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052898 Eosinophilia The New England Journal of Medicine Review Articles Mechanisms of Disease a review of the patient’s history, which may reveal wheezing, rhinitis, or eczema (indicating atopic causes); travel to areas where helminthic infections F RANKLIN H. EPSTEIN, M.D., Editor (e.g., schistosomiasis) are endemic; the presence of a pet dog (indicating possible infection with Toxo- cara canis); symptoms of cancer; or drug ingestion EOSINOPHILIA (indicating a possible hypersensitivity reaction). Eo- sinophilia caused by drugs is usually benign but can sometimes be accompanied by tissue damage, as in MARC E. ROTHENBERG, M.D., PH.D. hypersensitivity pneumonitis. In most cases, the eo- sinophilia resolves once the drug is withdrawn, but in some cases, such as the eosinophilia–myalgia syn- MARKED accumulation of eosinophils oc- drome due to the ingestion of contaminated tryp- curs in several important disorders, such as tophan, the disease can persist despite withdrawal of allergic diseases, parasitic infections, and can- the drug.6 Abnormal morphologic features of eosin- A1 cer. The level of eosinophils in the body is normally ophils, an increase in immature cells in the bone tightly regulated. In normal subjects, eosinophils ac- marrow or blood, or a karyotypic abnormality indi- count for only a small minority of peripheral-blood cates the presence of eosinophilic leukemia. An ac- leukocytes, and their presence in tissues is primarily cumulation of eosinophils that is limited to specific limited to the gastrointestinal mucosa.2 In certain organs is characteristic of particular diseases, such as disease states, however, eosinophils can selectively ac- eosinophilic cellulitis (Well’s syndrome), eosinophil- cumulate in the peripheral blood or any tissue in the ic pneumonias (e.g., Löffler’s syndrome), and eosin- body. Any perturbation that results in eosinophilia, ophilic fasciitis (Shulman’s syndrome). The associa- defined here as an abnormal accumulation of eosin- tion of eosinophilia with vasculitis, neuropathy, and ophils in blood or tissue, can have profound clinical a history of asthma indicates the presence of the effects. Eosinophilia may be harmful, because of the Churg–Strauss syndrome. In the absence of an iden- proinflammatory effects of eosinophils,3 or it may be tifiable cause of moderate-to-severe eosinophilia and helpful, because of the antiparasitic effects of these in the presence of end-organ involvement, the diag- cells.4 This article focuses on recent advances in our nosis of the idiopathic hypereosinophilic syndrome understanding of the accumulation of eosinophils, as should be considered. This disorder occurs predom- well as treatment approaches and the development of inantly in men and is usually a progressive, fatal new therapeutic agents. disease in the absence of effective medical manage- ment.7 CLINICAL ASPECTS OF EOSINOPHILIA Diagnostic studies that should be performed in Eosinophils normally account for only 1 to 3 per- patients with moderate-to-severe eosinophilia and cent of peripheral-blood leukocytes, and the upper should be considered in patients with persistent mild limit of the normal range is 350 cells per cubic mil- eosinophilia include morphologic examination of a limeter of blood. Eosinophilia occurs in a variety of blood smear, urinalysis, and serial stool examinations disorders (Table 1)5 and is arbitrarily classified as for ova and parasites.5,8,9 Parasitic infections that mild (351 to 1500 cells per cubic millimeter), mod- cause eosinophilia are usually limited to helminthic erate (>1500 to 5000 cells per cubic millimeter), or parasites, with the exception of two enteric protozo- severe (>5000 cells per cubic millimeter). The most ans, Isospora belli and Dientamoeba fragilis. Strongy- common cause of eosinophilia worldwide is helmin- loides stercoralis infection is important to diagnose, thic infections, and the most common cause in in- because it can cause disseminated fatal disease in im- dustrialized nations is atopic disease. munosuppressed patients; its detection often requires The differential diagnosis of eosinophilia requires serologic testing. Other infections to rule out are those with filarial parasites, trichinosis, and T. canis infection. Bone marrow and chromosomal analysis (to detect hematologic cancer) and a tissue biopsy may be indicated. From the Department of Pediatrics, Children’s Hospital Medical Center, Moderate-to-severe eosinophilia may persist in the University of Cincinnati, 3333 Burnet Ave., Cincinnati, OH 45229, where reprint requests should be addressed to Dr. Rothenberg. absence of an identifiable cause or end-organ in- ©1998, Massachusetts Medical Society. volvement. Patients with persistent, apparently be- 1592 · May 28, 1998 MECHANISMS OF DISEASE