Document Detail

Isolated central nervous system relapse in an adolescent with acute myelomonocytic leukemia, Charcot Marie Tooth syndrome, and paraneoplastic autoantibody.
MedLine Citation:
PMID:  20724950     Owner:  NLM     Status:  MEDLINE    
A 17-year-old boy, with acute myelomonocytic leukemia and inversion 16(p13q22) developed polyneuropathy and isolated central nervous system relapse. Scoliosis and high-arched feet suggested a diagnosis of Charcot Marie Tooth (CMT) syndrome and genetic testing confirmed duplication at the PMP22 locus at chromosome 17p11.12. No mutation was found in another CMT gene, the CMT C1 LITAF locus at 16p13.2, to suggest that this association is anything more than chance. Titres to VGKC, a paraneoplastic autoantibody, were elevated, suggesting an additional mechanism for the polyneuropathy. This case extends the clinical spectrum of cancer with CMT, and of paraneoplastic disorders.
Julie Blatt; Robert Greenwood; Spencer Weig; Kathleen Rao; George D Fedoriw; Georgette Dent
Publication Detail:
Type:  Case Reports; Journal Article    
Journal Detail:
Title:  Journal of pediatric hematology/oncology     Volume:  32     ISSN:  1536-3678     ISO Abbreviation:  J. Pediatr. Hematol. Oncol.     Publication Date:  2010 Oct 
Date Detail:
Created Date:  2010-10-06     Completed Date:  2010-10-25     Revised Date:  2011-10-06    
Medline Journal Info:
Nlm Unique ID:  9505928     Medline TA:  J Pediatr Hematol Oncol     Country:  United States    
Other Details:
Languages:  eng     Pagination:  571-3     Citation Subset:  IM    
Division of Pediatric Hematology Oncology, The University of North Carolina at Chapel Hill, Chapel Hill, NC, USA.
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MeSH Terms
Autoantibodies / blood*
Charcot-Marie-Tooth Disease / complications,  genetics,  immunology*
Leukemia, Myelomonocytic, Acute / complications,  immunology*
Myelin Proteins / genetics
Paraneoplastic Syndromes, Nervous System / complications,  immunology*,  pathology
Polyneuropathies / complications,  immunology
Reg. No./Substance:
0/Autoantibodies; 0/Myelin Proteins; 0/PMP22 protein, human

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