急性发热性嗜中性皮病伴凝血因子Ⅹ缺乏症1例并文献复习

<正>急性发热性嗜中性皮病又称Sweet综合征,是一种少见的炎症性疾病,临床表现包括发热、白细胞计数升高、皮肤红斑性病变(丘疹、结节和斑块)和弥漫性浸润,其发病机制尚不清楚,可能与血液系统等恶性肿瘤、系统性红斑狼疮、干燥综合征,以及呼吸道、消化道感染、妊娠等及药物相关^[1-4]。同时,凝血因子Ⅹ缺乏症较少见,且因子活性与出血风险相关。在临床上以Sweet综合征为首发疾病伴凝血因子Ⅹ缺乏症罕见,其诊断有一定难度。     

骨髓增生异常综合征转为急性嗜酸粒细胞白血病1例并文献复习

目的观察急性嗜酸粒细胞白血病(AEL)的形态特征、遗传特征、免疫表型及分子标记特征以提高对AEL的认识。方法对我院收治的1例难治性血细胞减少伴多系发育异常(MDS-RCMD)转为AEL患者的病历资料进行回顾性总结并复习相关文献。结果该例MDS-RCMD患者12个月后转为AEL;骨髓原始细胞占10.4%,嗜酸粒细胞占70.8%,其中嗜酸性早、中、晚幼粒细胞占69.6%;外周血嗜酸粒细胞占13.5%;骨髓原始细胞伴有复杂染色体异常、CD34、CD117、HLD-DR、CD33、CD38、CD13等阳性表达;FI1L1/PDGFRα和ETV6/PDGFRα融合基因阴性。按AML治疗2个月后患者死亡。结论该例AEL患者FI1L1/PDGFRα和ETV6/PDGFRα基因重排阴性,伊马替尼治疗无效。     

骨髓增生异常综合征误诊一例

【病例】女,35岁。因皮肤淤斑12天入院。患者发病前从事个体制鞋业2年。查体:体温37·4℃,脉搏82/min,呼吸20/min,血压100/70mmHg。一般情况尚好,上下肢皮肤有散在淤斑,胸骨无压痛,心肺听诊未闻及异常,肝、脾、淋巴结未触及。查血白细胞8·9×109/L,中性粒细胞0·88,淋巴细胞0·12,红细胞4·12×1012/L,血红蛋白129g/L,血小板32×109/L。骨髓象示有核细胞增生明显活跃,原始细胞0·035,巨核细胞832个/片,产血小板型巨核细胞占0·18。骨髓象主要表现为巨核细胞数量显著增多伴成熟障碍,符合特发性血小板减少性紫癜(ITP)的诊断。予泼尼松等…     

长期缓解的急性白血病发生骨髓增生异常综合征一例

患者,女,59岁。因头昏、乏力20天,于1992年11月15日入院。查体:贫血外观,胸骨压痛( ),心肺正常,肝、脾肋缘下未及。血象:Ｈｂ59ｇ/Ｌ,ＷＢＣ24×109/Ｌ,ＢＰＣ76×109/Ｌ,分类可见幼稚细胞。骨髓示有核细胞增生明显活跃,以原始和幼稚淋巴细胞增生为主,占有核细胞088,ＰＯＸ阴性,幼稚细胞胞浆糖原染色呈粗颗粒状,巨核细胞共8个/(21ｃｍ×20ｃｍ),形态正常。铁染色:细胞外铁( ),细胞内铁(占有核红细胞)032。诊断为急性淋巴细胞白血病(ＡＬＬ)Ｌ2型。诱导治疗用长春新碱(ＶＣＲ)、柔红霉素(ＤＮＲ)和泼尼松(ＶＤＰ)联合方案,1个疗程达到完全缓…     

白塞病合并骨髓增生异常综合征一例

白塞病合并骨髓增生异常综合征一例郝长来王巨德韩淑芹患者，男，３８岁，于１９９４年８月１０日入院。１年前出现口腔粘膜及外阴溃疡，服用泼尼松后好转，但仍反复发作。２０天前再次复发，伴右膝关节疼痛、腰痛，无游走性，并伴腹泻、发热，无头痛、头晕。曾有青霉素、...     

原发性胆汁淤积性肝硬化合并骨髓增生异常综合征一例

本科发现罕见的原发性胆汁淤积性肝硬化合并骨髓增生异常综合征一例，报告如下。     

外周血中性粒细胞发育异常对骨髓增生异常综合征的诊断意义

通过检测29例MDS患者外周血(PB)和骨髓(BM)标本,检测39例正常人及48例与MDS有鉴别意义的其它疾病患者的PB标本。结果显示MDS组外周血中性粒细胞颗粒积分(G-score),Pelger畸形所占百分率(PPP)与正常对照组相比差异显著(P＜0.001)。MDS组PB、BM的G-score、PPP有显著相关性。MDS组PBG-score、PPP与骨髓粒系和总的病态造血程度有显著相关性。表明外周血G-score和PPP间接反映了骨髓病态造血的程度,在MDS诊断、鉴别诊断中与骨髓检查有相互补充的作用。     

Photodistributed pustular acute febrile neutrophilic dermatosis revealing an acute myeloid leukemia

Sweet syndrome is a rare inflammatory dermatosis that can be associated with various diseases, including leukemias. Physicians should be aware that a photodistributed clinical presentation of a pustular SS may reveal underlying malignancies, particularly hemopathies. If the hemopathy is known, recurrence lesions should be suspected of a relapse.     

