Document Detail

Idiopathic Myelofibrosis in Children: Primary Myelofibrosis, Essential Thrombocythemia, or Transient Process?
MedLine Citation:
PMID:  23799525     Owner:  NLM     Status:  Publisher    
We report 3 pediatric cases of primary/idiopathic myelofibrosis (PMF/IMF). Two cases exhibited clinical courses not typically observed in adult patients in whom this process is much more common. One of these cases exhibited spontaneous clinical and bone marrow resolution, whereas the other case achieved near resolution of myelofibrosis in response to cytoreductive therapy alone. However, the third case of IMF that met diagnostic criteria for essential thrombocythemia with a JAK2V617F mutation had central venous thrombosis that resulted in blindness. PMF/IMF, a rare finding in children, does not seem to portend the same level of risk as seen in adults with the same process, thus less aggressive management may be appropriate. However, delayed diagnosis of mutation-associated PMF or essential thrombocythemia can lead to devastating consequences. We review the literature and discuss the complexities surrounding diagnosis, risk stratification, and management of pediatric PMF/IMF.
Jeremy S Slone; Megan C Smith; Adam C Seegmiller; Robert F Sidonio; Elizabeth Yang
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Publication Detail:
Type:  JOURNAL ARTICLE     Date:  2013-6-24
Journal Detail:
Title:  Journal of pediatric hematology/oncology     Volume:  -     ISSN:  1536-3678     ISO Abbreviation:  J. Pediatr. Hematol. Oncol.     Publication Date:  2013 Jun 
Date Detail:
Created Date:  2013-6-26     Completed Date:  -     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  9505928     Medline TA:  J Pediatr Hematol Oncol     Country:  -    
Other Details:
Languages:  ENG     Pagination:  -     Citation Subset:  -    
*Division of Pediatric Hematology-Oncology †Department of Pathology, Microbiology, and Immunology, Vanderbilt University, Nashville, TN.
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