Diffuse Alveolar Hemorrhage as an Initial Presentation of Systemic Lupus Erythematosus

Diffuse alveolar hemorrhage is a rare but fatal complication in patients with systemic lupus erythematosus. It has rarely been reported to occur as the initial presentation in lupus patients. We report a 55-year-old female Jehovah’s witness who presented with diffuse alveolar hemorrhage as the initial manifestation of systemic lupus erythematosus. She responded favorably to early intravenous pulse methylpredniso-lone and cyclophosphamide along with hemotherapy she accepted once over the course of her hospital stay. Initially, there was clinical improvement, but relapsing of her alveolar hemorrhage 15 days later. It is important to keep in mind that lupus patients can present initially with this lethal pulmonary complication. Early identification and initiation of therapy are crucial in order to affect survival of these patients.     

Delta Neutrophil Index as an Early Marker for Differential Diagnosis of Adult-Onset Still's Disease and Sepsis

Purpose

To investigate clinical implications of delta neutrophil index (DNI) to discriminate adult onset Still''s disease (AOSD) from sepsis.

Materials and Methods

We reviewed the medical records of 13 patients with AOSD and 33 gender and age-matched patients with sepsis. In all subjects, microbial tests were performed to exclude or confirm sepsis. All laboratory data were measured two or three times during the first 3 days and represented by their mean levels. DNI was measured automatically by ADVIA 2120 for the first 3 days.

Results

There were no significant differences in white blood cell counts, neutrophil proportion, erythrocyte sedimentation rate and C-reactive protein between two groups. AOSD patients had notably lower DNI than sepsis patients regardless of the presence of bacteremia or not. However, both DNI and ferritin were not significant independent factors for predicting sepsis in the multivariate logistic regression analysis. Meanwhile, the area under the receiver operating characteristic curve (AUROC) of DNI was slightly higher than that of ferritin. When we set DNI of 2.75% as the cut-off value for predicting sepsis, 11 (84.6%) of AOSD patients had a DNI value below 2.75% and 2 (15.4%) of them had a DNI over 2.75% (relative risk for sepsis 176).

Conclusion

We suggest that DNI may be a useful marker for differential diagnosis of AOSD from sepsis in the early phase as supplementary to ferritin.     

Wegener's Granulomatosis Associated with Diffuse Pulmonary Hemorrhage

The authors report a case of Wegner's granulomatosis with the unusual manifestation of diffuse pulmonary hemorrhage. A 58-year-old man complained of bloody sputum and fever. Chest X-ray films showed multiple nodular shadows in both lung fields. He was diagnosed as having Wegener's granulomatosis by transbronchial lung biopsy, which revealed necrotizing granulomatous inflammation with necrotizing vasculitis. Despite treatment with cyclo-phosphamide and prednisolone, his condition rapidly deteriorated. An extensive diffuse alveolar shadow appeared in both lung fields in chest X-ray films, anemia became worse, and he died of respiratory failure. Autopsy revealed diffuse alveolar hemorrhage with necrotizing capillaritis in addition to the typical pathological findings in Wegener's granulomatosis. The capillaritis was characterized by neutrophilic infiltration of alveolar septa, and fibrin thrombi in alveolar capillaries. Diffuse pulmonary hemorrhage is uncommon in Wegener's granulomatosis. However, once diffuse pulmonary hemorrhage occurs, the respiratory condition rapidly deteriorates and is life-threatening. Therefore, accurate diagnosis and appropriate treatment are required. Acta Pathol Jpn 42: 657–661, 1992.     

下肢流动性骨硬化症合并丹毒1例

患者,男性,48岁。右下肢红肿热痛伴发烧2d,门诊以“右胫骨骨髓炎”收入院。一般情况尚可,体温38．7℃,右小腿红、肿、热、压痛（＋）,柯足第2／3、3／4趾蹼间糜烂,腹股沟淋巴结肿大,压痛（＋）,左胫骨内侧可触及数个大小不等包块,质硬,无活动度。     

阿加曲班在尿毒症伴出血患者中的应用

目的了解阿加曲班注射液在尿毒症伴有出血及出血倾向患者中的临床效果及安全性。方法对我院2013年1月~2014年6月收治的共16例尿毒症合并出血患者,在常规治疗的基础上给予使用阿加曲班注射液抗凝行血液透析治疗,并观察患者病情变化及预后。结果16例患者均安全度过出血危险期,4w后转肝素规律血液透析。结论尿毒症合并出血及出血倾向患者使用阿加曲班抗凝行血液透析是一种安全有效的方法。     