Chronic recurrent annular neutrophilic dermatosis: A rare entity

We report a new case of chronic recurrent annular neutrophilic dermatosis in a woman. Through our observation, we aim to make the clinician aware of this rare entity, in order to consider it among the diagnostic hypotheses of annular dermatosis, with centrifugal, recurrent, and chronic evolution.     

Myelodysplastic syndrome transformed into B-lineage acute lymphoblastic leukemia: A case report

BACKGROUNDMyelodysplastic syndromes (MDSs) are a group of hematological diseases caused by expansion of an abnormal clone of hematopoietic stem cells. Primary MDS is a potentially premalignant clonal disorder that may progress to overt acute leukemia in 25%-50% of cases. However, most of these cases evolve into acute myeloid leukemia and rarely progress to acute lymphoblastic leukemia (ALL). Thus, transformation of MDS into B-cell ALL is rare.CASE SUMMARYA 58-year-old man was admitted to the hospital for reduced blood cell counts. Based on all the test results and the World Health Organization diagnosis and classification, the patient was finally diagnosed with ring-shaped sideroblastic MDS with refractory hemocytopenia due to multilineage dysplasia. We used red blood cell transfusions and other symptomatic support treatments. After 4 years, the patient felt dizziness, fatigue, and night sweats. We improved bone marrow and peripheral blood and other related auxiliary examinations. He was eventually diagnosed with B-lineage acute lymphocytic leukemia (MDS transformation).CONCLUSIONThe number of peripheral blood cells, type of MDS, proportion of primitive cells in bone marrow, and number and quality of karyotypes are all closely related to the conversion of MDS to ALL.     

Linear IgA/IgG bullous dermatosis successfully treated with omalizumab: A case report

Linear IgA/IgG bullous dermatosis (LAGBD) is a rare, autoimmune blistering skin disease. We report a case of LAGBD in a 70‐year‐old woman. All common treatments were discontinued due to side effects or lack of treatment response. The patient was successfully treated with omalizumab which cleared her lesions after three months.     

Hemorrhagic fever with renal syndrome complicated with aortic dissection: A case report

BACKGROUNDHemorrhagic fever with renal syndrome is caused by hantaviruses presenting with high fever, hemorrhage, and acute kidney injury. Microvascular injury and hemorrhage in mucus were often observed in patients with hantavirus infection. Infection with bacterial and virus related aortic aneurysm or dissection occurs sporadically. Here, we report a previously unreported case of hemorrhagic fever with concurrent aortic dissection.CASE SUMMARYA 56-year-old man complained of high fever and generalized body ache, with decreased platelet counts of 10 × 10⁹/L and acute kidney injury. The enzyme-linked immunosorbent assays test for immunoglobulin M and immunoglobulin G hantavirus-specific antibodies were both positive. During the convalescent period, he complained sudden onset acute chest pain radiating to the back, and the computed tomography angiography revealed an aortic dissection of the descending aorta extending to iliac artery. He was diagnosed with hemorrhagic fever with renal syndrome and Stanford B aortic dissection. The patient recovered completely after surgery with other support treatments. CONCLUSIONHemorrhagic fever with renal syndrome complicated with aortic dissection is rare and a difficult clinical condition. Hantavirus infection not only causes microvascular damage presenting with hemorrhage but may be risk factor for acute macrovascular detriment. A causal relationship has yet to be confirmed.     

急性白血病化疗后并发急性肿瘤溶解综合征

目的 探讨急性白血病患者化疗后并发急性肿瘤溶解综合征（ATLS）的临床特征、诊断、治疗及转归。方法 对10例化疗后发生ATLS的急性白血病患者化疗前后的各项血生化指标进行分析。结果 所有病例均有不同程度的血生化指标异常，主要表现为高尿酸血症、高钾血症、高磷血症、低钙血症和肾功能损害。10例患者中，1例死于急性肾功能衰竭，1例死于脑出血，2例死于高钾血症所致的心脏停搏。其余6例经及时治疗后继续原方案化疗，病情均获得缓解。结论 早期诊断并及时给予别嘌呤醇及静脉碱化尿液是治疗ATLS的关键。     

Disseminated infection by Fusarium solani in acute lymphocytic leukemia: A case report

BACKGROUNDIn recent years, the rate of immunosuppressed patients has increased rapidly. Invasive fungal infections usually occur in these patients, especially those who have had hematological malignances and received chemotherapy. Fusariosis is a rare pathogenic fungus, it can lead to severely invasive Fusarium infections. Along with the increased rate of immune compromised patients, the incidence of invasive Fusarium infections has also increased from the past few years. Early diagnosis and therapy are important to prevent further development to a more aggressive or disseminated infection.CASE SUMMARYWe report a case of a 19-year-old male acute B-lymphocytic leukemia patient with fungal infection in the skin, eyeball, and knee joint during the course of chemotherapy. We performed skin biopsy, microbial cultivation, and molecular biological identification, and the pathogenic fungus was finally confirmed to be Fusarium solani. The patient was treated with oral 200 mg voriconazole twice daily intravenous administration of 100 mg liposomal amphotericin B once daily, and surgical debridement. Granulocyte colony-stimulating factor was administered to expedite neutrophil recovery. The disseminated Fusarium solani infection eventually resolved, and there was no recurrence at the 3 mo follow-up.CONCLUSIONOur case illustrates the early detection and successful intervention of a systemic invasive Fusarium infection. These are important to prevent progression to a more aggressive infection. Disseminate Fusarium infection requires the systemic use of antifungal agents and immunotherapy. Localized infection likely benefits from surgical debridement and the use of topical antifungal agents.     

麻疹合并脑炎1例

患者男，35岁。因发热8d，皮疹5d，意识障碍及双下肢活动受限3d，于2007年2月25日收住入院。