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.     

A prospective comparative study of 2540 infants and children with newly diagnosed idiopathic thrombocytopenic purpura (ITP) from the Intercontinental Childhood ITP Study Group

OBJECTIVE: To analyze prospectively the impact of age at diagnosis in childhood idiopathic thrombocytopenic purpura (ITP). STUDY DESIGN: International registry from June 1997 to May 2001, with analysis of data from baseline and 6-month-follow-up questionnaires. RESULTS: Data from 2540 patients were analyzed, including 203 infants (7.6%), 1860 children > or =1 to <10 years of age (69.1%), and 477 children and adolescents between > or =10 and <16 years of age (17.7%). The mean platelet count at diagnosis was similar in all three groups, as was the percentage of patients with initial platelet count <20x10(9)/L. The male/female ratio was highest in infants and decreased with age (P=.009). Immunoglobulin therapy was used more often in infants and corticosteroids in patients > or =10 years of age. Follow-up information at 6 months was available for 1742 children (68.6%). Chronic ITP was seen less frequently in infants (23.1%) than in children >10 years of age (47.3%, P<.0001). Intracranial hemorrhage occurred in 3 of 1742 children during the first 6 months after the diagnosis of ITP. CONCLUSIONS: Pediatric patients with ITP from infancy to adolescence exhibit heterogeneity in clinical, demographic, and treatment factors.     

Initial management of children with newly diagnosed idiopathic thrombocytopenic purpura in the Nordic countries

AIM: To describe the management practices of newly diagnosed childhood idiopathic thrombocytopenic purpura (ITP) in the Nordic countries. METHODS: A prospective registration was done from 1998 to 2000, including all children with newly diagnosed ITP aged 0-14 years and at least one platelet count < 30 x 10(9)/L. RESULTS: 506 children from 98 departments were registered. A diagnostic bone marrow aspiration was obtained within 14 days in 33%. Platelet and/or red blood cell transfusion was given in 11%. 287 children (57%) received platelet-enhancing therapy with intravenous immune globulin (IVIG) or corticosteroids within 14 days of diagnosis, IVIG being the first line choice in over 90% of the cases. There were noticeable national differences in the management. The decision to start drug treatment within two days of diagnosis was influenced mainly by the platelet count. Neither early treatment nor response to treatment changed the risk of chronic disease. CONCLUSION: This study has shown a great variation in the management practices of children with newly diagnosed ITP. Prospective studies are required to produce evidence-based recommendations for this patient group.     

Clinical standards in the reformed NHS.

A UK survey was carried out to discover the frequency, circumstances, and outcome of intracranial haemorrhage (ICH) complicating idiopathic thrombocytopenic purpura (ITP) of childhood. A questionnaire was circulated through the membership of the UK Paediatric Haematology Forum, and thence to local paediatricians and haematologists. It sought information on any child with ITP who had had an ICH during the 20 year period to January 1994. Fourteen instances were discovered, seven before 1984 and seven after. Six children survived the event with minimal or no sequelae, four without craniotomy. An immediately precipitating cause was noted in four; two had arteriovenous malformations and two suffered head injuries. The event occurred over two weeks from diagnosis in seven cases and over two months in five. All children were profoundly thrombocytopenic at the time of their intracranial bleed. By calculation the 14 children would have represented some 0.1% of the total with ITP during the period under review. ICH in childhood ITP may have a precipitating cause and is not necessarily fatal. There is no period of maximum risk, and it can occur at any time during the course of the illness when the platelet count is less than 10-15 x 10(9)/l. It is an extremely rare event and previous estimates of its incidence may have been too high.     

Intracranial haemorrhage in idiopathic thrombocytopenic purpura. Paediatric Haematology Forum of the British Society for Haematology

A UK survey was carried out to discover the frequency, circumstances, and outcome of intracranial haemorrhage (ICH) complicating idiopathic thrombocytopenic purpura (ITP) of childhood. A questionnaire was circulated through the membership of the UK Paediatric Haematology Forum, and thence to local paediatricians and haematologists. It sought information on any child with ITP who had had an ICH during the 20 year period to January 1994. Fourteen instances were discovered, seven before 1984 and seven after. Six children survived the event with minimal or no sequelae, four without craniotomy. An immediately precipitating cause was noted in four; two had arteriovenous malformations and two suffered head injuries. The event occurred over two weeks from diagnosis in seven cases and over two months in five. All children were profoundly thrombocytopenic at the time of their intracranial bleed. By calculation the 14 children would have represented some 0.1% of the total with ITP during the period under review. ICH in childhood ITP may have a precipitating cause and is not necessarily fatal. There is no period of maximum risk, and it can occur at any time during the course of the illness when the platelet count is less than 10-15 x 10(9)/l. It is an extremely rare event and previous estimates of its incidence may have been too high.     

