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:

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

Tropical eosinophilia Visceral larval migrans (VLM, toxocariasis) Helminth infections Filariasis (Wuchereria bancrofti, Brugia malay) Onchocerciasis Schistosomiasis Fascioliasis Paragonimiasis Strongyloidiasis Trichinosis Hookworm Ascariasis Echinococcosis/hydatid disease

Fungal Infections

Coccidioidomycosis

Cryptococcosis (CSF eosinophilia) in HIV

Modified and updated from Mahanty S, Nutman, TB: Eosinophilia and eosinophil-related disorders. In Middleton EJ, Reed CE, Ellis EF, et al, editors: Allergy: Principles and practice, ed 4, St Louis, 1993, Mosby-Year Book, p 1077.

Table 31-2 Major Organ Involvement and Prominent Clinical Features of Patients With Hypereosinophilic Syndrome*

^*Listed in order of frequency.

^†Average percentage of 105 patients from American, French, and English studies combined.

^‡Reproduced from Spry CJ: The hypereosinophilic syndrome: Clinical features, laboratory findings and treatment. Allergy 37:539, 1982.

^§Reproduced from Weller PF, Bubley GJ: The idiopathic hypereosinophilic syndrome. Blood 83:2759, 1994.

Table 31-3 Criteria for the Diagnosis of Idiopathic Hypereosinophilic Syndrome

Reproduced with permission from Brito-Babapulle F: Clonal eosinophilic disorders and the hypereosinophilic syndrome. Blood Rev 11:129, 1997.

Table 31-4 End-Organ Damage Produced by Hypereosinophilia

Reproduced from Brito-Babapulle F: Clonal eosinophilic disorders and the hypereosinophilic syndrome. Blood Rev 11:129, 1997.

Figure 31-2 DERMATOLOGIC MANIFESTATIONS OF HES.

Erythematous pruritic papules and nodules (A). Partially centrally ulcerated (B) hematoxylin-eosin-stained biopsy tissues demonstrating inflammatory cell infiltrates largely consisting of eosinophils and lymphocytes (C). Larger magnification of perivascular infiltrate with numerous eosinophils (D).

(From Plötz, S.G., Hüttig B, Aigner B, et al: Clinical overview of cutaneous features in hypereosinophilic syndrome. Curr Allergy Asthma Rep 12:85, 2012.)