Naslov
Idiopathic hypereosinophilic syndrome (HES) in a 15 year old girl.

Autori
Jelušić, Marija ; Tambić Bukovac, Lana ; Malčić, Ivan

Vrsta, podvrsta i kategorija rada
Sažeci sa skupova, sažetak, znanstveni

Izvornik
Pediatric Rheumatology / - , 2008, 134-134

Skup
XV European Paediatric Rheumatology Congress (PRES)

Mjesto i datum
London, Ujedinjeno Kraljevstvo, 12.09.2008. - 15.09.2008

Vrsta sudjelovanja
Poster

Vrsta recenzije
Međunarodna recenzija

Ključne riječi
Idiopathic hypereosinophilic syndrome; child

Sažetak
The hypereosinophilic syndrome (HES) is a group of diseases characterized by persistent and marked blood eosinophilia, with end-organ involvement and no recognized secondary cause. We present a 15 year-old girl who was admitted to our Department in January 2008 with a four week history of headache, arthralgias, myalgias, sore troath and angioedema. Laboratory test revealed significant leucocytosis (76 x 10ex9/L with 88% eosinophils), thrombocytosis (758 x 10ex9/L), elevated ESR (82 mm/h) and IgE 348.3 (n.v.< 114 g/L), and hypergamaglobulinemia. Extensive allergologic, immunologic, infectious, and toxicological studies were negative. Bone marrow biopsy showed increased cellularity with increased granulopoiesis predominated by cells of the eosinophilic lineage, with a normal karyotype. Patient was negative for the FIP1L1-PDGFRA fusion kinase and BCR-ABL gene fusion by RT-PCR: Abdomen CT showed diffuse small intestine wall thickness, and cardiac echocardiogram showed thickness of the left ventricular wall and interventricular septum. The biopsy of myocardium and small intestine was planned, but in a mean time patient&#8217; s condition was worsened. She developed hypoproteinemia (46 g/L), generalised oedema, and diarrhoea. A diagnosis of idiopathic HES was made and methylprednisolone was introduced in the therapy. She had rapid response to methylprednisolone (within 12 hours), with normalisation of the blood counts, protein level and regression of oedema. Methylprednisolone was slowly tapered, and at present, HES is in complete clinical and laboratory remission. Conclusion: Although the HES is extremely rare in childhood, when patient is presented with significant leucocytosis with eosinophilia, in abscence of other causes of eosinophilia, such as parasite infestation, allergy, malignancy and collagen vascular disease, in differential diagnosis the HES has to be considered

Izvorni jezik
Engleski

Znanstvena područja
Kliničke medicinske znanosti

POVEZANOST RADA