EB病毒相关性噬血细胞综合征1例

患儿,女,2岁3个月,因发热1.5个月,面色苍白1个月,牙龈出血2次入院。1.5个月前患儿首次在我院查EBV—IgM( );骨髓报告为增生性贫血骨髓象,予对症处理,无效,近3周出现弛张热,面色苍白进行性加重,自动出院。1周前牙龈出血2次入我院。既往史、个人史、家族史无异常。查体:血压10.5/7.0kPa,神志模糊,面色苍黄,全身轻度水肿,     

噬血细胞综合征1例

患儿 ,女 ,10月 ,发热 4ｄ。 4ｄ前开始无明原因发热 ,体温 37～ 40℃ ,有时呕吐 ,余无异常。既往史、个人史及家族史无异常。查体 :体温 38℃ ,一般状况好 ,面色苍黄 ,巩膜黄染不明显 ,无皮疹 ,双侧耳后、颈部及腹股沟区均可扪及数个淋巴结 ,直径 2～ 6ｍｍ ,活动度好 ,无触痛 ,咽稍红 ,心肺无异常 ,腹软 ,肝肋下 3～ 4ｃｍ ,剑下 2ｃｍ ,质韧 ,表面光滑 ,脾肋下3ｃｍ ,质韧 ,均无触痛 ,余无异常。予对症、支持疗法 ,仍持续发热 ,肝脾进行性增大 ,呕吐加重。血ＷＢＣ(1.0～ 1.5 )×10 9/Ｌ ,Ｎ 0 .41～ 0 .47,Ｌ 0 .34～ 0 .42 ,Ｍ 0 .11～…     

儿童感染相关性噬血细胞综合征10例

目的探讨儿童感染相关性噬血细胞综合征(IAHS)的临床及实验室特点。方法对10例IAHS的临床及实验室资料结合文献进行分析。结果小儿IAHS主要表现为高热,肝、脾、淋巴结大,多脏器损伤及快速进展的全血细胞减少,骨髓涂片可见吞噬血细胞的组织细胞。结论IAHS的临床及实验室特点类似于恶性组织细胞病,骨髓组织细胞形态学特点及细胞遗传学检测有助于两者的鉴别。     

采用噬血细胞综合征-04方案治疗噬血细胞综合征5例

目的观察噬血细胞综合征(HLH)-04方案治疗儿童HLH 5例的早期疗效。方法回顾性分析确诊为HLH患儿5例的临床特点,总结其对以鬼臼乙叉甙(VP16)为基础的免疫化学方案治疗的反应及转归。结果HLH患儿5例中4例在早期治疗阶段完全缓解(CR),CR时间17~22 d(平均18.8 d);另1例规则采用VP16治疗84 d未CR,改用鬼臼甲叉甙(VM26)于d130 CR;病例均存活9~11个月,目前仍CR。1例治疗14 d时出现手足震颤,怀疑为环孢素(CSA)不良反应,改用骁悉替代后手足震颤消失。结论儿童HLH可通过采用HLH-04方案的免疫化学治疗,早期得以有效控制。     

儿童伤寒并发噬血细胞综合征 1 例临床分析

目的分析儿童伤寒并发噬血细胞综合征(Ty-AHS)的临床特点和转归。方法回顾分析1例Ty-AHS患儿的临床资料,并复习相关文献。结果患儿,男,4岁,持续腹泻、高热与低体温交替、表情淡漠、肝脾肿大、急性腹膜炎表现、腹腔积液。血常规示嗜酸性粒细胞为0,血红蛋白、血小板明显下降,C反应蛋白、降钙素原明显升高,血浆纤维蛋白原下降至0.8 g/L,乳酸脱氢酶升高3 835 U/L,血清铁蛋白1 884 ng/mL,血三酰甘油2.42 mmol/L,血EBV-DNA滴度2.81×10~4/m L,血培养伤寒沙门菌血清型Ⅲb;腹部彩超提示肠系膜淋巴结增大,中量腹腔积液;胸片提示肺炎;淋巴细胞分析示CD4~+/CD8~+比值降低,CD3~-CD16~+56~+细胞降低,CD19~+细胞降低;骨髓细胞学检查,骨髓增生明显活跃,粒、红、巨三系均未见明显异常,全片见较多组织细胞及噬血组织细胞。给予抗感染,地塞米松治疗2周后患儿症状消失,体征和实验室检查渐恢复正常。结论儿童Ty-AHS是一种罕见并发症,起病急,进展快,抗生素加激素治疗有效。     

病毒相关性噬血细胞综合征1例报告

患儿 ,男 ,14岁 ,因发热 10ｄ ,浅表淋巴结肿大 3ｄ入院。 10ｄ前无明显诱因出现发热 ,呈稽留高热 ,最高达 4 0℃ ,无寒颤、头痛、抽搐、皮疹等症状。当时查血常规ＷＢＣ 2 .9× 10 9/Ｌ ,Ｎ 0 .2 3,Ｌ 0 .6 0 ,Ｍ0 .17,ＰＬＴ 16 1× 10 9/Ｌ ,门诊给予病毒唑静脉点滴 ,疗效欠佳 ,3ｄ前发现浅表淋巴结肿大。入院查体 :Ｔ 38.5℃ ,急性病容 ,全身皮肤未见黄染及皮疹 ,腋窝及腹股沟均可触及数个肿大淋巴结 ,压痛明显 ,活动度可 ,咽部充血 ,扁桃体Ⅱ度肿大 ,心肺检查未见异常 ,腹平软 ,肝肋下未触及 ,脾肋下约 1ｃｍ ,质中 ,无压痛 ,神经系统…     

重症肺炎相关性噬血细胞综合征30例临床分析

目的:探讨重症肺炎相关性噬血细胞综合征的病原、临床特点及预后,分析与死亡相关的危险因素。方法:回顾性分析2009年2月至2019年2月深圳市儿童医院收治的诊断重症肺炎相关性噬血细胞综合征患儿的临床资料,包括患儿的一般资料、病原学、临床表现、实验室检查、治疗和转归,对死亡组和生存组一般资料和实验室检查采用独立样本 ...     

