Document Detail


Clinical characteristics of patients with chronic eosinophilic leukaemia (CEL) harbouring FIP1L1-PDGFRA fusion transcript--results of Polish multicentre study.
MedLine Citation:
PMID:  19728396     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
A small subgroup of patients with hypereosinophilic syndrome (HES) demonstrates imatinib-sensitive fusion transcript-the FIP1L1-PDGFRA (F/P+). These cases are currently diagnosed as chronic eosinophilic leukaemia (CEL). In this paper, we screened 77 patients to estimate the frequency of FIP1L1-PDGFRA transcript among patients with unexplained, long-term hypereosinophilia exceeding 1.5 x 10(9)/L and to analyse the clinical and serological features in F/P+ CEL population. The FIP1L1-PDGFRA chimeric protein was detectable in 16 (14 males and 2 females) out of 77 examined HES patients (20%) by RT-PCR. Two patients suffered from cough at diagnosis. Three out of 16 (18%) patients had no organ involvements, in 5-one organ was affected and in the remaining eight cases-at least two. Eosinophilic organ damage/dysfunction identified splenomegaly in the majority of studied patients. We compared clinical and serological features between CEL F/P+ (n = 16) and HES (n = 61) patients. F/P+ cases had significantly increased WBC and absolute eosinophil count (AEC) at diagnosis (p = 0.008 and 0.02), whereas platelet count was decreased in this population (p = 0.03). Serum B12 and tryptase levels were increased (p = 0.002 and 0.004) in CEL F/P+ patients when compared to HES cases whereas serum IL-5 levels were significantly increased in the latter group (p = 0.01). Male gender and splenomegaly occurred more frequent in CEL F/P+ population (p = 0.002 and 0.0007, respectively). Additionally, patients with F/P+ CEL (n = 16) were compared with F/P- CEL (n = 8). The latter group, was significantly older, had lower AEC and higher platelet count. In conclusion, significant clinical symptoms are infrequent present and splenomegaly remains the most common organ involvement in patients with CEL expressing F/P fusion transcript. Our study confirmed the long-term remission on imatinib in this patient population.
Authors:
Grzegorz Helbig; Andrzej Moskwa; Marek Hus; Jaros?aw Piszcz; Alina Swiderska; Alina Urbanowicz; Ma?gorzata Ca?becka; Justyna Gajkowska; Ilona Sefery?ska; Magdalena Ha?asz; Dariusz Woszczyk; Miroslaw Markiewicz; S?awomira Krzemie?
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Publication Detail:
Type:  Journal Article; Multicenter Study; Research Support, Non-U.S. Gov't    
Journal Detail:
Title:  Hematological oncology     Volume:  28     ISSN:  1099-1069     ISO Abbreviation:  Hematol Oncol     Publication Date:  2010 Jun 
Date Detail:
Created Date:  2010-06-07     Completed Date:  2010-06-28     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  8307268     Medline TA:  Hematol Oncol     Country:  England    
Other Details:
Languages:  eng     Pagination:  93-7     Citation Subset:  IM    
Copyright Information:
(c) 2009 John Wiley & Sons, Ltd.
Affiliation:
Departament of Haematology and Bone Marrow Transplantation, Silesian Medical University, Katowice, Poland. ghelbig@o2.pl
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MeSH Terms
Descriptor/Qualifier:
Adult
Age Factors
Aged
Aged, 80 and over
Cough / etiology
Female
Hepatomegaly / etiology
Humans
Hypereosinophilic Syndrome / blood,  complications,  drug therapy,  epidemiology,  genetics*
Incidental Findings
Leukocyte Count
Male
Middle Aged
Oncogene Proteins, Fusion / genetics*
Piperazines / therapeutic use
Platelet Count
Poland / epidemiology
Protein Kinase Inhibitors / therapeutic use
Pyrimidines / therapeutic use
RNA, Messenger / genetics
RNA, Neoplasm / genetics
Receptor, Platelet-Derived Growth Factor alpha / genetics*
Splenomegaly / etiology
Young Adult
mRNA Cleavage and Polyadenylation Factors / genetics*
Chemical
Reg. No./Substance:
0/FIP1L1-PDGFRA fusion protein, human; 0/Oncogene Proteins, Fusion; 0/Piperazines; 0/Protein Kinase Inhibitors; 0/Pyrimidines; 0/RNA, Messenger; 0/RNA, Neoplasm; 0/mRNA Cleavage and Polyadenylation Factors; 152459-95-5/imatinib; EC 2.7.10.1/Receptor, Platelet-Derived Growth Factor alpha

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