Background Hypereosinophilic syndrome (HES) is a heterogeneous group of rare disorders defined by persistent blood eosinophilia ≥1.5 × 109 /L, absence of a secondary cause, and evidence of ...eosinophil-associated pathology. With the exception of a recent multicenter trial of mepolizumab (anti–IL-5 mAb), published therapeutic experience has been restricted to case reports and small case series. Objective The purpose of the study was to collect and summarize baseline demographic, clinical, and laboratory characteristics in a large, diverse cohort of patients with HES and to review responses to treatment with conventional and novel therapies. Methods Clinical and laboratory data from 188 patients with HES, seen between January 2001 and December 2006 at 11 institutions in the United States and Europe, were collected retrospectively by chart review. Results Eighteen of 161 patients (11%) tested were Fip1-like 1–platelet-derived growth factor receptor α ( FIP1L1-PDGFRA ) mutation—positive, and 29 of 168 patients tested (17%) had a demonstrable aberrant or clonal T-cell population. Corticosteroid monotherapy induced complete or partial responses at 1 month in 85% (120/141) of patients with most remaining on maintenance doses (median, 10 mg prednisone equivalent daily for 2 months to 20 years). Hydroxyurea and IFN-α (used in 64 and 46 patients, respectively) were also effective, but their use was limited by toxicity. Imatinib (used in 68 patients) was more effective in patients with the FIP1L1-PDGFRA mutation (88%) than in those without (23%; P < .001). Conclusion This study, the largest clinical analysis of patients with HES to date, not only provides useful information for clinicians but also should stimulate prospective trials to optimize treatment of HES.
Peripheral blood eosinophilia is commonly encountered in clinical practice. The causes of peripheral blood eosinophilia are varied, ranging from benign eosinophilia to malignancy. A careful history ...and physical examination along with directed clinical evaluation may help determine the cause. When uncontrolled, peripheral blood eosinophilia may result in end-organ damage and life-threatening complications. This article summarizes the differential diagnosis and evaluation of persistent marked eosinophilia.
Vitamin D deficiency has been associated with increased risk for severe asthma, challenge-proven food allergy, and severe atopic dermatitis. Vitamin D levels have not been reported in patients with ...eosinophilic esophagitis (EoE).
To determine levels of 25-hydroxyvitamin D in a cohort of patients with EoE.
Total serum 25-hydroxyvitamin D was measured using liquid chromatography with tandem mass spectroscopy in adults (n = 35) and children (n = 34) with EoE. Results were compared with patient demographics, EoE-specific disease parameters, markers of sensitization, and features of severity using multivariable logistic regression.
The median vitamin D level was 28.9 ng/mL. Patients with insufficient vitamin D (<30 ng/mL) were older (median 25.5 vs 16.2 years) and had a higher body mass index (median 25.2 vs 19.8 kg/m(2)). Peak median esophageal eosinophil counts were not significantly different for vitamin D insufficient and sufficient patient groups; however, higher vitamin D levels correlated with higher histologic eosinophil counts (R = 0.61, P = .03). Although there were no statistical differences in total IgE or levels of specific IgE between patients with vitamin D insufficiency and those with sufficiency, a positive skin prick test reaction to peanut was more common in patients who had vitamin D insufficiency (adjusted odds ratio 7.57, P = .009). Vitamin D insufficiency was not associated with surrogate markers of severity (dilation in adults or hospitalization or emergency visits in children).
In these patients with EoE, vitamin D levels were low overall (median <30 ng/mL). The only marker of sensitization associated with insufficient vitamin D in these patients with EoE was a positive skin prick test reaction to peanut.
Chronic idiopathic urticaria (CU) has been associated with other autoimmune diseases and basophil-activating autoantibodies to FcεRI or IgE. It is unknown whether patients with systemicautoimmune ...diseases have a similar prevalence of these autoantibodies.
To compare the prevalences of basophil-activating autoantibodies (elevated CU Index) in patients with CU, rheumatoid arthritis (RA), and systemic lupus erythematosus (SLE). Clinical characteristics and laboratory studies were examined for an association with the CU Index.
Adult patients, 27 with CU, 27 with RA, and 26 with SLE, and 20 healthy controls were compared on the basis of the CU Index panel, anti-IgE, and antithyroid antibodies.
The CU Index values were significantly higher in the CU group when compared with the RA group but not when compared with the SLE group. 33% of CU, 23% of SLE, 3.7% of RA, and 15% of controls had apositive CU Index. Elevated antithyroid antibody levels did not correlate with a positive CU Index in any of the groups. An elevated CU Index in the SLE group was not associated with age, sex, ethnicity, disease severity, or history of atopy.
The CU Index values were elevated in patients with CU and SLE. The presence of these autoantibodies did not correlate with disease activity or presence of thyroid antibodies. Functional autoantibodies may not be specific for chronic idiopathic urticaria, and their role in nonurticarial systemic autoimmune diseases requires further investigation.
Although IgE antibodies to cow's milk and wheat are common in patients with eosinophilic esophagitis (EoE), titers are low and responses to diet are not dependent on having IgE antibodies.
To better ...define specific IgE antibody responses to foods, focusing on those foods that appear to play a role in EoE.
Adult (n = 46) and pediatric (n = 51) patients with EoE were recruited for skin prick testing and serum measurement (whole and diluted) of IgE antibodies specific for aeroallergens, food extracts, and component allergens by ImmunoCAP. Immuno Solid-phase Allergen Chip analysis was also used to measure the specificity of IgE antibodies to 112 allergen molecules.
In adults and children, there was a higher prevalence of sensitization to food extracts by ImmunoCAP than by skin prick testing. Using Immuno Solid-phase Allergen Chip to assess the specificity of IgE antibodies to 112 allergen molecules, we found that results for food allergens were mostly negative. In contrast, ImmunoCAP assays for specific milk allergens gave positive IgE antibody results in 31 of 34 sera. The correlations between specific IgE antibody to Bos d 4 or Bos d 5 and milk extract were strong (R = 0.89 and 0.76, respectively; P < .001). The evidence that IgE antibodies to foods were directed at minor components of the extracts was further supported by measurements on diluted sera.
The IgE responses in cow's milk-sensitized patients with EoE are frequently to whey proteins Bos d 4 and Bos d 5, minor components of the extract. These IgE assays may be able to identify the proteins that are relevant to EoE even though IgE is not the primary mechanism.
Conclusions It is important for pediatric providers to educate peanut- and tree nut-allergic individuals and their caregivers on avoidance measures for peanuts and tree nuts including the ability to ...properly identify them.
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