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[青少年嗜酸性粒细胞增多综合征作为副肿瘤性表现]

[Hypereosinophilic syndrome as paraneoplastic presentation in an adolescent].

作者信息

Arias-Martínez Isabel, Venancio-Hernández Marco

机构信息

Servicio de Alergia e Inmunología Clínica, Unidad Médica de Alta Especialidad, Hospital de Especialidades Antonio Fraga Mouret, Centro Médico Nacional La Raza, Instituto Mexicano de Seguro Social, México, DF.

出版信息

Rev Alerg Mex. 2013 Oct-Dec;60(4):193-7.

Abstract

Hypereosinophilic syndrome is characterized by peripheral eosinophilia over 1,500 cell/mm3 and/or tissue eosinophilia, with dysfunction or damage to organ, once other causes were ruled out. This paper presents a case of hypereosinophilic syndrome (HS) which presented as lymphoblastic leukemia in a teenager. This is a 13 year old female, with B cell lymphoblastic leukemia at 9 years old, who received chemotherapy for 2 years achieving remission. One year after remission she presented malar rash, hair loss, arthralgias, conjuntival redness, dyspnea and thoracic oppression. The initial blood count only showed hypereosinophilia, and a bone marrow biopsy did not show blasts and had a negative immunophenotyping. Autoantibodies were negative, except for ANA (1:1,280 in one determination after one negative), complement was normal, lupic band in skin was negative for complement and immunoglobulins; serum IgG 2,195 mg/dL, IgA 231, IgM 327, IgE 109 U/mL; skin testing for aeroallergens and food allergens were negative. Prednisone was started at 1 mg/kg. Abdominal ultrasound only reported biliary sludge flow and hepatosplenomegaly; chest tomography showed centrolobullar interstitial pattern, suggesting eosinophilic pneumonitis. The patient started with a generalized dermatosis, and a biopsy reported leucocytoclastic vasculitis. Six months after the onset of symptomatology there were generalized malaise, uncontrolled fever, gingival haemorrhage, asthenia and adynamia; a blood cell count reported blasts, and bone marrow smear confirmed the diagnosis of cell B lymphoblastic leukemia. The patient deteriorated rapidly showing signs of respiratory difficulty and acute pulmonary edema, therefore chemotherapy was started without response, and finally the patient died. There are several causes of HS, yet one of the least frequent presentations in childhood is the association with neoplasms.

摘要

高嗜酸性粒细胞综合征的特征为外周血嗜酸性粒细胞计数超过1500个细胞/mm³和/或组织嗜酸性粒细胞增多,且排除其他病因后存在器官功能障碍或损害。本文报告了一例青少年高嗜酸性粒细胞综合征(HS),该病例最初表现为淋巴细胞白血病。患者为一名13岁女性,9岁时诊断为B细胞淋巴细胞白血病,接受了2年化疗后缓解。缓解1年后,她出现了面部皮疹、脱发、关节痛、结膜充血、呼吸困难和胸部压迫感。最初的血常规检查仅显示嗜酸性粒细胞增多,骨髓活检未发现原始细胞,免疫表型分析为阴性。自身抗体均为阴性,但抗核抗体(ANA)在一次阴性结果后有一次检测结果为1:1280,补体正常,皮肤狼疮带试验中补体和免疫球蛋白均为阴性;血清IgG 2195mg/dL,IgA 231,IgM 327,IgE 109U/mL;吸入性变应原和食物变应原皮肤试验均为阴性。开始使用泼尼松,剂量为1mg/kg。腹部超声仅报告有胆泥流动和肝脾肿大;胸部断层扫描显示小叶中心间质型,提示嗜酸性肺炎。患者最初出现全身性皮肤病,活检报告为白细胞破碎性血管炎。症状出现6个月后,患者出现全身不适、持续发热、牙龈出血、乏力和肌无力;血细胞计数发现原始细胞,骨髓涂片确诊为B细胞淋巴细胞白血病。患者病情迅速恶化,出现呼吸困难和急性肺水肿的症状,因此开始化疗但无反应,最终患者死亡。HS有多种病因,然而在儿童中最不常见的表现之一是与肿瘤相关。

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