Infection-Associated Hemophagocytic Syndrome Complicated by Infectious Lymphoproliferation: A Case Report

The case of a 7-year-old boy with virus-associated hemophagocytic syndrome (VAHS) and serologically proven parvovirus B-19 infection is described. The patient with VAHS presented with fever, hepatosplenomegaly, pancytopenia, and hyperlipidemia type IV. After induction therapy with VP-16 and prednisone, partial remission was achieved. Despite maintenance therapy, reinductions, and the addition of cyclosporine A for 3 months, several relapses occurred. The therapy was stopped because of life-threatening complications (Klebsiella sepsis, neutropenic enterocolitis, and stercoral peritonitis). The complications were treated successfully. The patient status was stabilized after splenectomy. However, hepatomegaly progressed slowly and the hyperlipidemia endured. Ten months after the diagnosis, leukocytosis with absolute T lymphocytosis appeared. Reactivation of VAHS was suspected and intravenous immunoglobin and then antilymphocyte immunoglobulin ALG therapy were started. The resultant decrease in leukocytosis was prompt, but lymphopenia did not occur. Virostatic treatment withfoscarnet was introduced based on human herpesvirus-6 seroconversion. Twenty-six months after the diagnosis, the patient is well, without any sign of VAHS or lymphoproliferation.     

Simultaneous Occurrence of Viral-Associated Hemophagocytic Syndrome and Langerhans Cell Histiocytosis: A Case Report

Langerhans cell histiocytosis (LCH) is a class I histiocytosis characterized by the presence of the pathologic Langerhans cell, an unique histiocyte. In contrast to LCH, class II histiocytosis is characterized by the proliferation of mononuclear phagocytes other than Langerhans cells and includes sinus histiocytosis with massive lymphadenopathy, viral-associated hemophagocytic syndrome, and familial hemophagocytic lymphohistiocylosis. Until now, these two classes have been considered separate, if related, entities. We report a 10-month-old girl who presented with pyrexia, hepatosplenomegaly, an edematous skin rash, anemia, thrombocytopenia, and a markedly elevated serum IgG and IgM antibody level to cytomegalovirus. Histologic proof of both hemophagocytosis in the liver and bone marrow and LCH in the skin was obtained at presentation. The clinical course and response to treatment over 6.5 years is recorded. Although the etiology of both class I and class II histiocytosis remains unknown, we speculate that the monocytic/macrophage disorder, as well as the LCH, were both triggered by virus or viral-related monokines secreted by activated macrophages.     

Reactive Hemophagocytic Syndrome

Two cases o f reactive hemophagocytic syndrome (RHS) are reported, and the clinical and pathological features are compared with other histiocytic proliferative disorders, including familial hemophagocytic lymphohistiocytosis (FHL) and malignant histiocytosis. RHS can be associated with a variety o f infections, including viral, bacterial, fungal, and parasitic. RHS may also be familial as exemplified by our 2 cases in siblings. The isolation of an effective agent appears t o be the only criterion by which a diagnosis o f RHS can be made.     

Wolfram综合征1例

患儿,男,13岁,因多饮、多尿、消瘦20 d,呼吸深长、神志恍惚5 h入院。既往体健,其父母及16岁姐姐体健。父母非近亲结婚。血糖63．3 mmol/L,尿糖 ,尿酮体 , 血pH 6．94,BE-25．9 mmol/L,以糖尿病并酮症酸中毒住院。查体:体温36．5℃,呼吸32次/min,血压12／8 kPa,身长 132 cm,体质量28 kg,神志恍惚,面色潮红,呼吸深长,皮肤干燥,无水肿。心肺查体无异常。腹软,肝肋下2 cm,质地中等,     

Bardet-Biedl综合征1例

1临床资料 患儿,女,9岁6个月.因肥胖、生长发育迟缓9 a,进行性行走困难4 a入院.患儿生后体形较肥胖,进行性加重,体格及智力发育较同龄儿明显落后,左手及双足六指趾畸形,有多饮多尿史.4 a前患儿无明显诱因出现进行性行走困难,逐渐出现x型腿.3 a前上学后发现视力较差,0.5 a前发现尿液有异味,无颜色及性状改变.患儿为第2胎,第2产,足月顺产,无窒息史.     

Landau-Kleffner综合征1例

患儿,男,10岁,右利手,第3胎,出生史正常,生后精神运动发育同同龄儿,家族史无异常。6岁时出现发作性摔倒,四肢抽搐伴意识丧失,发作频率为1、2次/d或2、3次/d,每次数分钟,脑电图有样放电,当地按癫治疗后抽搐控制,此后4年患儿无发作。6岁后其父母发现患儿渐对喊声无反应,仍可讲话,     

小婴儿Kasabach-Merritt综合征1例

Kasabach-Merritt 综合征（KMS）是以巨大血管瘤伴血小板减少和全身出血倾向为特征的一种综合征,由于有严重的血小板进行性消耗,可表现为全身出血倾向,往往发病急,短期内症状进行性加重,甚至威胁生命.KMS并不多见,易误诊或漏诊.现将本院1例KMS报告如下.