Eosinophilia, Eosinophil-Associated Diseases, Chronic Eosinophil Leukemia, and the Hypereosinophilic Syndromes

Chapter 31 Eosinophilia, Eosinophil-Associated Diseases, Chronic Eosinophil Leukemia, and the Hypereosinophilic Syndromes

Marina Kremyanskaya, Steven J. Ackerman, Joseph H. Butterfield, John Mascarenhas, Ronald Hoffman

Treatment Algorithm for Patients With Severe Eosinophilia

Modified from Gotlib J: World Health Organization-defined eosinophilic disorders: 2011 update on diagnosis, risk stratification, and management. Am J Hematol 86:677, 2011.

Diagnosis and Workup of Hypereosinophilic Syndrome

Patients presenting with eosinophilia of unknown etiology should undergo a thorough clinical and laboratory evaluation consisting of the following:

• Complete/detailed history and physical examination

• Review of all medications (including herbal medications and nutritional supplements); withdrawal of all noncritical medications

• Complete blood count with total eosinophil count and review of peripheral blood smear

• Hepatic and renal function tests, urine analysis

• Vitamin B₁₂ level

• Serologic assays: erythrocyte sedimentation rate, rheumatoid factor, human immunodeficiency virus (HIV)

• Serum tryptase level

• Quantitation of total IgE level

• Cardiac troponin level

• Stool for ova, parasites ×3

• Serologic assays for Strongyloides, Trichinella, Toxocara, Entamoeba histolytica, Echinococcus, filariasis, and schistosomiasis

• Bone marrow aspirate and biopsy

• Cytogenetics, FISH, and molecular analyses (PDGFRA, PDGFRB, FGFR1 fusion genes, BCR-ABL, JAK2 V617F, KIT D816V, clonal TCR gene rearrangement)

• T-cell phenotyping by flow cytometry

• Chest radiograph; computed tomographic scan of chest, abdomen, and pelvis

• Electrocardiogram and echocardiogram

• Pulmonary function tests

Diagnostic Criteria for HES/CEL

1. Persistent eosinophilia of greater than 1500 eosinophils/mm³ for more than 6 months

2. Exclusion of other potential “reactive” etiologies for the eosinophilia including parasitic, allergic, or other causes, and

3. Presumptive signs and symptoms of organ system dysfunction or involvement that appears related to the eosinophilia or is of unknown cause in the clinical presentation

Figure 31-1 EOSINOPHIL AND EOSINOPHILIA.

A, Eosinophil in a standard peripheral blood smear (center) compared with a neutrophilic granulocyte (left). The eosinophil usually has a bilobed nucleus and heavily condensed chromatin usually with two lobes. The granules are considerably larger than those of a neutrophil and are spherical red-orange and refractile. They fill the cytoplasm and frequently overlie the nucleus. B, An eosinophilia is illustrated in a CSF specimen from a patient with hydrocephalus and a V-P shunt; apparently the eosinophilia is due to an allergic response.

Table 31-1 Diseases, Syndromes, and Conditions Commonly Associated With Peripheral Blood Eosinophilia and/or Tissue Eosinophilia

INFECTIOUS AGENTS

Parasitic Infections

Fungal Infections

Coccidioidomycosis

Cryptococcosis (CSF eosinophilia) in HIV

ALLERGIC DISEASES

RESPIRATORY TRACT DISORDERS

Hypersensitivity pneumonitis (rare)

Allergic bronchopulmonary aspergillosis

Eosinophilic pneumonia

Transient pulmonary infiltrates (Löeffler syndrome)

Prolonged pulmonary infiltrates with eosinophilia (PIE syndrome)

Only gold members can continue reading. Log In or Register to continue

Related

Stay updated, free articles. Join our Telegram channel

Tags: Hematology Diagnosis and Treatment

Jun 12, 2016 | Posted by admin in HEMATOLOGY | Comments Off

Full access? Get Clinical Tree

Get Clinical Tree app for offline access

Get Clinical Tree app for offline access