Matches in SemOpenAlex for { <https://semopenalex.org/work/W1966636038> ?p ?o ?g. }
- W1966636038 endingPage "262" @default.
- W1966636038 startingPage "256" @default.
- W1966636038 abstract "Transient leukemia of Down syndrome (DS-TL), also known as transient myeloproliferative disorder of Down syndrome (DS) and transient abnormal myelopoiesis of DS, occurs in approximately 10% of DS neonates and in phenotypically normal neonates with trisomy 21 mosaicism. In DS-TL, peripheral blood analysis shows variable numbers of blasts and, usually, thrombocytopenia; other cytopenias are uncommon. Bone marrow characteristics of DS-TL are, likewise, variable, though (in contrast to other leukemias) the bone marrow blast differential can be lower than the peripheral blood blast differential. The blasts of DS-TL typically show light microscopic, ultrastructural, and flow cytometric evidence of megakaryocyte differentiation. DS-TL neonates have a approximately 15% risk of developing potentially fatal liver disease and show <10% incidence of hydrops fetalis. Additional manifestations of DS-TL include cutaneous involvement, hyperviscosity, myelofibrosis, cardiopulmonary failure, splenomegaly, and spleen necrosis. Despite its typical transient nature, 20% to 30% of DS-TL patients develop overt (nontransient) acute leukemia, usually within 3 years and typically of the M7 phenotype (acute megakaryoblastic leukemia). The pathogenesis of DS-TL (and of subsequent acute leukemia) involves mutation of GATA1 (on chromosome X), which normally encodes a transcription factor integral to normal development of erythroid, megakaryocytic, and basophilic/mast cell lines. The pathogenetic role of trisomy 21 in DS-TL is unclear. Though indications for chemotherapy in DS-TL have not been firmly established, the blasts of DS-TL are sensitive to low-dose cytosine arabinoside." @default.
- W1966636038 created "2016-06-24" @default.
- W1966636038 creator A5050805983 @default.
- W1966636038 date "2006-09-01" @default.
- W1966636038 modified "2023-09-27" @default.
- W1966636038 title "Transient Leukemia (Transient Myeloproliferative Disorder, Transient Abnormal Myelopoiesis) of Down Syndrome" @default.
- W1966636038 cites W103664678 @default.
- W1966636038 cites W111018162 @default.
- W1966636038 cites W1484357201 @default.
- W1966636038 cites W1566161459 @default.
- W1966636038 cites W187528536 @default.
- W1966636038 cites W1969012158 @default.
- W1966636038 cites W1969751819 @default.
- W1966636038 cites W1970974913 @default.
- W1966636038 cites W1973328753 @default.
- W1966636038 cites W1974150123 @default.
- W1966636038 cites W1974957584 @default.
- W1966636038 cites W1975033967 @default.
- W1966636038 cites W1976386009 @default.
- W1966636038 cites W1979306518 @default.
- W1966636038 cites W1981600597 @default.
- W1966636038 cites W1982659649 @default.
- W1966636038 cites W1988996736 @default.
- W1966636038 cites W1991711682 @default.
- W1966636038 cites W1998428834 @default.
- W1966636038 cites W1999820945 @default.
- W1966636038 cites W2001573299 @default.
- W1966636038 cites W2002765955 @default.
- W1966636038 cites W2005388260 @default.
- W1966636038 cites W2008297559 @default.
- W1966636038 cites W2009987531 @default.
- W1966636038 cites W2021069529 @default.
- W1966636038 cites W2021434249 @default.
- W1966636038 cites W2027478369 @default.
- W1966636038 cites W2034314978 @default.
- W1966636038 cites W2036667633 @default.
- W1966636038 cites W2038290309 @default.
- W1966636038 cites W2045669041 @default.
- W1966636038 cites W2046286855 @default.
- W1966636038 cites W2049064360 @default.
- W1966636038 cites W2049523938 @default.
- W1966636038 cites W2050658534 @default.
- W1966636038 cites W2054816172 @default.
- W1966636038 cites W2056516352 @default.
- W1966636038 cites W2068013911 @default.
- W1966636038 cites W2068717515 @default.
- W1966636038 cites W2070784564 @default.
- W1966636038 cites W2077287649 @default.
- W1966636038 cites W2078755045 @default.
- W1966636038 cites W2090511809 @default.
- W1966636038 cites W2095616902 @default.
- W1966636038 cites W2108405301 @default.
- W1966636038 cites W2121788229 @default.
- W1966636038 cites W2124994818 @default.
- W1966636038 cites W2128687487 @default.
- W1966636038 cites W2129652709 @default.
- W1966636038 cites W2132473369 @default.
- W1966636038 cites W2135486939 @default.
- W1966636038 cites W2138390807 @default.
- W1966636038 cites W2143566773 @default.
- W1966636038 cites W2164914133 @default.
- W1966636038 cites W2169635474 @default.
- W1966636038 cites W2171689896 @default.
- W1966636038 cites W2190388056 @default.
- W1966636038 cites W2209189187 @default.
- W1966636038 cites W2274800635 @default.
- W1966636038 cites W2395271414 @default.
- W1966636038 cites W2409020528 @default.
- W1966636038 cites W2416272961 @default.
- W1966636038 cites W4239466169 @default.
- W1966636038 cites W4254264846 @default.
- W1966636038 cites W62044941 @default.
- W1966636038 cites W79877756 @default.
- W1966636038 cites W97477215 @default.
- W1966636038 cites W1978675074 @default.
- W1966636038 doi "https://doi.org/10.1097/01.pap.0000213039.93328.44" @default.
- W1966636038 hasPubMedId "https://pubmed.ncbi.nlm.nih.gov/16998319" @default.
- W1966636038 hasPublicationYear "2006" @default.
- W1966636038 type Work @default.
- W1966636038 sameAs 1966636038 @default.
- W1966636038 citedByCount "36" @default.
- W1966636038 countsByYear W19666360382012 @default.
- W1966636038 countsByYear W19666360382013 @default.
- W1966636038 countsByYear W19666360382014 @default.
- W1966636038 countsByYear W19666360382015 @default.
- W1966636038 countsByYear W19666360382016 @default.
- W1966636038 countsByYear W19666360382017 @default.
- W1966636038 countsByYear W19666360382018 @default.
- W1966636038 countsByYear W19666360382019 @default.
- W1966636038 countsByYear W19666360382020 @default.
- W1966636038 countsByYear W19666360382021 @default.
- W1966636038 countsByYear W19666360382022 @default.
- W1966636038 crossrefType "journal-article" @default.
- W1966636038 hasAuthorship W1966636038A5050805983 @default.
- W1966636038 hasConcept C109159458 @default.
- W1966636038 hasConcept C118552586 @default.
- W1966636038 hasConcept C126322002 @default.
- W1966636038 hasConcept C142724271 @default.