Document Detail


Perinatal visceral fibrosis accompanying the megakaryoblastic leukemoid reaction of Down syndrome.
MedLine Citation:
PMID:  2140891     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
Two infants with Down syndrome, one 4 weeks old and the other stillborn, at necropsy showed hepatic and pancreatic fibrosis, which was very severe in the liver of the liveborn infant and in the pancreas of the stillbirth. The liveborn infant had typical hematological features of the transient congenital leukemoid reaction of Down syndrome, and the identification of a megakaryoblastic component was consistent with recent opinion that this is a spontaneously-remitting congenital megakaryoblastic leukemia. The hydropic stillborn infant had intense extramedullary megakaryocytosis. The visceral fibrosis may have had a pathogenesis similar to that postulated for the myelofibrosis of megakaryoblastic leukemia in older children.
Authors:
D M Becroft; L J Zwi
Publication Detail:
Type:  Case Reports; Journal Article    
Journal Detail:
Title:  Pediatric pathology / affiliated with the International Paediatric Pathology Association     Volume:  10     ISSN:  0277-0938     ISO Abbreviation:  Pediatr Pathol     Publication Date:  1990  
Date Detail:
Created Date:  1990-07-11     Completed Date:  1990-07-11     Revised Date:  2004-11-17    
Medline Journal Info:
Nlm Unique ID:  8303527     Medline TA:  Pediatr Pathol     Country:  UNITED STATES    
Other Details:
Languages:  eng     Pagination:  397-406     Citation Subset:  IM    
Affiliation:
Princess Mary Laboratory, Auckland Hospital, New Zealand.
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MeSH Terms
Descriptor/Qualifier:
Down Syndrome / complications*,  pathology
Female
Fibrosis
Humans
Infant, Newborn
Infant, Newborn, Diseases / pathology
Leukemia / etiology,  pathology
Leukemoid Reaction / complications*,  pathology
Liver Cirrhosis / etiology,  pathology*
Male
Megakaryocytes / pathology*
Pancreas / pathology*
Pancreatic Diseases / etiology,  pathology

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine


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