Incidence of thrombocytopenia in infants born to mothers with idiopathic thrombocytopenic purpura

Abstract Neonatal thrombocytopenia related to maternal idiopathic thrombocytopenic purpura (ITP) is reportedly uncommon but may have severe complications. The present report reviews records of 15 infants born to mothers with ITP during a 10-year period, and the incidence of neonatal thrombocytopenia and the risk of hematological complications is examined. Severe thrombocytopenia (platelets < 50 000/μL) was seen in three infants despite successful therapy with high-dose gamma globulin prior to delivery, which elevated maternal platelet counts. Although the platelet counts of these three infants fell to < 10 000/μL, none had severe complications. Moreover, no infants required treatment such as adrenocorticosteroids, platelets transfusion, or high doses of gamma globulin. No maternal markers predicted the degree of neonatal thrombocytopenia. The risk of complications arising from neonatal thrombocytopenia is low, but careful observation is required for the thrombocytopenic newborn of ITP mothers even when the infant has no bleeding complications at delivery.     

Prednisone treatment of acute idiopathic thrombocytopenic purpura of childhood. Advantages

Idiopathic thrombocytopenic purpura (ITP), an acquired hemorrhagic disorder, is characterized by the abrupt onset of thrombocytopenia despite normal megakaryocytic productivity. An accumulating body of evidence, including the recent demonstration of elevated levels of platelet-associated IgG, points to an immune etiology of acute childhood ITP. Although spontaneous recovery occurs in 80-90% of patients within 4 months of diagnosis, hemorrhagic complications may occur. Considerable evidence exists which suggests the efficacy of corticosteroid use in this disorder. Several clinical studies have shown that steroids may shorten the time to platelet count recovery, thus reducing the time at risk for hemorrhagic complications. The authors conclude with treatment recommendations for childhood ITP which include a brief course of prednisone.     

儿童免疫性血小板减少症与幽门螺杆菌感染

目的探讨幽门螺杆菌(Hp)感染与儿童免疫性血小板减少症(ITP)发病的关系。方法应用酶联免疫法检测54例ITP患儿粪便Hp抗原,观察Hp抗原阳性与阴性患儿的临床表现、血小板减少程度及对治疗的反应。结果 54例患儿,Hp阳性率19%(10例),不同发病年龄患儿阳性率差异无显著性。47例急性ITP患儿中Hp阳性9例(19%),治疗后血小板恢复正常平均需7.3 d;38例Hp阴性患儿血小板恢复正常平均需5.1 d,两组间差异无显著性(P>0.05)。慢性ITP患儿Hp阳性率14%,与急性ITP差异无显著性。结论未发现ITP患儿Hp感染率高于一般人群;Hp阳性率与患儿年龄无明显相关;Hp感染不影响ITP患儿对治疗的反应。     

Chronic immune thrombocytopenic purpura in children: a survey of the canadian experience

BACKGROUND: Immune thrombocytopenic purpura (ITP) in children is a common pediatric bleeding disorder with heterogeneous manifestations and a natural history that is not fully understood. To better understand the natural history of chronic ITP and detect response trends and outcomes of therapy, we conducted a 10-year retrospective survey of children from age 1 to 18 years with a diagnosis of chronic ITP. RESULTS: Data on 198 patients from 8 Canadian Pediatric Hematology/Oncology centers were analyzed. The majority of patients were female (58%), and were previously diagnosed with acute (primary) ITP (85%). The age at diagnosis of chronic ITP ranged from 1.1 to 17.2 years with a mean of 8.2+/-4.4 years. Ninety percent of patients received some form of treatment. Untreated patients had a higher mean platelet count at diagnosis of chronic ITP (P=0.009) despite similarities in mean age at first presentation and mean duration of follow-up. Thirty-four (17%) patients underwent splenectomy. Splenectomized patients tended to be significantly older, had a lower mean platelet count at diagnosis of chronic ITP, and had a longer duration of follow-up. CONCLUSIONS: The results from this study are consistent with published reports.     

