Document Detail

Childhood idiopathic myelofibrosis: a case report and review of literature.
MedLine Citation:
PMID:  20514856     Owner:  NLM     Status:  MEDLINE    
Idiopathic myelofibrosis occurs predominantly in older adults. It is very rarely seen in children. We describe a 3-year-old girl with Down's syndrome who presented with recurrent chest infections associated with anaemia and easy bruising. There was mild hepatosplenomegaly. Full blood picture revealed pancytopaenia with leucoerythroblastosis with absence of circulating blast cells. Repeated attempts at bone marrow aspiration and trephine biopsy were unsuccessful. A trephine biopsy from the tibia showed depressed myelopoiesis and erythropoiesis, megakaryocytes with atypical morphology and increased bone marrow reticulin fibres, findings compatible with idiopathic myelofibrosis. She was treated symptomatically as she was clinically stable. Review of the English literature online yielded 46 reported cases of childhood idiopathic myelofibrosis with variable outcome from spontaneous remission to an indolent course with shortened survival. 6 cases evolved to another malignancy. 5 cases were associated with Down's syndrome.
Z Noor-Fadzilah; C F Leong; M N Sabariah; S K Cheong
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Publication Detail:
Type:  Case Reports; Journal Article    
Journal Detail:
Title:  The Malaysian journal of pathology     Volume:  31     ISSN:  0126-8635     ISO Abbreviation:  Malays J Pathol     Publication Date:  2009 Dec 
Date Detail:
Created Date:  2010-06-02     Completed Date:  2010-06-22     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  8101177     Medline TA:  Malays J Pathol     Country:  Malaysia    
Other Details:
Languages:  eng     Pagination:  129-32     Citation Subset:  IM    
Faculty of Medicine and Health Sciences, Islamic Science University of Malaysia, Kuala Lumpur, Malaysia.
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MeSH Terms
Bone Marrow / pathology*
Child, Preschool
Down Syndrome / complications,  pathology*
Megakaryocytes / pathology
Pancytopenia / etiology,  pathology
Primary Myelofibrosis / complications,  pathology*

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