Clinical characteristics of patients with chronic eosinophilic leukaemia (CEL) harbouring FIP1L1‐PDGFRA fusion transcript—results of Polish multicentre study

• DOI: 10.1002/hon.919
• Publication Date: 2009-09-03T07:40:59Z
• Authors: Grzegorz Helbig; Andrzej Moskwa; Marek Hus; Jarosław Piszcz; Alina Swiderska; Alina Urbanowicz; Małgorzata Całbecka; Justyna Gajkowska; Ilona Seferyńska; Magdalena Hałasz; Dariusz Woszczyk; Miroslaw Markiewicz; Sławomira Krzemień
• Keywords: Hypereosinophilia; Hypereosinophilic Syndrome; PDGFRA; Imatinib Mesylate

Abstract 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 to estimate the frequency transcript among unexplained, long‐term hypereosinophilia exceeding 1.5 × 10 9 /L and analyse clinical serological features in F/P+ CEL population. The chimeric protein was detectable 16 (14 males 2 females) out examined HES (20%) by RT‐PCR. Two suffered from cough at diagnosis. Three (18%) had no organ involvements, 5‐one affected remaining eight cases—at least two. Eosinophilic damage/dysfunction identified splenomegaly majority studied patients. We compared between ( n = 16) 61) significantly increased WBC absolute eosinophil count (AEC) diagnosis p 0.008 0.02), whereas platelet decreased population 0.03). Serum B12 tryptase levels were 0.002 0.004) when serum IL‐5 latter group 0.01). Male gender occurred more frequent 0.0007, respectively). Additionally, F/P− 8). group, older, lower AEC higher count. conclusion, significant symptoms infrequent present remains most common involvement expressing F/P transcript. Our study confirmed remission on imatinib patient Copyright © 2009 John Wiley & Sons, Ltd.