Idiopathic Hypereosinophilia: A Multicenter Retrospective Analysis

Figures & data

Table 1 Demographic and Clinical Characteristics in Patients with Idiopathic HE

	iHES (N=45)	HEus (N=28)	P-value
Female, n (%)	12 (26.7)	5 (17.9)	0.387
Age at diagnosis, year	51.84 ± 17.29	60.21 ± 18.01	0.052
Time interval between symptom onset and diagnosis, months	10.54 ± 28.67
Malignancy, n (%)	2 (4.4)	2 (7.1)	0.622
Autoimmune disease, n (%)	0	1 (3.6)	0.202
Atopy, n (%)	19 (42.2)	9 (32.1)	0.389
At initial presentation
Whole blood count, × 10^9 /L	16.62 ± 8.91	11.48 ± 4.87	0.007
Eosinophil count, × 10^9 /L	7.53 ± 7.39	4.12 ± 3.88	0.012
Hemoglobin, g/dL	13.32 ± 1.83	13.23 ± 2.21	0.852
Platelet, × 10^9 /L	242.60 ± 114.36	239.57 ± 86.14	0.905
The highest level or count during the study period
Whole blood count, × 10^9 /L	19.89 ± 10.92	12.35 ± 6.20	<0.001
Eosinophil count, × 10^9 /L	11.29 ± 8.68	7.02 ± 10.43	0.063
Total IgE, IU/mL (n=42)	1680.18 ± 2605.16	–
Vitamin B12, pg/mL (n=12)	1054.08 ± 954.61	–
Tryptase, ug/L (n=8)	5.55 ± 3.34	–
ECP, ug/L (n=14)	142.64 ± 65.68	–

Note: Values are mean ± SD unless otherwise indicated.

Abbreviations: iHES, idiopathic hypereosinophilic syndrome; HEus, hypereosinophilia with undetermined significance; IgE, immunoglobulin E; ECP, eosinophil cationic protein; SD, standard deviation.

Table 2 Clinical Manifestations of Study Subjects at Initial Presentation

Clinical Manifestations	iHES N=45, (%)	HEus N=28, (%)	Description of Clinical Symptoms and/or Organ Dysfunction
Cutaneous	22 (48.9)	5 (17.9)	Urticaria, angioedema, pruritus, rash, vesicle or bullae, nodular and/or mass like lesion
Pulmonary	27 (60)	4 (14.3)	Cough, dyspnea, pleural effusion, sputum, pulmonary infiltration
Gastrointestinal	27 (60)	4 (14.3)	Abdominal pain, nausea, vomiting, dyspepsia, diarrhea, colitis, ascites, liver infiltration
Cardiac	6 (13.3)	0 (0.0)	Chest pain, heart failure, cardiomyopathy, myocarditis, pericardial effusion, endocarditis
Neurologic	6 (13.3)	1 (3.6)	Vertigo, dizziness, neuropathy
Rheumatologic	1 (2.2)	1 (3.6)	Arthritis, arthralgia, myalgia
Hematologic	20 (44.4)	4 (14.3)	Anemia, thrombocytopenia, thromboembolism, bone marrow abnormality
Constitutional	10 (22.2)	0 (0.0)	Fever, weight loss, malaise, fatigue

Figure 1 Evaluation of organ involvement in patients with idiopathic hypereosinophilia.

Abbreviations: iHES, idiopathic hypereosinophilic syndrome; HEus, hypereosinophilia with undetermined significance; CT, computed tomography; APCT, abdominal and pelvic CT; PFT, pulmonary function test; EGDS, esophagogastroduodenoscopy; CFS, colonoscopy; BM, bone marrow examination.

Figure 2 Treatment response of patients with iHES.

Abbreviations: iHES, idiopathic hypereosinophilic syndrome; CR, complete response; PR, partial response.

Figure 3 Treatment response of patients with HEus.

Abbreviations: HEus, hypereosinophilia with undetermined significance; CR, complete response; PR, partial response.

Table 3 Predictive Factors of Complete Response to Treatment in Patients with iHES

	Univariate			Multivariate
	OR	CI	P	OR	CI	P
Female	1.882	0.473–7.488	0.369	256.096	0.755–86,870.318	0.062
Age at diagnosis, year	1.011	0.976–1.046	0.541	1.085	0.939–1.254	0.270
Time interval between symptom onset and diagnosis, months	0.985	0.955–1.016	0.337	0.970	0.919–1.024	0.273
Asthma	0.773	0.138–4.319	0.769	11.335	0.011–11,211.417	0.490
Allergic rhinitis	0.348	0.057–2.132	0.254	0.060	0.000–56.090	0.421
Atopy	0.563	0.170–1.864	0.347	0.198	0.011–3.554	0.272
Presence of clinical manifestation at initial presentation	0.444	0.113–1.743	0.245	0.969	0.033–28.829	0.985
Number of involved organs	1.861	1.016–3.409	0.044	4.395	1.033–18.699	0.045
Whole blood count_initial, × 10^9 /L	1.000	1.000–1.000	0.900	1.000	1.000–1.000	0.584
Eosinophil count_initial, × 10^9 /L	1.000	1.000–1.000	0.876	1.000	1.000–1.000	0.948
Hemoglobin_initial, g/dL	0.901	0.648–1.253	0.535	3.387	0.745–15.399	0.114
Platelet_initial, × 10^9 /L	0.993	0.987–0.999	0.034	0.983	0.964–1.002	0.074
Total IgE, IU/mL	1.000	0.999–1.000	0.086	0.999	0.998–1.000	0.051
Maximal daily dose of corticosteroids	1.011	0.994–1.029	0.212	1.036	0.970–1.107	0.295

Abbreviations: iHES, idiopathic hypereosinophilic syndrome; OR, odds ratio; CI, confidence interval; IgE, immunoglobulin E.