Platelet function in autoimmune (idiopathic) thrombocytopenic purpura

Platelets play an essential role in the formation of haemostatic plugs. The quantitative defect of platelets in autoimmune (idiopathic) thrombocytopenic purpura (ITP) can result in bleeding complications, but most ITP patients have platelets with normal or enhanced function. Platelets in ITP are large, young, so-called "stress" platelets with increased platelet-associated autoimmune antibody (immunoglobulin G). Young stress platelets are more functional platelets, and their presence may account for bleeding times in ITP patients that are shorter than would be predicted on the basis of the patients' (low) platelet counts. Some ITP patients have significant mucocutaneous bleeding with platelet counts >50 × 10⁹ l⁻¹; this may be due to qualitative platelet dysfunction (e.g. brought about by inhibitory antiplatelet autoantibodies).     

Intravenous gamma globulin therapy for thrombocytopenic purpura associated with active varicella infection

Thrombocypenic purpura was noted in a 3-year-old Japanese girl with an active varicella infection. The administration of intravenous gamma globulin (IVG) drastically improved bleeding manifestations as well as the active varicella infection. We believe that IVG should be the first choice of therapy for thrombocytopenic purpura associated with active infections.Abbreviations IVG intravenous gamma globulin - ITP idiopathic thrombocytopenic purpura     

Idiopathic thrombocytopenic purpura in adults

Immune thrombocytopenic purpura (ITP) is an organ-specific autoimmune disorder in which platelets opsonized by antiplatelet antibodies are destroyed by the reticuloendothelial system. As a result the peripheral blood platelet count is low; if sufficiently severe, it may lead to bruising and mucocutaneous bleeding. The disorder may occur in adults and in children; in the former the disease affects primarily females, whereas the childhood type affects the sexes equally. For most individuals the disorder is minor, requiring little treatment, but patients with more severe forms of the disease require therapy to elevate the platelet count to a safe level to prevent serious bleeding. Therapies include corticosteroids, intravenous immunoglobulin, splenectomy, and immunosuppressive agents. Because there have been few trials in ITP, there is little evidence to guide treatment, and all the treatments may have adverse effects in terms of morbidity and mortality. The American Society of Hematology and British Committee for Standards in Haematology guidelines provide a framework for management but nonetheless are based on little evidence. In this paper the authors review the management of ITP in adults, children, and pregnant women and highlight some of the novel therapies being explored in this disorder, in addition to some of the ongoing laboratory research exploring the mechanisms underlying the disease.     

Duration and morbidity of newly diagnosed idiopathic thrombocytopenic purpura in children: A prospective Nordic study of an unselected cohort

OBJECTIVE: To determine the duration of the risk period with platelet counts <20 x 10(9)/L and the frequency of bleeding episodes in unselected children with idiopathic thrombocytopenic purpura (ITP). STUDY DESIGN: We established a registry for patients with newly diagnosed ITP in the five Nordic countries, enrolling children aged 0 to 14 years with platelet counts <30 x 10(9)/L. Treatment centers prospectively reported presenting features, management details, and disease-related events during the first six months after diagnosis. RESULTS: At presentation (n=501), more than half of the children had a platelet count <10 x 10(9)/L, but only 15 (3.0%) had a hemorrhage requiring blood transfusion. During follow-up of 409 patients, thrombocytopenia resolved uneventfully in 277. A risk period was present in 376 cases. Among 283 with self-limiting ITP, 26 were at risk >1 month and 25 had 30 events. Among 93 patients with chronic ITP, 73 were at risk >1 month and 44 had 111 events. Events occurred with an average frequency of 0.39 per month at risk. Life-threatening hemorrhages did not occur in the first six months after diagnosis. CONCLUSION: Most children with ITP are at risk for serious bleeding for less than one month. Continuing severe thrombocytopenia is associated with little morbidity, bleeding episodes being infrequent and very rarely serious.     

