Clinical characteristics of patients with chronic eosinophilic leukaemia (CEL) harbouring FIP1L1-PDGFRA fusion transcript--results of Polish multicentre study.
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-PDGFR...
| Publicado en: | Hematological Oncology Vol. 28; no. 2; pp. 93 - 98 |
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| Autores principales: | , , , , , , , , , , , , , , , , , , , |
| Formato: | research Journal Article |
| Publicado: |
Wiley-Blackwell
Jun2010
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| Acceso en línea: | Ver este registro en EBSCOhost |
| fields | @attributes: recordID: 1 pdfLink: plink: https://search.ebscohost.com/login.aspx?direct=true&db=ccm&AN=105022047&site=ehost-live header: @attributes: shortDbName: ccm uiTerm: 105022047 longDbName: CINAHL Complete uiTag: AN controlInfo: bkinfo: dissinfo: jinfo: jid: 02780232 O85 jtl: Hematological Oncology issn: 02780232 maglogo: Y pubinfo: dt: Jun2010 vid: 28 iid: 2 pid: 480 pub: Wiley-Blackwell place: Malden, Massachusetts artinfo: ui: 105022047 105022047 NLM19728396 2010678190 10.1002/hon.919 NLM19728396 105022047 ppf: 93 ppct: 5 formats: tig: atl: Clinical characteristics of patients with chronic eosinophilic leukaemia (CEL) harbouring FIP1L1-PDGFRA fusion transcript--results of Polish multicentre study. aug: au: Helbig G Moskwa A Hus M Piszcz J Swiderska A Urbanowicz A Calbecka M Gajkowska J Seferynska I Halasz M Woszczyk D Markiewicz M Krzemien S Helbig, Grzegorz Moskwa, Andrzej Hus, Marek Piszcz, Jarosław Swiderska, Alina Urbanowicz, Alina Całbecka, Małgorzata affil: Departament of Haematology and Bone Marrow Transplantation, Silesian Medical University, Katowice, Poland sug: subj: Eosinophilia Proteins Receptors, Cell Surface Adult Age Factors Aged Aged, 80 and Over Cough Etiology Female Hepatomegaly Etiology Human Eosinophilia Blood Eosinophilia Complications Eosinophilia Drug Therapy Eosinophilia Epidemiology Diagnosis Leukocyte Count Male Middle Age Heterocyclic Compounds Therapeutic Use Platelet Count Poland Enzyme Inhibitors Therapeutic Use RNA Splenomegaly Etiology Young Adult Adult: 19-44 years Aged: 65+ years Aged, 80 & over Middle Aged: 45-64 years Female Male ab: 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. pubtype: Academic Journal doctype: research Journal Article ougenre: Article language: English refInfo: holdings: @attributes: islocal: N |
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