-09-27" @default.
- W2892057736 creator A5052079637 @default.
- W2892057736 creator A5088234044 @default.
- W2892057736 date "2017-05-20" @default.
- W2892057736 modified "2023-09-27" @default.
- W2892057736 title "Implementation of a screening program for identification of unrecognized inherited marrow failure syndromes." @default.
- W2892057736 doi "https://doi.org/10.1200/jco.2017.35.15_suppl.7055" @default.
- W2892057736 hasPublicationYear "2017" @default.
- W2892057736 type Work @default.
- W2892057736 sameAs 2892057736 @default.
- W2892057736 citedByCount "0" @default.
- W2892057736 crossrefType "journal-article" @default.
- W2892057736 hasAuthorship W2892057736A5052079637 @default.
- W2892057736 hasAuthorship W2892057736A5088234044 @default.
- W2892057736 hasConcept C116834253 @default.
- W2892057736 hasConcept C126322002 @default.
- W2892057736 hasConcept C143998085 @default.
- W2892057736 hasConcept C177713679 @default.
- W2892057736 hasConcept C2780007613 @default.
- W2892057736 hasConcept C59822182 @default.
- W2892057736 hasConcept C71924100 @default.
- W2892057736 hasConcept C86803240 @default.
- W2892057736 hasConceptScore W2892057736C116834253 @default.
- W2892057736 hasConceptScore W2892057736C126322002 @default.
- W2892057736 hasConceptScore W2892057736C143998085 @default.
- W2892057736 hasConceptScore W2892057736C177713679 @default.
- W2892057736 hasConceptScore W2892057736C2780007613 @default.
- W2892057736 hasConceptScore W2892057736C59822182 @default.
- W2892057736 hasConceptScore W2892057736C71924100 @default.
- W2892057736 hasConceptScore W2892057736C86803240 @default.
- W2892057736 hasLocation W28920577361 @default.
- W2892057736 hasOpenAccess W2892057736 @default.
- W2892057736 hasPrimaryLocation W28920577361 @default.
- W2892057736 hasRelatedWork W106329933 @default.
- W2892057736 hasRelatedWork W1540905435 @default.
- W2892057736 hasRelatedWork W1782693745 @default.
- W2892057736 hasRelatedWork W2057746461 @default.
- W2892057736 hasRelatedWork W2061828269 @default.
- W2892057736 hasRelatedWork W2064833483 @default.
- W2892057736 hasRelatedWork W2087003147 @default.
- W2892057736 hasRelatedWork W2125875796 @default.
- W2892057736 hasRelatedWork W2127947926 @default.
- W2892057736 hasRelatedWork W2416164840 @default.
- W2892057736 hasRelatedWork W2530030168 @default.
- W2892057736 hasRelatedWork W2809200995 @default.
- W2892057736 hasRelatedWork W2918313080 @default.
- W2892057736 hasRelatedWork W3000157017 @default.
- W2892057736 hasRelatedWork W3013510758 @default.
- W2892057736 hasRelatedWork W3146577857 @default.
- W2892057736 hasRelatedWork W3184691007 @default.
- W2892057736 hasRelatedWork W582598643 @default.
- W2892057736 hasRelatedWork W603409736 @default.
- W2892057736 hasRelatedWork W7467202 @default.
- W2892057736 isParatext "false" @default.
- W2892057736 isRetracted "false" @default.
- W2892057736 magId "2892057736" @default.
- W2892057736 workType "article" @default.