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A clinicopathologic correlation of the idiopathic hypereosinophilic
syndrome. II. Clinical manifestations
RT Schooley, MA Flaum, HR Gralnick and AS Fauci
The idiopathic hypereosinophilic syndrome, a disorder characterized by
peripheral blood and bone marrow eosinophilia associated with single or
multiple organ system dysfunction attributable to tissue invasion by
eosinophils has, in the past, been associated with an extremely poor
prognosis. Recently, we reported the favorable impact of a therapeutic
protocol consisting of prednisone and/or hydroxyurea on the morbidity and
mortality of this syndrome. We have reviewed the clinical and hematologic
features upon admission and the subsequent clinical courses of 32 patients
with this disease referred to the NIH between 1965 and 1979 in an effort to
determine which features suggest a more rapidly progressive course. A
grading system based on 22 clinical features involving the 8 organ systems
commonly affected by the illness was devised. The disease followed a more
aggressive course in patients with evidence of cardiac or neurologic
dysfunction at the time of initial NIH evaluation. Although splenomegaly,
in and of itself, caused little morbidity, splenic enlargement at
presentation appeared to be a predictor of a more aggressive course. The
clinical grading system accurately predicted which patients would require
no specific antihypereosinophilic therapy, which patients would respond
adequately to corticosteroids, and which patients would require therapy
with cytotoxic agents. It is proposed that this clinical grading system,
and the hematologic grading system outlined in the accompanying report be
used as aids in the selection of initial therapy in this group of patients.
Volume 58,
Issue 5,
pp. 1021-1026,
11/01/1981
Copyright © 1981 by The American Society of Hematology
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