A Case of VEXAS Syndrome Complicated by Hemophagocytic Lymphohistiocytosis

Journal of Clinical Immunology -     

生长抑素联合泮托拉唑治疗肝硬化合并上消化道出血的疗效观察

目的 探究生长抑素联合泮托拉唑治疗肝硬化合并上消化道出血的治疗效果。方法 选取大连市普兰店区第二人民医院内科2017年1月~2018年4月收治的肝硬化合并上消化道出血者共60例,以抽取红黄球方式分组,红球为实验组,黄球为对照组,每组30例。实验组采用生长抑素联合泮托拉唑治疗,对照组采用生长抑素治疗。分别观察两组患者临床治疗效果、止血时间、输血量、住院所需时间、血红蛋白水平、不良反应总出现率。结果 实验组止血时间[（23.62±4.92）vs（34.55±6.20）h]、输血量[（243.92±50.33）ml vs（492.39±55.20）ml]、住院所需时间[（7.66±1.09）d vs（17.20±2.09）d]、不良反应总出现率（6.67% vs 30.00%）均低于对照组,差异具有统计学意义（P＜0.05）。实验组临床治疗总有效率（86.67% vs 60.00%）、血红蛋白水平[（120.60±15.00）g/L vs （103.01±15.37）g/L]均高于对照组,差异具有统计学意义（P＜0.05）。结论 与单一用药相比,给予肝硬化合并上消化道出血患者采用生长抑素联合泮托拉唑治疗,不仅利于改善其血红蛋白水平,更利于减少输血量、住院时间、用药不良反应,进而提升治疗效果。     

传染性单核细胞增多症合并langerhans细胞组织细胞增生症1例报告并文献复习

目的探讨传染性单核细胞增多症以及langerhans细胞组织细胞增生症的临床表现和诊治方法。方法对诊治的1例传染性单核细胞增多症合并langerhans细胞组织细胞增生症患者进行病例分析和相关文献复习。结果患者为17岁男性,因＂咽痛发热10d,皮疹2d＂收入院。经头孢美唑钠、亚胺培南西司他丁钠、替考拉宁、利奈唑胺、头胞哌酮舒巴坦抗细菌及利巴韦林、更昔洛韦抗病毒治疗后无效,经骨髓细胞学检测确诊为传染性单核细胞增多症,经淋巴结病理学检查确诊为langerhans细胞组织细胞增生症,经COP化疗1疗程后体温正常。后经5疗程COP方案巩固治疗。结论传染性单核细胞增多症同时合并langerhans细胞组织细胞增生症极为罕见,经COP方案治疗效果较好。     

A Case of Moyamoya Disease in a Girl with Thyrotoxicosis

Moyamoya disease is a cerebrovascular disorder of unknown cause, characterized by slowly progressive bilateral stenosis or occlusion of the internal carotid arteries and produces collateral vessels. Moyamoya syndrome has rarely been reported in association with Graves'' disease, especially in children. Several reports suggest that a cerebral infarction might have occurred in patients with clinical and laboratory evidence of hyperthyroid function. We report a case of Moyamoya disease in a girl with Down syndrome and thyrotoxicosis, and we review the relevant literature. To our best knowledge, this is the first report of Moyamoya disease associated with thyrotoxicosis in a young person in Korea.     

A Case of Primary Adrenal Diffuse Large B-cell Lymphoma Achieving Complete Remission with Rituximab-CHOP Chemotherapy

Primary adrenal lymphoma is a very rare extranodal lymphoma; its clinical features consist of a high incidence of bilateral adrenal involvement and diffuse large B-cell lymphoma. We report a patient with primary bilateral adrenal diffuse large B-cell lyphoma who achieved complete remission with R-CHOP (rituximab-cyclophosphamide, doxorubicin, vincristine, and prednisone) chemotherapy. A 52-yr-old man presented with fever and progressive fatigue for 3 months. Computed tomography (CT) scans of the abdomen and pelvis demonstrated large bilateral adrenal masses, and a needle biopsy of the left adrenal mass revealed diffuse large B-cell lymphoma. After 6 cycles of R-CHOP chemotherapy, CT scans showed no residual disease. To our knowledge, this is the second report to date of a patient with primary bilateral adrenal diffuse large B-cell lymphoma who achieved complete remission using R-CHOP chemotherapy.     

急诊介入治疗子宫大出血1例

子宫大出血是妇产科的常见病，常会危及产妇生命。2004年6月我们采用子宫动脉栓塞术急诊治疗子宫大出血1例，报道如下。