Releasability of human hypereosinophilic eosinophils is related to the density of the cells

The activity of eosinophils and neutrophils with respect to the release of granule proteins was studied in 11 patients with the hypereosinophilic syndrome (HES). Granulocytes or purified eosinophils were stimulated with serum-opsonized Sephadex particles (C3b-induced release), and the released amounts of eosinophil cationic protein (ECP), eosinophils protein-X (EPX) and myeloperoxidase (MPO) were measured by means of specific radioimmunoassays (RIA). Eosinophils obtained from patients with HES released significantly more ECP ( P <0·002) and EPX ( P <0·01) after 20 min of incubation than cells from the control group. The cellular content of ECP and EPX in eosinophils obtained from the patients with HES was significantly reduced to 50% and 62%, respectively, of the content of these granule proteins of eosinophils from the control group. In separated eosinophils light-density eosinophils released more of both ECP and EPX than normal density eosinophils. There was no difference in MPO release between the patients and the control group. We conclude that the eosinophils from patients with HES have an increased propensity to release their granule proteins and the releasability seems to be related to the density of the cells.     

Life threatening coronary artery spasm in childhood Kimura's disease

A 13 year old boy is described with hypereosinophilia associated with Kimura's disease, who showed repeated life threatening syncopal attacks during daily activities or at rest. Coronary arteriography demonstrated small aneurysms with irregular vessel walls of both coronary arteries, and the absence of organic stenotic lesions. Infusion of a minimal dose of ergonovine into the right coronary artery induced severe spasm of the vessel. Ventricular fibrillation recurred even after administration of nifedipine and isosorbide was started, but was completely inhibited by prednisolone.


Keywords: coronary vasospasm; hypereosinophilia; Kimura's disease; ventricular tachyarrhythmia     

Chronic eosinophilic perimyositis with persistent myalgias

A 43-year-old man with an 8-year history of hypereosinophilia was evaluated for persistent muscle pain. Methotrexate and corticosteroids were ineffective. Examination, limited by pain even with passive motion, showed only mild weakness. Electromyography and muscle enzymes were normal. A needle muscle biopsy specimen revealed eosinophilic perimyositis. This case illustrates that the diagnosis of eosinophilic perimyositis requires histopathological evaluation, which should be pursued in patients with eosinophilia and persistent myalgia despite normal electromyography and muscle enzymes.     

Chronic eosinophilic leukemia presenting with autoimmune hemolytic anemia and erythrophagocytosis by eosinophils

Eosinophils function primarily as secretory cells and phagocytosis by eosinophils is rarely seen. We describe a case of chronic eosinophilic leukemia (CEL) in a 72-year-old male with a history of previously treated non-Hodgkin's lymphoma (NHL) presenting with erythrophagocytosis by eosinophils and an associated autoimmune hemolytic anemia (AIHA). This patient did not show evidence of relapsed NHL. The patient's blood showed a markedly elevated eosinophil count of 16 x 10(9)/L [normal 0-0.45 x 10(9)/L] on a background of myelodysplasia and features of AIHA. Prominent erythrophagocytosis by eosinophils was visualized in the blood and in the bone marrow. Numerous Charcot-Leyden crystals were also seen in the bone marrow amid increased numbers of eosinophils and the presence of dysplastic granulopoiesis. AIHA is rarely described in the setting of CEL. More significantly, this represents the first case report to describe erythrophagocytosis by eosinophils.     

A rat model of hypereosinophilic syndrome

Hypereosinophilia-occurring rats without chemical and antigen treatment have been maintained in our laboratory. The rat, Matsumoto Eosinophilia Shinshu (mes), showed hypereosinophilia at the age of 9 weeks or older and developed eosinophil-related inflammatory lesions in many organs. These lesions included: aortitis, granulomatous lesion in the mesenteric lymph node, inflammatory fibroid polyp of the stomach and pulmonary vasculitis with septal infiltration. These lesions were involved with cellular infiltration of eosinophils and macrophages, and deposition of eosinophilic crystals which immunohistologically showed major basic protein and eosinophilic peroxidase derived from eosinophilic lysosomal constituents. Although the distribution of lesions in mes is a little different from that of hypereosinophilic syndrome (HES) in humans, in that endomyocardial fibrosis appears in HES while aortitis appears in mes, mes is probably comparable with HES. The present paper describes the pathological aspects of the lesions in mes and discusses the pathogenesis of tissue injury related to eosinophilic infiltration.     

Pathological and High Resolution CT Findings in Churg-Strauss Syndrome

Objective To investigate the Churg-Strauss syndrome (CSS) associated lung involvement,concentrating on clinical characteristics,pathological findings of lung involvements,response to treatment,and prognosis.Methods We retrospectively analyzed the characters of the clinical manifestations,thin-section CT and pathological findings of CSS.The study involved 16 patients.Clinical data were obtained by chart review.All patients underwent transbronchial lung biopsy (TBLB).Six of them underwent surgical lung biopsy as well.Results The patients included 7 men and 9 women,aged from 14 to 61 years (median,47.5 years).Extrathoracic organs involved included nervous system (7/16) and skin (5/16).Respiratory symptoms included cough (12/16),exertional dyspnea (11/16),hemoptysis (4/16),and chest pain (3/16).CT findings included bilateral ground-glass opacities (12/16),bilateral patchy opacities (12/16),and centrilobular nodules (6/16).The pathological findings of TBLB demonstrated increased eosinophils (3/16),vasculitis (3/16),and interstitial pneumonia (16/16).The pathological findings of surgical lung biopsy of 6 cases showed necrotizing vasculitis in 4 cases,capillaries in 5,eosinophilic pneumonia in 3,granulomas in 2,and airway abnormalities in 3.All patients improved in symptoms after therapy during the study period (range,3 to 51 months;median,15 months).Conclusions Asthma may be present in CSS patient when there is bronchial involvement.Ground-glass opacities and consolidation seen on high-resolution CT reflect the presence of eosinophilic pneumonia,vasculitis,and pulmonary alveolar hemorrhage.TBLB has significant limitations for the diagnosis of CSS.Early diagnosis and therapy can result in satisfactory prognosis.     

原发嗜酸细胞增多症临床分析

目的探讨原发嗜酸细胞增多症的临床特点.方法回顾性分析55例原发嗜酸细胞增多症患者的临床资料.结果55例均符合原发嗜酸细胞增多症的诊断标准,男性为女性的2倍.胃肠道异常为最常见的临床表现(50.8%),其次为肺部病变(37.7%)、心脏病变(26.2%)和神经系统病变(21.3%);其他临床表现包括皮肤改变(19.7%)、浅表淋巴结肿大(18%)、血栓形成(9.8%)和哮喘(3.3%).肠黏膜和浅表淋巴结活检均表现为大量嗜酸细胞浸润,经糖皮质激素治疗后 临床表现均明显好转.结论男性较女性更易患原发嗜酸细胞增多症,最常见的受累器官为胃肠道、肺、心脏和神经系统,糖皮质激素治疗有效.