[Hypereosinophilia syndrome. Apropos of 2 cases and literature review].
Idiopathic hypereosinophilic syndrome is characterized by prolonged eosinophilia of undetected cause and multiple organ system involvement (lung, kidney, nervous system, skin,...). Nevertheless, the prognosis has been correlated with heart involvement, which usually results in a restrictive cardiomyopathy with apical obliteration by fibrosis, mural thrombi and mitral and tricuspid regurgitation. This disease has a wide range of severity: some patients suffer from a real myeloproliferative syndrome and may develop blastic transformation while others present only skin involvement or are asymptomatic. Corticosteroids and hydroxyurea are both effective treatments. Interferon alpha seems to be active for the myeloproliferative form of the disease. Cytotoxic activity of activated eosinophil granular proteins may play an important role in tissue damage. The cause of eosinophilic proliferation (primitive malignant proliferation or resulting from a T lymphocyte stimulus) and activation remains uncertain.
Straetmans
N
N
Service d'Hématologie, Cliniques Universitaires St-Luc, Bruxelles, Belgique.
Ferrant
A
A
Martiat
P
P
Sokal
G
G
Michaux
J L
JL
D000368:Aged; D002313:Cardiomyopathy, Restrictive / Q000150:complications; D004802:Eosinophilia / Q000150:complications* / Q000628:therapy; D006801:Humans; D006918:Hydroxyurea / Q000627:therapeutic use; D016898:Interferon-alpha / Q000627:therapeutic use; D008297:Male; D008875:Middle Aged; D009196:Myeloproliferative Disorders / Q000150:complications; D011379:Prognosis; D013163:Splenomegaly / Q000150:complications; D013577:Syndrome
D016898:Interferon-alpha; D006918:Hydroxyurea
Le syndrome hypereosinophilique (SHE). A propos de deux cas et revue de la littérature.