Most of temporal arteritis occurs in the older patient over 50 years old, and the histopathologic finding shows a granulomatous inflammation, so this called giant cell arteritis. However, the young patients also present with a nodular lesion in their temple, and juvenile temporal arteritis (JTA) should be considered as one of the differential diagnosis, although it is very rare. For both diagnosis and treatment of JTA, excisional biopsy is essential. The pathologic finding of the temporal artery shows panarteritis with lymphoeosinophilic infiltrates, but no giant cell or granulomatous lesion. JTA is a localized disease with low level of systemic inflammatory marker, so the symptom is usually relieved by excision of affected lesion. Peripheral blood eosinophilia present in some cases of JTA, but its relation with clinical course and prognosis is not yet been known. Herein, we report the case of a 24-year-old man diagnosed with concurrent JTA and hypereosinophilic syndrome. We also reviewed the literature of JTA focusing on the impact of combined peripheral eosinophilia on the course of the disease. Combined peripheral eosinophilia may increase the risk of recurrence of JTA after local treatment such as excision only.
Biopsy ; Diagnosis ; Diagnosis, Differential ; Eosinophilia ; Giant Cell Arteritis ; Giant Cells ; Humans ; Hypereosinophilic Syndrome ; Inflammation ; Prognosis ; Recurrence ; Temporal Arteries ; Young Adult

PURPOSE: We aimed to analyze the frequency of eosinophilia-associated diseases and to search for possible markers that may be useful for their differential diagnosis. METHODS: We retrospectively reviewed the medical records of 148 patients with peripheral blood eosinophil count of more than 500/µL who visited the Allergy Department of Chonnam National University Hospital for the first time from January to December 2016. Blood eosinophilia was categorized as mild (<1,500/µL), moderate (1,500–5,000/µL), and severe (>5,000/µL). RESULTS: Blood eosinophilia was mostly caused by allergic diseases (41.9%), parasitic infestation (23.6%), and drug allergy (19.6%). Eosinophil count was higher in patients with parasitic infestation (P<0.01), drug allergy (P<0.01), hypereosinophilic syndrome (HES, P<0.001), or eosinophilic granulomatosis with polyangiitis (EGPA, P<0.001) than in those with allergic diseases. The eosinophilic cationic protein level was higher in patients with HES than in those with allergic diseases (P<0.05) and parasitic infestation (P<0.05). The total IgE level was lower in patients with HES than in those with parasitic infestation (P<0.05) and EGPA (P<0.05). The vitamin B12 level was higher in patients with HES than in those with parasitic infestation (P<0.05). There was no statistically significant difference in tryptase levels between the groups. The most common cause of mild eosinophilia was allergic diseases (59.8%), followed by parasitic infestation (22.7%) and drug allergy (13.4%). The common causes of moderate eosinophilia were drug allergy (37.8%), parasitic infestation (29.7%), and allergic diseases (10.8%). The common causes of severe eosinophilia were EGPA (28.6%), HES (21.4%), parasitic infestation (14.3%), and drug allergy (14.3%). CONCLUSION: Common causes of blood eosinophilia in patients who visit the allergy department are allergic diseases, parasitic infestation, and drug allergy. Several markers, including eosinophil count, total IgE, and vitamin B12, may be useful for the differential diagnosis of eosinophilia-associated diseases.
Diagnosis, Differential ; Drug Hypersensitivity ; Eosinophilia ; Eosinophils ; Granulomatosis with Polyangiitis ; Humans ; Hypereosinophilic Syndrome ; Hypersensitivity ; Immunoglobulin E ; Jeollanam-do ; Medical Records ; Parasitic Diseases ; Retrospective Studies ; Tryptases ; Vitamin B 12

We report a patient with massive eosinophilia and a complex karyotype that was initially misdiagnosed as chronic eosinophilic leukemia (CEL), but later diagnosed as anaplastic large cell lymphoma (ALCL) masked by massive eosinophilia. The complex karyotype observed at initial diagnosis remained unchanged later, after the evidence of bone marrow involvement of ALCL was obtained. At diagnosis, genetic aberrations corresponding to metaphase cytogenetics were not identified by interphase fluorescence in situ hybridization, although abnormal results were noted at follow-up. Together, these observations indicate that the complex karyotype at initial work-up has been derived from a low proportion of lymphoma cells with high mitotic ability that were not identified by microscopy, rather than from massive eosinophils. These findings suggest that our patient had ALCL with secondary eosinophilia rather than CEL since initial diagnosis.
Bone Marrow ; Cytogenetics ; Diagnosis ; Eosinophilia* ; Eosinophils* ; Fluorescence ; Follow-Up Studies ; Humans ; Hypereosinophilic Syndrome* ; In Situ Hybridization ; Interphase ; Karyotype* ; Lymphoma ; Lymphoma, Large-Cell, Anaplastic* ; Masks ; Metaphase ; Microscopy

