PMID- 16768285
OWN - NLM
STAT- MEDLINE
DCOM- 20060727
LR  - 20161124
IS  - 0028-2162 (Print)
IS  - 0028-2162 (Linking)
VI  - 150
IP  - 21
DP  - 2006 May 27
TI  - [Two patients with hypereosinophilic syndrome].
PG  - 1193-7
AB  - Persistent eosinophilia was diagnosed in a 19-year-old woman with general 
      malaise, dyspnoea attacks, coughing and episodes of angioedema and associated 
      swallowing problems, and in a 21-year-old man with visual problems, dyspnoea, 
      fatigue, reduced appetite, weight loss and gastrointestinal problems. Both had 
      hypereosinophilic syndrome (a rare disease) with organ damage. In both patients, 
      fluorescence-in-situ-hybridisation (FISH) was negative for the fusion gene 
      FIP1L1-PDGFRA (FIPI-like-1-platelet-derived growth factor receptor alpha). The 
      female patient's disease did not respond to either oral corticosteroids or 
      imatinib, but did respond to hydroxycarbamide. The male patient successively 
      received prednisone, interferon alpha and hydroxycarbamide. His eosinophilia 
      progressed nonetheless, but responded partially to imatinib. In addition, the 
      patient underwent an allogenic non-myeloblative stem cell transplantation from 
      his HLA-identical sister. In patients with persistent eosinophilia accompanied by 
      organ damage or organ dysfunction, hypereosinophilic syndrome can be diagnosed 
      providing all secondary causes of the eosinophilia have been ruled out. 
      Complementary investigations should include cytogenetic and clonal analysis to 
      rule out haemopoietic malignancy. Prednisone, hydroxycarbamide, interferon alpha 
      and the promising imatinib are all treatment options.
FAU - van Ruth, S
AU  - van Ruth S
AD  - Universitair Medisch Centrum Utrecht. s.ruth@umcutrecht.nl
FAU - Lokhorst, H M
AU  - Lokhorst HM
FAU - Bruijnzeel-Koomen, C A F M
AU  - Bruijnzeel-Koomen CA
LA  - dut
PT  - Case Reports
PT  - Journal Article
TT  - Twee patienten met hypereosinofiel syndroom.
PL  - Netherlands
TA  - Ned Tijdschr Geneeskd
JT  - Nederlands tijdschrift voor geneeskunde
JID - 0400770
RN  - 0 (Benzamides)
RN  - 0 (Enzyme Inhibitors)
RN  - 0 (Interferon-alpha)
RN  - 0 (Oncogene Proteins, Fusion)
RN  - 0 (Piperazines)
RN  - 0 (Pyrimidines)
RN  - 0 (mRNA Cleavage and Polyadenylation Factors)
RN  - 8A1O1M485B (Imatinib Mesylate)
RN  - EC 2.7.10.1 (FIP1L1-PDGFRA fusion protein, human)
RN  - EC 2.7.10.1 (Receptor, Platelet-Derived Growth Factor alpha)
RN  - VB0R961HZT (Prednisone)
RN  - X6Q56QN5QC (Hydroxyurea)
SB  - IM
CIN - Ned Tijdschr Geneeskd. 2006 May 27;150(21):1188-92. PMID: 16768284
MH  - Adult
MH  - Benzamides
MH  - Diagnosis, Differential
MH  - Enzyme Inhibitors/*therapeutic use
MH  - Female
MH  - Humans
MH  - Hydroxyurea/therapeutic use
MH  - *Hypereosinophilic Syndrome/diagnosis/genetics/therapy
MH  - Imatinib Mesylate
MH  - In Situ Hybridization, Fluorescence
MH  - Interferon-alpha/therapeutic use
MH  - Male
MH  - Oncogene Proteins, Fusion/*genetics
MH  - Piperazines/therapeutic use
MH  - Prednisone/therapeutic use
MH  - Pyrimidines/therapeutic use
MH  - Receptor, Platelet-Derived Growth Factor alpha/*genetics
MH  - Stem Cell Transplantation
MH  - mRNA Cleavage and Polyadenylation Factors/*genetics
EDAT- 2006/06/14 09:00
MHDA- 2006/07/28 09:00
CRDT- 2006/06/14 09:00
PHST- 2006/06/14 09:00 [pubmed]
PHST- 2006/07/28 09:00 [medline]
PHST- 2006/06/14 09:00 [entrez]
PST - ppublish
SO  - Ned Tijdschr Geneeskd. 2006 May 27;150(21):1193-7.