A clinicopathologic correlation of the idiopathic hypereosinophilic syndrome. II. Clinical manifestations

Abstract

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.

Acute encephalopathy and idiopathic hypereosinophilic syndrome: clinical and MRI response to early steroid treatment

“…A treatment consisting of steroids allows a remission of the HES but the prognosis depends on the degree of involvement of vital organs, especially cardiac and neurological dysfunctions (Schooley et al, 1981). As shown with our case, an early steroid treatment in HES is essential to prevent the occurrence of irreversible damage.…”

Successful bone marrow transplantation for idiopathic hypereosinophilic syndrome

“…It is very difficult to determine whether HES is MPD or overproduction of eosinophilopoietic cytokines produced by the T-cell clone (Weller & Bubley, 1994). Some reports state that characteristics of HES/MPD are elevated B12 level, abnormal leucocyte alkaline phosphatase score, splenomegaly, cytogenetic abnormalities, myelofibrosis anaemia, myeloid dysplasia and basophilia (Fauci et al, 1982;Schooley et al, 1981). Our patient had the following clinical features: eosinophils with morphological abnormalities, appearance of immature myeloid cells and increase of basophils in PB, existence of myelofibrosis, no effect of methylprednisolone for eosinophilia, and eosinophilic infiltrations to skin and heart.…”

A Case of the Hypereosinophilic Syndrome Complicated by Malignant Lymphoma

“…The idiopathic hypereosinophilic syndrome is known to include eosinophilic leukemia, disseminated eosinophilic collagen disease (4), and endomyocardial disease and eosinophilia (1,2). As these several nomenclatures indicate, the disease displays multiple signs and symptoms depending on the organ systems involved.…”

“…A constant feature of this disease is both bone marrow and peripheral blood eosinophilia (1,2). Skin lesions occur in about 50% of the patients with this syndrome (5).…”

“…If malignant lymphoma had existed, the eosinophilia in the patient could have been clearly ascribed to malignant lymphoma (including Hodgkin's disease). On the other hand, lymph nodes and the liver could have been affected by DECD (2,4), or the lymph nodes might have proliferated in reaction to the skin eruptions the patient had had for ten years. Several biopsies of the skin lesions of this patient always showed characteristics common for DECD and never revealed any evidence oflymphoma cutis, Hodgkin's disease, or mycosis fungoides.…”