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Chronic Childhood Idiopathic Myelofibrosis in Down's Syndrome: A Case Report.
MedLine Citation:
PMID:  23997458     Owner:  NLM     Status:  PubMed-not-MEDLINE    
Abstract/OtherAbstract:
Idiopathic myelofibrosis a disease of elderly is rarely seen in children. A case of chronic idiopathic myelofibrosis in an 8 year old boy with Down's syndrome is reported here, who presented with progressive pallor and hepatosplenomegaly. Peripheral blood examination revealed pancytopenia, macrocytic anemia and tear drop cells. No blasts were found. Bone marrow aspirate yielded a dry tap and trephine biopsy showed marrow fibrosis with osteosclerosis. Focally megakaryocytes were increased in number with atypical morphology. No blasts were seen. Review of literature revealed 47 reported cases of childhood idiopathic myelofibrosis. Six cases were associated with Down's syndrome and only 3 of them had features of chronic idiopathic myelofibrosis without evidence of acute megakaryoblastic leukaemia.
Authors:
Smita Singh; Sunita Sharma; Narender Tejwani; Jagdish Chandra
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Publication Detail:
Type:  Journal Article     Date:  2011-10-18
Journal Detail:
Title:  Indian journal of hematology & blood transfusion : an official journal of Indian Society of Hematology and Blood Transfusion     Volume:  28     ISSN:  0971-4502     ISO Abbreviation:  Indian J Hematol Blood Transfus     Publication Date:  2012 Sep 
Date Detail:
Created Date:  2013-09-02     Completed Date:  2013-09-02     Revised Date:  2013-09-04    
Medline Journal Info:
Nlm Unique ID:  9425818     Medline TA:  Indian J Hematol Blood Transfus     Country:  India    
Other Details:
Languages:  eng     Pagination:  184-6     Citation Subset:  -    
Affiliation:
Department of Pathology, Lady Hardinge Medical College & Associated Kalawati Saran Children Hospital, A-207, Narwana Appartments, Plot no. 89, I.P. Extension, New Delhi, 110092 India.
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