Seifert Mandy, Gerth Jens, Gajda Mieczyslaw, Pester Frank, Pfeifer Rüdiger, Wolf Gunter
Klinik für Innere Medizin III, Friedrich-Schiller-Universität Jena, Jena, Germany.
Med Klin (Munich). 2008 Aug 15;103(8):591-7. doi: 10.1007/s00063-008-1094-z. Epub 2008 Sep 21.
Eosinophilia is not uncommon in clinical practice. The main causes are allergies and parasitic infections. Rarely, eosinophilia is associated with pulmonary affections, malignant tumors, gastroenteritis, and autoimmune diseases. A new classification based on pathophysiological data for the hypereosinophilic syndrome in order to simplify diagnosis and therapy was introduced in 2006.
A 22-year-old man was admitted to another hospital because of acute abdominal pain. An unspecific colitis was diagnosed. Blood counts showed a mild neutrophilic leukocytosis (12.6 Gpt/l) with a severe relative eosinophilia (30%), thrombocytopenia (67 Gpt/l), and an increased C-reactive protein (CRP 122 mg/l). The patient also had a deep venous thrombosis of the left leg. An explorative laparotomy was performed because of a strong suspicion of a presacral abscess. Pulmonary embolism and embolic pneumonia developed after surgery. A macular-cockade exanthema on the trunk and extremities was found. Histological examination revealed perivascular eosinophilic infiltrates. Histological and cytological analysis of bone marrow showed many eosinophilic granulocytes and a hypercellular medulla without increased numbers of blasts. No parasites in the blood and stools were found, and there was no evidence of neoplasm or cardiac involvement. p- and c-ANCAs (antineutrophil cytoplasmic antibodies), ANAs (antinuclear antibodies), and antibody against dsDNA were negative. Further genetic, FISH (fluorescence in situ hybridization), and PCR (polymerase chain reaction) analyses showed no evidence for chromosomal aberrations. An undefined hypereosinophilic syndrome with multiple organ involvement was diagnosed. Shortly after starting an oral prednisolone therapy (1 mg/kg body weight), the eosinophilia normalized. This therapy was stopped after 2 months and the patient is now, 6 months after diagnosis, in normal health.
As demonstrated in this case, eosinophilia requires a broad differential diagnosis. A hypereosinophilic syndrome can involve many organs and mimic other diseases. The new classification of the hypereosinophilic syndrome from 2006, based on pathophysiological insights, may foster better diagnosis and therapy for this rare disease.
嗜酸性粒细胞增多在临床实践中并不少见。主要病因是过敏和寄生虫感染。嗜酸性粒细胞增多很少与肺部疾病、恶性肿瘤、肠胃炎及自身免疫性疾病相关。2006年引入了一种基于病理生理数据的高嗜酸性粒细胞综合征新分类法，以简化诊断和治疗。
一名22岁男性因急性腹痛入住另一家医院。诊断为非特异性结肠炎。血常规显示轻度中性粒细胞增多（12.6×10⁹/L）伴严重相对嗜酸性粒细胞增多（30%）、血小板减少（67×10⁹/L）及C反应蛋白升高（CRP 122mg/L）。患者还患有左下肢深静脉血栓形成。因高度怀疑骶前脓肿而进行了探查性剖腹手术。术后发生肺栓塞和栓塞性肺炎。在躯干和四肢发现了斑疹-帽状皮疹。组织学检查显示血管周围嗜酸性粒细胞浸润。骨髓组织学和细胞学分析显示有许多嗜酸性粒细胞和细胞增多的骨髓，原始细胞数量未增加。血液和粪便中未发现寄生虫，也没有肿瘤或心脏受累的证据。抗中性粒细胞胞浆抗体（p-和c-ANCA）、抗核抗体（ANA）及抗双链DNA抗体均为阴性。进一步的基因、荧光原位杂交（FISH）和聚合酶链反应（PCR）分析未发现染色体畸变证据。诊断为不明原因的多器官受累高嗜酸性粒细胞综合征。开始口服泼尼松龙治疗（1mg/kg体重）后不久，嗜酸性粒细胞增多恢复正常。2个月后停止该治疗，目前患者在诊断后6个月，健康状况正常。
如本病例所示，嗜酸性粒细胞增多需要进行广泛的鉴别诊断。高嗜酸性粒细胞综合征可累及多个器官并酷似其他疾病。2006年基于病理生理学见解的高嗜酸性粒细胞综合征新分类法可能有助于更好地诊断和治疗这种罕见疾病。