Intravenous immunoglobulin for idiopathic thrombocytopenic purpura (ITP) in childhood

IgG-SRK (identical with Sandoglobulin) is a polyvalent IgG concentrate obtained by modified alcohol cryoprecipitation, including mild acidification at pH 4. This product was given in high doses intravenously for the treatment of six children with acute ITP, four children with intermittent ITP, and three children with severe chronic idiopathic thrombocytopenic purpura (ITP). An impressive initial response was observed in all patients, the extent of which may be of prognostic significance in acute ITP. Maintenance therapy was required in two of six patients with acute ITP, in three out of four patients with intermittent ITP, and in all of the patients with severe chronic ITP. In the cases of severe chronic ITP, the disease could not be adequately controlled over long periods of time, but bleeding episodes subsided or became considerably less frequent. Although little is known of the effects of IgG-SRK, possible mechanisms were discussed. It is emphasized that a new model has been discovered to study the interrelations between structure and function of human immunoglobulin molecules.     

PLATELET RELEASE DEFECT IN A CHILD WITH EHLERS-DANLOS SYNDROME

Nine children with chronic refractory immune thrombocytopenic purpura and moderate to severe episodes of bleeding were treated with Danazol in an attempt to increase their platelet counts and improve clinical hemostasis. Only one patient experienced an excellent response while a good response was obtained in another. Cessation of bleeding episodes was observed in two other children with only marginal increases in platelet counts. Four of the nine patients failed to show any clinical or hematological improvement. The drug was well tolerated but its efficacy in childhood ITP needs further evaluation.     

Danazol Therapy in Immune Thrombocytopenic Purpura

Nine children with chronic refractory immune thrombocytopenic purpura and moderate to severe episodes of bleeding were treated with Danazol in an attempt to increase their platelet counts and improve clinical hemostasis. Only one patient experienced an excellent response while a good response was obtained in another. Cessation of bleeding episodes was observed in two other children with only marginal increases in platelet counts. Four of the nine patients failed to show any clinical or hematological improvement. The drug was well tolerated but its efficacy in childhood ITP needs further evaluation.     

Management of idiopathic thrombocytopenic purpura

Idiopathic thrombocytopenic purpura is not an uncommon bleeding disorder with a prevalence of 40–80 per million children per year. Over the last six decades, the subject of ITP has attracted the attention of pediatricians and hematologists. It is one of the subjects which has many controversies because of its unpredictable course heralded by remission relapses, and chronicity with mortality in less than 1% of cases. In the present review only the controversies in the management of acute and chronic ITP have been reviewed as it interests most pediatricians. Management of intracranial hemorrhage (ICH), severe gastrointestinal hemorrhage and menorrhagia continues to still remain a challenge in spite of newer therapies.     

儿童难治性特发性血小板减少性紫癜的治疗进展

特发性血小板减少性紫癜(ITP)是一种自身免疫性疾病,是临床最常见的出血性疾病.治疗以糖皮质激素、免疫抑制剂、脾切除等非特异性手段为主,但副反应明显,且约1/3的患儿治疗无效,成为难治性ITP.儿童难治性ITP目前尚无特效根治约物及方法,治疗应个体化,治疗选择应根据血小板计数和出血状态而定.近年对ITP自身免疫发病机制的深入研究令许多新的定向免疫干预措施开始进入临床研究阶段,实施定向免疫干预将是今后ITP诊疗的方向.文章结合ITP的发病机制对这些进展作一概述.     

The clinical course of immune thrombocytopenic purpura in children who did not receive intravenous immunoglobulins or sustained prednisone treatment

