6个月内年幼婴儿特发性血小板减少性紫癜23例临床分析

分析６个月内小婴儿特发性血小板减少性紫癜２３例,发现小婴儿病例出血部位单纯,以皮肤出血点,瘀斑为主,自然病程短,治疗效果好,故为治疗上应删繁就简。     

婴儿特发性血小板减少性紫癜巨细胞病毒感染临床观察

目的探讨巨细胞病毒(CMV)感染对婴儿特发性血小板减少性紫癜(ITP)发病的影响。方法对2004年6月-2009年12月我院收治的178例婴儿ITP患儿,检测血清CMV IgM或DNA,根据其阳性与否分为观察组和对照组,对发病年龄、入院前病程、发病诱因、出血程度、实验室检查及转归进行回顾性分析。结果观察组81例,中位年龄3个月;对照组97例,中位年龄6个月;两组前驱感染率分别为37%和42%,差异无显著性(P>0.05),前驱感染主要为上呼吸道感染。两组分别有72%和75%在发病前有预防接种史,两组差异无显著性(P>0.05)。两组临床出血程度比较差异无显著性(P>0.05)。入院时两组均有半数以上血小板计数<10.00×109/L,其均值分别为12.28×109/L和11.67×109/L,两者比较差异无显著性(P>0.05)。两组骨髓涂片巨核细胞数均正常或增多,并有成熟障碍和血小板生成不良;两组分别有86%(31/36)和81%(26/32)存在免疫球蛋白异常,差异无显著性(P>0.05)。经泼尼松和(或)静脉注射用丙种球蛋白治疗后,观察组有75例(93%)、对照组有93例(96%)血小板于2周内恢复正常,两组比较差异无显著性(P>0.05)。结论婴儿ITP中CMV感染在小月龄患儿中常见,可能是血小板减少性紫癜的诱发因素之一。CMV感染的ITP患儿其临床经过与非CMV感染者无明显差异。婴儿ITP无论有无CMV感染,均对肾上腺皮质激素或大剂量丙种球蛋白治疗有良好反应,但如何减少过度治疗有待于进一步研究。     

婴儿特发性血小板减少性紫癜临床特点分析

目的 分析婴儿特发性血小板减少性紫癜(idiopathic thrombocytopenic purpura,ITP)的特点.方法 从性别、临床表现、实验室检查、治疗效果等方面对77例ITP患儿进行观察和分析评价.结果婴儿组男性占75%,年长儿组男性占60%; 诱因为疫苗接种:婴儿组占28%,年长儿组占4%;诱因为感染...     

6个月内年幼婴儿特发性血小板减少性紫癜23例临床分析

分析６个月内小婴儿特发性血小板减少性紫癜（ＩＴＰ）２３例,发现小婴儿病例出血部位单纯,以皮肤出血点、瘀斑为主,自然病程短,治疗效果好,故认为治疗上应删繁就简。     

小剂量静注丙种球蛋白治疗儿童免疫性血小板减少性紫癜的疗效观察

为寻求应用静脉丙种球蛋白 (IVIG)治疗免疫性血小板减少性紫癜 (ITP)的最佳方法 ,将我院自 1 996年 1 1月以来收住的 ITP患儿随机分为两组 :A组应用小剂量 IVIG,即 0 .4g/ (kg·d)连用 2天 ;B组应用大剂量 IVIG,即 0 .4g/ (kg·d)连用5天。结果显示 :小剂量 IVIG与大剂量 IVIG治疗 ITP的疗效相当。小剂量 IVIG可以替代大剂量 IVIG作为 ITP患儿的急救方法     

Immune thrombocytopenic purpura as a presentation of childhood tuberculosis

Immune thrombocytopenic purpura as a presentation of childhood tuberculosis is a rare event and occasional reports are described in the pediatric literature. We describe a 8-yr-old girl with mediastinal tubercular lymphadenitis-induced immune thrombocytopenia, who was successfully treated with anti-tubercular drugs. We also review the published reports. Tuberculosis should be considered a cause of immune thrombocytopenia in areas where tuberculosis is highly endemic.     