Eosinophilic cholangiopathy is a rare disease characterized by dense transmural eosinophilic infiltration of the gallbladder and bile duct. It's clinical and laboratory manifestations are not different from those of other causes of cholangiopathy, and the diagnosis is usually made based on pathologic findings after cholecystectomy. Moreover, the occurrence of eosinophilic cystitis accompanied by cholangiopathy is extremely rare. We report a rare case of hypereosinophilic syndrome manifested as eosinophilic cholangiopathy accompanied with eosinophilic cystitis, for the first time in Korea, in a 49-year-old woman who presented with persistent right upper quadrant pain. After performing imaging study to validate the diagnosis of acute acalculous cholecystitis and cholangitis, an urgent cholecystectomy was performed. Pathologic examination of the excised gallbladder was consistent with eosinophilic cholecystitis. The patient underwent bladder biopsy because there was persistant irritative voiding symptoms combined with constant mild peripheral eosinophilia even after cholecystectomy, and the pathologic findings revealed eosinophilic cystitis. Symptoms and peripheral eosinophilia were improved after steroid therapy for an indicated period.
Acalculous Cholecystitis ; Bile Ducts ; Biopsy ; Cholangitis ; Cholecystectomy ; Cholecystitis ; Cystitis* ; Diagnosis ; Eosinophilia ; Eosinophils* ; Female ; Gallbladder ; Humans ; Hypereosinophilic Syndrome* ; Korea ; Middle Aged ; Rare Diseases ; Urinary Bladder

Eosinophilic myocarditis is a condition resulting from various eosinophilic diseases, including helminth infection, drug hypersensitivity, systemic vasculitis or idiopathic hypereosinophilic syndromes. Clinical manifestations of eosinophilic myocarditis may vary from early necrosis to endomyocardial fibrosis. Eosinophilic myocarditis is one of the most fatal complications of hypereosinophilia. However, eosinophilic myocarditis has been rarely reported in the literature, particularly in Asia Pacific regions, reflecting the under-recognition of the disease among clinicians. Early recognition is crucial for improving clinical outcomes of eosinophilic myocarditis. Early administration of systemic corticosteroid is necessary in eosinophilic myocarditis regardless of underlying causes, as delayed treatment may result in fatal outcomes. In addition, differential diagnoses of underlying causes for eosinophilia are necessary to improve long-term outcomes.
Asia ; Diagnosis, Differential ; Drug Hypersensitivity ; Endomyocardial Fibrosis ; Eosinophilia ; Eosinophils ; Fatal Outcome ; Helminths ; Hypereosinophilic Syndrome ; Myocarditis ; Necrosis ; Systemic Vasculitis ; Toxocariasis

Eosinophilic cholecystitis (EC) is a rare form of acute cholecystitis, of which diagnosis is based on classical symptoms of cholecystitis with a presence of >90% eosinophilic infiltration within the gall bladder. EC rarely manifests in idiopathic hypereosinophilic syndrome (IHES). Here, we report two cases of EC with IHES. One is a 57-year-old male who presented with acute right upper quadrant (RUQ) pain, jaundice and fever. The initial peripheral blood eosinophil count was 2,070/mm3, and further elevated to 12,590/mm3. Acute acalculous cholecystitis with cholangitis was confirmed by computed tomography (CT). He improved with endocopic nasobiliary drainage and antibiotic therapy. The other is a 64-year-old female who presented with acute RUQ pain. She also complained of dyspnea and tingling sensation of both hands and feet. The initial peripheral blood eosinophil count was 10,400/mm3. Abdominal CT revealed findings suggestive of acute acalculous cholecystitis. She improved with systemic glucocorticosteroid therapy. No other causes of hypereosinophilia were found in either patients. Thus, cholecystectomy may not be mandatory for the treatment of EC with IHES.
Acalculous Cholecystitis ; Cholangitis ; Cholecystectomy ; Cholecystitis* ; Cholecystitis, Acute ; Diagnosis ; Drainage ; Dyspnea ; Eosinophils* ; Female ; Fever ; Foot ; Hand ; Humans ; Hypereosinophilic Syndrome* ; Jaundice ; Male ; Middle Aged ; Sensation ; Tomography, X-Ray Computed ; Urinary Bladder