ophil lineage.10 Three cytokines — interleukin-3, in- TABLE 1. DISEASES ASSOCIATED WITH EOSINOPHILIA. terleukin-5, and granulocyte–macrophage colony- stimulating factor (GM-CSF) — are particularly im- portant in regulating the development of eosinophils. TYPE OF DISEASE EOSINOPHILIA EXAMPLES OF CAUSES These cytokines are encoded by closely linked genes PERIPHERAL BLOOD TISSUE on chromosome 5q31 and bind to receptors that have a common beta chain and different alpha chains. Infectious Present Present or Infections with especially absent invasive helminths Of the three cytokines, interleukin-5 (also known Respiratory Present or Present Eosinophilic pneumonitis, as eosinophil-differentiation factor) is the most spe- absent asthma cific for the eosinophil lineage and is responsible for Gastrointestinal Present or Present Inflammatory bowel dis- selective differentiation of eosinophils.11 Interleukin- absent ease, eosinophilic gastro- enteritis, allergic colitis 5 also stimulates the release of eosinophils from bone 12 Allergic Present or Present Allergic rhinoconjunctivitis, marrow into the peripheral circulation. The critical absent asthma, eczema role of interleukin-5 in the production of eosino- Systemic Present Present Idiopathic hypereosinophil- phils is best demonstrated by genetic manipulation ic syndrome, vasculitis in mice. Overproduction of interleukin-5 in trans- Iatrogenic Present Present or Drug reaction, cytokine in- 11 absent fusions (e.g. granulo- genic mice results in profound eosinophilia, and cyte–macrophage colo- deletion of the interleukin-5 gene causes a marked ny-stimulating factor) reduction of eosinophils in the blood and lungs after Malignant Present or Present or Lymphoma, colonic carci- 13 absent absent noma an allergen challenge. The overproduction of one or more of the three cytokines occurs in humans with eosinophilia. Diseases involving eosinophilia without increases in other blood-cell lineages are usually accompanied by an overproduction of inter- leukin-5.11 The mechanisms of cytokine overproduc- nign eosinophilia usually do not need therapy, and tion may involve a response of T-helper lymphocytes spontaneous resolution generally occurs within sev- of the Th2 type in patients with allergic conditions eral years. However, such patients should have peri- (see below) or parasitic diseases,14,15 the malignant odic clinical and echocardiographic examinations to expansion of T-cell clones that produce interleukin- detect eosinophil-mediated cardiac damage, which 5 in some patients with lymphoma,16 or the activa- can occur insidiously at any time and which may not tion of gene transcription due to a chromosomal be correlated with the severity of eosinophilia.8 Al- translocation in some patients with leukemia.17,18 though no therapy is indicated, it is often helpful to determine whether eosinophilia will resolve with a Adhesion and Migration short trial of prednisone (1 mg per kilogram of body The migration of eosinophils from the circulation weight per day for three to five days). If so, this may into tissues involves a stepwise interaction between be a suitable option in the event of future deteriora- eosinophils and endothelial cells.19 The steps are me- tion. Glucocorticoid-resistant cases may respond to diated by adhesion molecules on endothelial cells drugs that are reserved for therapy rather than trials. and counter-ligands on eosinophils and are followed The therapeutic approach to patients who have eo- by the passage of eosinophils between endothelial sinophilia with an identifiable cause is discussed below. cells (Fig. 1). Although the different types of leuko- cytes migrate into tissues in similar ways, their mi- PHYSIOLOGIC FEATURES gration is mediated by different molecules. Eosino- OF EOSINOPHILS phils initially adhere to the endothelium by means of Eosinophilia occurs as a result of four processes three selectins (adhesion molecules on endothelial (Fig. 1): differentiation of progenitor cells and pro- cells) and their corresponding ligands. The rolling of liferation of eosinophils in bone marrow; interac- circulating eosinophils on the endothelium is me- tions between eosinophils and endothelial cells that diated primarily by P-selectin, whereas neutrophil involve rolling, adhesion, and migration of eosino- rolling is mediated primarily by E-selectin.20,21 After phils; chemoattraction directing eosinophils to a cellular activation (e.g., by exposure to chemoat- specific location; and activation and destruction of tractants such as platelet-activating factor or eotax- eosinophils. in), eosinophils adhere firmly to the endothelium through adhesion molecules of the integrin family. Proliferation These include the CD18 family (b2 integrins) and Eosinophils are produced in bone marrow from
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