Treatment of an infant with severe acute refractory immune thrombocytopenic purpura using combination therapy including rituximab

Some infants with acute immune thrombocytopenic purpura (ITP) do not respond to first‐line therapy, and currently there is no consensus on therapy for these refractory cases. We describe a 12‐week‐old infant with acute ITP who was unresponsive to intravenous immunoglobulin and corticosteroid, and developed gastrointestinal bleeding. Several combination therapies were unsuccessful. After four doses of rituximab followed by intravenous immunoglobulin and corticosteroid, his platelet counts gradually increased. Combined therapy which includes rituximab may be a promising treatment for severe acute refractory ITP. Pediatr Blood Cancer 2009;53:203–205. © 2009 Wiley‐Liss, Inc.     

Immunthrombocytopenic purpura as a model for pathogenesis and treatment of autoimmunity

In honour of Professor Rossi's 80th birthday we review the development of our understanding of the immune and auto-immune nature of the pathogenesis of immune thrombocytopenic purpura (ITP). The immune aspects have been documented by postviral alterations of the cellular and humoral immune system, by new methods of specific auto-antibody detection against platelet glycoproteins and by the therapeutic effect of administering immunoglobulin concentrate from healthy blood donors. The various possible mechanisms of action of immunoglobulin treatment have led to use of this treatment as an alternative for other immune-related disorders. The treatment of severe chronic ITP in children, however, remains unsatisfactory. With a new international clinical and laboratory study of children and adolescents with early chronic ITP we are continuing the investigation of the pathogenesis and treatment of ITP.     

小儿特发性血小板减少性紫癜的有关临床特点

目的探讨小儿特发性血小板减少性紫癜(ITP)的临床特点。方法对我院收治的255例ITP患儿的临床资料进行分析。结果1、男:女=1.43,中位年龄31个月,2岁以下占47.06%;急性型占91.37%,慢性型占8.63%。2、47.84%有前驱感染病史,31.76%在发病前1~4周有预防接种史。3、病原学检查阳性率73.81%,其中HPVB1945.24%。4、预防接种疫苗中乙肝疫苗34.57%,百白破疫苗24.69%,麻疹疫苗8.64%。5、临床表现94.12%以轻、中度皮肤粘膜出血为主,重度出血仅占5.88%。6、就诊时血小板数量:平均22.47×109/L,≤20×109/L占56.47%。7、骨髓常规涂片巨核细胞总数增多的占77.06%,分类中成熟无血小板产生的巨核细胞数>原始幼稚巨核细胞数>成熟有血小板产生的巨核细胞数>裸核巨核细胞数。8、给予以肾上腺皮质激素为主的治疗,97.42%血小板在2周内达正常,复发率4.29%。9、疫苗相关ITP的中位年龄6月,就诊时平均血小板数量22.3×109/L,95.06%患儿为轻中度出血;骨髓巨核细胞数增多者占75.68%;病原学检查阳性率为85.71%,其中HPVB19占64.29%;93.83%患儿治疗后平均4.90天血小板恢复正常水平,复发率3.7%。结论1、小儿ITP患者大多数为急性型,预后良好。2、病毒感染与小儿ITP关系密切,HPVB19在小儿ITP发病中有重要意义。3、疫苗相关的ITP发生率高于以往报道,除发病年龄小外临床特点与其他ITP相似,相关疫苗中以乙肝、百白破疫苗多见,应引起注意。4、HPVB19阳性患儿临床特点与一般ITP大致相同。5、以肾上腺皮质激素为主的治疗方案治疗小儿ITP疗效显著;大剂量丙种球蛋白和大剂量肾上腺皮质激素对有严重出血或血小板极低的患儿止血效果明显,可以避免血小板输注和相关死亡的发生。     

Intended management of children with acute idiopathic thrombocytopenic purpura: a national survey

