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Peripheral Eosinophilia and Diagnosis of Hypereosinophilic Syndrome

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Peripheral Eosinophilia and Diagnosis of Hypereosinophilic Syndrome CE Update Received 11.14.05 | Revisions Received 2.5.06 | Accepted 2.7.06 Peripheral Eosinophilia and Diagnosis of Hypereosinophilic Syndrome Kenneth L. Sims, MD Downloaded from https://academic.oup.com/labmed/article/37/7/440/2504503 by guest on 01 October 2021 (American University of the Caribbean School of Medicine, Sint Maarten, Netherlands Antilles) DOI: 10.1309/RK234QMGLX0JPG0E Abstract classification of peripheral blood eosinophilia the “hypereosinophilic syndromes” based on This article first reviews the mechanisms, disorders as well as the relative frequency of the their molecular biology and their relationship to frequency, and diagnostic criteria for peripheral various disorders. The remainder of the article clonal disorders involving esosinophils (eg, blood eosinophilia. Subsequent emphasis is on highlights recent advances in classification of chronic esosinophic leukemia). The reader should better understand first the mechanisms, causes, and Immunology exam 40602 questions and corresponding answer form are classification of peripheral blood esosinophilia. After achieving this basic located after the CE Update section on p. 443. understanding of peripheral blood eosinophilia, the reader can then understand the diagnosis and clinical relationship of eosinophilia to the uncommon clonal and highly aggressive disorders known collectively as hypereosinophilic syndrome (HES). Paul Ehrlich first observed the eosinophil (terming it an in blood is 18 hours.7 Eosinophils quickly exit the blood via the acidophil) in 1879 because of the affinity of its granules for post-capillary venule in response to action of various chemokines eosin as part of his overall observations with staining peripheral (eotaxin-1 and eotaxin-2 are relatively specific for eosinophils).6 blood constituents.1 In Ehrlich’s textbook in 1900,2 the blood The intravascular compartment is by far the smallest of the 3 and tissue appearance of the eosinophil using Romanowsky stain compartments with only 1 peripheral blood eosinophil per 100 is depicted as the bilobed circulating granulocyte with pink tissue eosinophils.5 The ratio of tissue eosinophils to blood granules, which is now well known to all medical professionals eosinophils markedly increases in disease states such as asthma in and investigators. The clinical association between peripheral the bronchopulmonary mucosa where eosinophils are not nor- blood eosinophilia and parasitic (helminthiasis) disease, allergic mally present in significant numbers.8 The primary tissue loca- conditions, and various malignancies was noted in the first half tion of eosinophils under non-disease conditions is in the of the 20th century. However, the definitive physiologic func- gastrointestinal tract and gall bladder where they are localized tions of the eosinophil remain poorly understood other than its almost exclusively with the lamina propria of the mucosa.3,9 essential role in response to parasitic infections.”3 In the last 15 Eosinophil survival in tissue is highly dependent on cytokines years, there has been renewed interest in the eosinophil and (interleukins IL-3 and IL-5) that inhibit activation and subse- both its physiologic functions and its destructive role in various quent apoptosis. Under normal non-disease conditions, disease states, particularly those characterized collectively as the eosinophils may survive in tissue for 12 to 14 days.5,6 “hypereosinophilic syndromes.”4 Measurement—Peripheral Blood Eosinophilia Normal Distribution and Dynamics of the With the advent of modern automated analyzers which Eosinophil sample 10,000 cells or greater per determination and the 5- The eosinophil is fundamentally a tissue-based cell with 3 part differential in automated cell counters, the measurement major compartments; bone marrow, blood, and tissue.5 of eosinophils is highly precise.10 Measurements of eosinophils Eosinophils are derived from hematopoietic stem cells that dif- are expressed either as % of the total white cell count (“differ- ferentiate via the basophil-eosinophil lineage, and their produc- ential count”)—common reference range 0% to 5% total gran- tion in the bone marrow is tightly regulated by various cytokines ulocytes or as an absolute eosinophil count expressed as (value) with the most important being interleukin-5.6 Mature x 109cells/L blood (multiply % eosinophils by total granulo- eosinophils are neither accumulated nor stored or reserved in cyte count). The preferred measurement is the absolute significant numbers in the bone marrow, but are released into eosinophil count that has an upper limit of the reference range the blood as they are produced. Their average survival (half-life) varying from 0.5 to 0.7 x 109/L, depending on testing location 440 LABMEDICINE ᭿ Volume 37 Number 7 ᭿ July 2006 labmedicine.com CE Update and instrumentation. The degree of peripheral eosinophilia has Table 1_Secondary (Reactive) Causes of Peripheral arbitrarily been divided into 3 categories11,12: Eosinophilia* Mild 0.5 (0.7) – 1.4 x 109/L Moderate 1.5 – 4.9 x 109/L Allergic Diseases Immunologic Diseases Severe >5.0 x 109/L Asthma (extrinsic and intrinsic) Churg-Strauss syndrome Allergic rhinitis Eosinophilic myalgia syndrome Chronic sinusitis Wegener’s granulomatosis Etiology of Elevated Peripheral Blood Drug-induced hypersensitivity Polyarteritis nodosa Atopic dermatitis Serum sickness Eosinophil Counts Chronic urticaria Rheumatoid arthritis Peripheral blood eosinophilia is quite uncommon in the 13 Parasitic Diseases Neoplasms United States. Brigden and Graydon, in a study in the Pacific Helminthes Hodgkin lymphoma Northwest, examined 193,300 hematology profiles and found Schistosoma T-cell lymphomas 225 patients (0.1% of total patients sampled) that had >0.7 x Tapeworm (all types) Acute lymphoblastic leukemia/lymphoma 109/L peripheral eosinophil counts. Seventy percent were mild, Filiariasis Mastocytosis Liver and lung flukes Downloaded from https://academic.oup.com/labmed/article/37/7/440/2504503 by guest on 01 October 2021 25% to 28% were moderate, and fewer than 5% were severe. All Pinworm (Enterobius vermicularis) patients with eosinophilia were studied to determine etiology. Trichinosis Allergic disease (asthma, allergic rhinitis, eczema, and drug al- Viscera larva migrans lergy) accounted for 56%, “unknown” for 36%, and parasitic *Table assembled from information from Brito-Babapulle,11 Tefferi,12 Whitty,14 and Moore.15 disease for 2% of the cases of eosinophilia. In this entire group, The table is not comprehensive but representative of the major disorders in each group of there were no examples of hypereosinophilic syndrome as de- diseases associated with eosinophilia. fined in a subsequent section. The most common cause of eosinophila worldwide is para- sitic disease. In travelers returning to the United States or Eng- Table 2_WHO Classification Chronic Eosinophilic land from a prolonged stay in endemic areas for various types of Leukemia (CEL) and Hypereosinophilic Syndrome (HES)* parasitic disease, 20% to 25% of these patients had eosinophilia secondary to parasitic disease.14,15 1. Exclude all causes of reactive eosinophilia 2o to secondary causes such as allergy or parasitic diseases. 2. Exclude all neoplastic disorders with secondary reactive eosinophilia: Classification of Peripheral Blood Eosinophilia T-cell lymphomas including mycosis fungoides, Sczary syndrome Hodgkin Lymphoma Familial versus Acquired: Virtually all eosinophilia is Acute lymphoblastic leukemia/lymphoma acquired. The entity, familial eosinophilia, represents “an Mastocytosis extremely rare autosomal dominant disorder.”12 3. Exclude other neoplastic disorders in which eosinophils are part of the Acquired: Secondary versus Primary11,12,20,21: The term neoplastic clone secondary is often used interchangeably with “reactive” to indi- CML (Philadelphia chromosome or BCR-ABL-positive) AML including those with inv(16), t(16;16)(p13:q22) cate eosinophilia caused by another clinical condition or disease. Other myeloproliferative diseases (polycythemia vera, essential Secondary (reactive) eosinophilia accounts for the vast majority thrombocytosis) of all instances of peripheral eosinophilia (>99%). The basic Myelodysplastic syndromes groups of diseases as well as a sampling of the common specific 4. Exclude T-cell population with aberrant phenotype and abnormal cytokine diseases in the groups are summarized in Table 1. What these population secondary causes have in common is that they (1) usually give 5. If there is no demonstrable disease that could cause eosinophilia, not rise to mild or moderate peripheral eosinophilia, (2) represent a abnormal T-cell population and no evidence of a clonal myeloid disorder, physiologic response by bone marrow to increased tissue diagnosis HES demand for eosinophils, and (3) eosinophilia ceases or declines 6. If requirements 1-4 have been met, and if the myeloid elements demonstrate a clonal cytogenetic abnormality or clonality is shown by other means, or if with cessation of the causative disease process. blasts are present in the peripheral blood (>2%) or marrow (>5% but less 11,12,20,21 Primary : The cause of the peripheral eosinophilia than 19%), diagnosis CEL is either a clonal (DNA-related) process involving progenitor *Modified from Gotlib21 cells involved in eosinophil production (regulation) or no cause or genetic (clonal) abnormality is identified. This second group is designated “idiopathic hypereosinophilic syndrome.” Clinical findings associated with this primary group are
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