Although idiopathic hypereosinophilic syndrome(IHES) commonly involves the lung, it is rarely associated with acute respiratory distress syndrome (ARDS). Here we describe a case of IHES presented in conjunction with ARDS. A 37-year-old male visited the emergency department at Samsung Medical Center, Seoul, Korea, with a chief complaint of dyspnea. Blood tests showed profound peripheral eosinophilia and thrombocytopenia. Patchy areas of consolidation with ground-glass opacity were noticed in both lower lung zones on chest radiography. Rapid progression of dyspnea and hypoxia despite supplement of oxygen necessitated the use of mechanical ventilation. Eosinophilic airway inflammation was subsequently confirmed by bronchoalveolar lavage, leading to a diagnosis of IHES. High-dose corticosteroids were administered, resulting in a dramatic clinical response.
Adrenal Cortex Hormones ; Adult ; Anoxia ; Bronchoalveolar Lavage ; Diagnosis ; Dyspnea ; Emergency Service, Hospital ; Eosinophilia ; Eosinophils ; Hematologic Tests ; Humans ; Hypereosinophilic Syndrome* ; Inflammation ; Korea ; Lung ; Male ; Oxygen ; Pulmonary Embolism ; Radiography ; Respiration, Artificial ; Respiratory Distress Syndrome, Adult* ; Seoul ; Thorax ; Thrombocytopenia

A 39-year-old man presented with cough, chest discomfort, and weight loss. On the basis of the patient history and laboratory findings, he was diagnosed with the hypereosinophilic syndrome. Transthoracic echocardiography revealed a large thrombus in the left ventricle. Medical treatment with anticoagulation and immunosuppression was commenced immediately. Fourteen days after the initial diagnosis, the patient presented with acute pain in his right leg. Computed tomographic angiogram showed embolic occlusion of the infrarenal abdominal aorta and bilateral iliac (including common, external, and internal iliac) arteries. Emergent thromboembolectomy and left ventricular thrombectomy were performed. The postoperative course was uneventful, and the patient has undergone follow-up for 2 months without any evidence of recurrence of thromboembolism.
Acute Pain ; Adult ; Aorta, Abdominal ; Arteries ; Cough ; Diagnosis ; Echocardiography ; Follow-Up Studies ; Heart Ventricles ; Humans ; Hypereosinophilic Syndrome* ; Immunosuppression ; Leg ; Recurrence ; Thorax ; Thrombectomy ; Thromboembolism* ; Thrombosis* ; Weight Loss

Hypereosinophilic syndrome (HES) is defined as peripheral eosinophilia, organ involvement of eosinophils and exclusion of other disorders or dysfunction. HES is subclassified as myeloproliferative variant, T-lymphocytic, overlap, idiopathic, familial and associated type according to the new classification using molecular and immunologic markers. HES presenting with gastrointestinal symptoms is very rare but characterized by peripheral eosinophilia, eosinophilic infiltration of the gastrointestinal tract with other organs such as liver and bone marrow. We report a rare case of idiopathic HES in 18-year-old adolescent who presented with gastrointestinal symptoms including abdominal pain, diarrhea and ascites which resolved rapidly with corticosteroid therapy. HES should be included in the differential diagnosis of abdominal pain and ascites in children and adolescents.
Abdominal Pain ; Adolescent* ; Ascites ; Biomarkers ; Bone Marrow ; Child ; Classification ; Diagnosis, Differential ; Diarrhea ; Eosinophilia ; Eosinophils ; Gastrointestinal Tract ; Humans ; Hypereosinophilic Syndrome* ; Liver

Adult ; Humans ; Hypereosinophilic Syndrome ; complications ; drug therapy ; Lymphatic Diseases ; diagnosis ; etiology ; pathology ; Male ; Treatment Outcome