OBJECTIVE: In Australia acute idiopathic thrombocytopenic purpura (ITP) is mainly treated by paediatricians (either general paediatricians or paediatric haematologists/oncologists). A survey was conducted to gauge the current practice of treating children with acute ITP in Australia. METHODS: All practising Australian paediatricians registered by the Royal Australasian College of Physicians were surveyed regarding their intended management of children with acute ITP. The questionnaire, adapted from a study of paediatric haematologists/oncologists in North America, presented four clinical scenarios of children with acute ITP with a platelet count of 3000 x 10(9)/L, with and without mucosal bleeding (wet and dry purpura, respectively). Questionnaires were returned by mail or filled in online at a dedicated webpage. RESULTS: Five hundred and sixty-three of 1097 (51%) paediatricians responded to the survey. Data from 140 who had treated at least one child with ITP in the previous 12 months were analysed. Respondents indicated that children with acute ITP are usually or always hospitalised (58-92%) and that 48% would be given active treatment, even with dry purpura. Various regimens of i.v. immunoglobulin or corticosteroids are used when treatment is administered. In comparing Australian and North American management of acute ITP there were many similarities, although Australian paediatricians were less likely to arrange a bone marrow aspirate if corticosteroids were prescribed. CONCLUSIONS: There is great variation in the intended management of children with acute ITP in Australia. Previously published management recommendations regarding investigation and treatment have had little impact on intended practice. Prospective studies are required to evaluate hypotheses so as to produce evidence-based recommendations for treatment of patients with acute ITP.     

T cell receptor V gene usage by CD4+ and CD8+ peripheral blood T lymphocytes in immune thrombocytopenic purpura

AIM: To identify T cell expansions, i.e. increased frequencies of T cells using a particular T cell receptor (TCR) V alpha or V beta gene segment, in patients with immune thrombocytopenic purpura (ITP). METHODS: The TCR repertoires of CD4+ and CD8+ peripheral blood lymphocytes of 16 patients with chronic ITP were analysed by staining with a panel of anti-TCR V alpha and V beta antibodies followed by flow cytometry. RESULTS: Four of the 16 patients exhibited a total of 6 expansions of CD8+ T cells using a particular V beta segment, but no expansions were detected in the CD4+ subset. For three of the expansions where a follow-up blood sample after treatment with intravenous immunoglobulin was available, only one expansion remained. CONCLUSION: Overall T cell expansion frequency was the same as in healthy individuals. However, the presence of expansions that normalized with treatment suggests the presence of specific T cells implicated in the pathogenesis of ITP.     

Kabuki make-up syndrome associated with chronic idiopathic thrombocytopenic purpura

Although susceptibility to infections in Kabuki make-up syndrome (KMS) has frequently been reported, there have been few immunological studies. We describe a 14 year old girl with KMS exhibiting chronic idiopathic thrombocytopenic purpura (chronic ITP), including immunological studies. Corticosteroid therapy was not effective therefore splenectomy was performed. The patient's platelet count increased transiently. Immunological studies revealed normal T cell functions and low serum immunoglobulin A (IgA) levels. Because of the abnormalities of B cell functions in chronic ITP and low serum IgA levels in our patient, we speculate that there may be some abnormalities of humoral immunity in KMS.     

Steroid-free interval with anti-D in chronic idiopathic thrombocytopenic purpura

Chronic idiopathic thrombocytopenic purpura (ITP) is an autoimmune disease characterized by the antibody-mediated destruction of platelets. To maintain the platelets above the symptomatic level we administered 100 μg of anti-D for 5 consecutive days to 19 children with ITP. Four patients did not respond to the treatment. Fifteen responded with an increase in the average platelet number to 76 000/μL 7 days postinjection. However, the platelet count dropped within 45 days to 27 000/μL. Three months after this study, two patients from the study group were then administered monthly anti-D after reinjecting anti-D daily for 5 consecutive days, as previously performed. Platelet levels in these two patients were maintained above 30 000/μL for 5 and 6 months respectively. We concluded that anti-D administration for 5 consecutive days can induce an increase in platelets followed by a decrease below 30 000/μL after 30–45 days. However, monthly administration of anti-D after daily injections for 5 consecutive days can keep platelets above the symptomatic level and may provide a corticosteroid-free safe interval for nearly 5 months.