Document Detail


Hemophagocytosis associated with leukemia: a striking association with juvenile myelomonocytic leukemia.
MedLine Citation:
PMID:  19798502     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
The aim of this study was to describe the characteristics and outcome in a group of pediatric patients with hematological malignancies who developed hemophagocytosis at diagnosis or during the disease course. Eight patients with hematological malignancy and associated hemophagocytosis were included. The initial diagnosis was juvenile myelomonocytic leukemia (JMML) in five, nonlymphoblastic leukemia (ANLL) in two, and T-cell lymphoma associated with myeloproliferative syndrome in one patient. Hemophagocytosis was concomitantly present at the time of diagnosis of the primary disease in four of the five patients with JMML and in the two patients with ANLL. Three had abnormalities related to chromosome 8 [(trisomy 8, monosomy 8, and t (8;13) (p11; p12)], and one had inversion 16. Multiple chromosomal losses were present in one patient, including both chromosomes 8 and 16. Bone marrow karyotyping revealed 46, XX; 47, XXX mosaicism in one patient. Two patients had PTPN11 mutation and one patient k-RAS mutation. The patients with JMML and neurofibromatosis (n = 2), the patient with lymphoma and t (8;13) positive AML, and a fourth patient with PTPN11 mutation did not remit and had unfavorable outcomes.
Authors:
Sule Unal; Mualla Cetin; Nuket Yurur Kutlay; Selin Aytac Elmas; Fatma Gumruk; Ajlan Tukun; Murat Tuncer; Aytemiz Gurgey
Publication Detail:
Type:  Journal Article; Research Support, Non-U.S. Gov't     Date:  2009-10-02
Journal Detail:
Title:  Annals of hematology     Volume:  89     ISSN:  1432-0584     ISO Abbreviation:  Ann. Hematol.     Publication Date:  2010 Apr 
Date Detail:
Created Date:  2010-02-19     Completed Date:  2010-03-09     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  9107334     Medline TA:  Ann Hematol     Country:  Germany    
Other Details:
Languages:  eng     Pagination:  359-64     Citation Subset:  IM    
Affiliation:
Department of Pediatrics, Division of Pediatric Hematology, Hacettepe University, Faculty of Medicine, 06100 Sihhiye, Ankara, Turkey.
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MeSH Terms
Descriptor/Qualifier:
Adolescent
Antineoplastic Agents / therapeutic use
Child
Child, Preschool
Chromosome Aberrations
Female
Genes, ras
Humans
Infant
Leukemia, Myeloid, Acute / drug therapy,  genetics,  pathology
Leukemia, Myelomonocytic, Juvenile / drug therapy,  genetics,  pathology*
Lymphoma, T-Cell / complications,  drug therapy,  genetics,  pathology
Male
Mutation
Myeloproliferative Disorders / complications,  drug therapy,  genetics,  pathology
Phagocytosis*
Protein Tyrosine Phosphatase, Non-Receptor Type 11 / genetics
Treatment Outcome
Chemical
Reg. No./Substance:
0/Antineoplastic Agents; EC 3.1.3.48/PTPN11 protein, human; EC 3.1.3.48/Protein Tyrosine Phosphatase, Non-Receptor Type 11

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