OBJECTIVE: To demonstrate the result of watchful waiting without specific therapy in unselected children with acute immune thrombocytopenic purpura (ITP). STUDY DESIGN: Between May 1992 and October 1999, 55 consecutive children (aged 2 months to 16 years; 28 boys and 27 girls) with acute ITP did not receive intravenously administered immune globulin G (IVIG) or sustained prednisone treatment. Patients with extensive mucosal bleeding were given prednisone, 2 mg/kg/d, for 3 days. RESULTS: In 37 of 55 patients the initial platelet count was <10,000/microL. Ten of these patients had active mucosal bleeding. Five additional patients with bleeding had platelet counts between 10,000 and 20,000/microL. Four patients were given a 3-day course of prednisone. Chronic ITP occurred in 7 (13%) of the patients; 29 patients achieved remission within 6 weeks, and 19 patients, between 6 weeks and 6 months. No life-threatening bleeding occurred, and no patient died. CONCLUSION: Most children with severe thrombocytopenia do not have active mucosal bleeding. This management approach, which did not administer specific therapy, avoided side effects, reduced cost, and was effective.     

Idiopathische thrombozytopenische Purpura im Kindesalter

Idiopathic thrombocytopenic purpura (ITP) is a bleeding disorder characterised by shortened platelet survival. ITP is heterogeneous and is affected by endogenous and acquired factors. The diagnosis is one of exclusion, meaning that differential diagnosis is always important. Because of the unknown aetiology and the lack of clinical data collected from controlled prospective studies, the diagnosis and management of patients with ITP are controversial. The objective in prospective trials has been to alter the velocity of platelet increase. This study goal is frequently extrapolated to clinical situations, but no evidence has been found to show that a fast increase in the platelet count is clinically significant. The treatment of a patient with ITP is generally directed at preventing life-threatening haemorrhages, which are very rare in childhood. The goals of management and the study end-points in clinical trials should be re-evaluated. Important aspects are bleeding, quality of life for the patient and adherents, side effects of drugs and economic matters.     

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。     

Idiopathic thrombocytopenic purpura of childhood

Idiopathic thrombocytopenic purpura (ITP) is a benign hemorrhagic disorder characterised by peripheral thrombocytopenia and increased megakaryocytes in the bone marrow. The exact pathogenesis of ITP is not well understood. The adherence of viral induced immune complexes to the platelet membrane is thought to trigger the phagocytosis of damaged platelets by macrophages in the reticuloendothelial system. The role of platelet associated IgG in the pathogenesis of ITP is under investigation. Although spontaneous recovery is observed in 80–90% of patients, a short course of steroid therapy is recommended to reduce the duration of thrombocytopenia. The steroids however, have no influence on the course or outcome of the disease, and their possible role in reducing the incidence of intracranial hemorrhage (ICH) is unknown. Emergency management of patients presenting with signs and symptoms suggestive of ICH is essential to prevent the fatal outcome. Approximately 10–20% of patients develop chronic ITP. Splenectomy, considered the treatment of choice in these patients, is not always curative. The post-splenectomy sepsis also imposes a great risk for these individuals. Recent experience with intravenous immunoglobulin (IV IgG) treatment indicates that the splenectomy could safely be deferred, or even avoided in chronic ITP. The use of IV IgG in acute ITP is being investigated.     

Efficacy and safety of anti-D for immune thrombocytopenic purpura in children

This study was conducted in 20 children (16 males) (mean age 9.2 ± 4.34y) with immune thrombocytopenic purpura (ITP) to assess the response to anti-D immunoglobulin. Six patients had newly diagnosed ITP, 6 had persistent ITP and 8 had chronic ITP. The overall response rate was 70% (14/20). The median time to response was 3 days (1–13 days). Response to anti-D was not related to age, sex, severity of bleeding, platelet counts at presentation, ABO blood group, or prior steroid or IVIG response.     

Childhood immune thrombocytopenic purpura: diagnosis and management

Immune thrombocytopenic purpura (ITP) is an autoimmune disorder characterized by a low circulating platelet count caused by destruction of antibody-sensitized platelets in the reticuloendothelial system. ITP can be classified as childhood versus adult, acute versus chronic, and primary versus secondary. Persistence of thrombocytopenia defines the chronic form of the disorder. Secondary causes of ITP include collagen vascular disorders, immune deficiencies, and some chronic infections. This review focuses on the diagnosis and management of children who have acute and chronic ITP. Emphasis is placed on areas of controversy and new therapies.     

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.