Intracranial haemorrhage in idiopathic thrombocytopenic purpura. Paediatric Haematology Forum of the British Society for Haematology.

A UK survey was carried out to discover the frequency, circumstances, and outcome of intracranial haemorrhage (ICH) complicating idiopathic thrombocytopenic purpura (ITP) of childhood. A questionnaire was circulated through the membership of the UK Paediatric Haematology Forum, and thence to local paediatricians and haematologists. It sought information on any child with ITP who had had an ICH during the 20 year period to January 1994. Fourteen instances were discovered, seven before 1984 and seven after. Six children survived the event with minimal or no sequelae, four without craniotomy. An immediately precipitating cause was noted in four; two had arteriovenous malformations and two suffered head injuries. The event occurred over two weeks from diagnosis in seven cases and over two months in five. All children were profoundly thrombocytopenic at the time of their intracranial bleed. By calculation the 14 children would have represented some 0.1% of the total with ITP during the period under review. ICH in childhood ITP may have a precipitating cause and is not necessarily fatal. There is no period of maximum risk, and it can occur at any time during the course of the illness when the platelet count is less than 10-15 x 10(9)/l. It is an extremely rare event and previous estimates of its incidence may have been too high.     

Immune thrombocytopenic purpura in childhood in Norway: a prospective, population-based registration

A prospective, population-based registration of children with immune thrombocytopenic purpura (ITP) was performed in Norway in 1996 and 1997. Ninety-two cases were identified, indicating an incidence of 5.3 per 100,000 children under 15 years. The sex ratio (female/male) was 1.2/1. Fifty-six percent presented with cutaneous signs only. The lowest platelet count was < 20 x 10(9)/L in 91%. In spite of mild bleeding symptoms, medical treatment was given in 68%, in most cases (57/63) with intravenous immunoglobulin. A total of 41/44 patients with platelet counts of < or = 5 x 10(9)/L were treated, regardless of whether they had mucous bleedings or not. Eighteen percent had platelet counts < 150 x 10(9)/L at 6 months, and 9% at 12 months following diagnosis. One patient with therapy-resistant chronic ITP died 16 months after diagnosis from an anesthesia complication related to profound epistaxis. This study shows a relatively high incidence. As in other studies, there was a tendency to treat platelet counts rather than bleeding symptoms.     

Elective splenectomy in children with idiopathic thrombocytopenic purpura

PURPOSE: The aim of this study was to review the safety and efficacy of elective splenectomy in children with idiopathic (immune) thrombocytopenic purpura (ITP). METHODS: The authors reviewed the medical records of children with ITP treated with elective splenectomy at Children's Medical Center of Dallas since 1961. Indication for splenectomy was symptomatic thrombocytopenia unresponsive to medical management. RESULTS: Thirty-eight evaluable patients who had elective splenectomy for ITP were identified. Twenty-one (55%) were girls and 17 (45%) were boys. Twenty-two had splenectomy since January 1990. Age at diagnosis ranged from 6 months to 15.9 years (median 9 years), and age at splenectomy ranged from 3.6 to 16.4 years (median 11.8). Laparoscopic splenectomy was performed in 11 patients. No patient died and only one (2.6%) had postoperative hemorrhage. There were no other complications related to surgery. No cases of postsplenectomy sepsis were observed. At follow-up ranging from 1 month to 19.9 years (median 2.1 years), 29 patients (76.3%) had a normal platelet count (>150 x 109/L) and 4 (10.5%) had a platelet count between 50 and 150 x 109/L. Only two of the five (13.2%) remaining patients who continued to have a platelet count less than 50 x 109/L had hemorrhagic manifestations necessitating intermittent therapy with corticosteroids. CONCLUSION: Laparoscopic or open splenectomy is a safe and effective procedure for children with chronic or refractory ITP and should be considered when medical management fails or causes excessive toxicity.     

Does treatment of newly diagnosed idiopathic thrombocytopenic purpura reduce morbidity?

AIM: To explore whether early treatment of children with idiopathic thrombocytopenic purpura (ITP) with immunoglobulin and/or corticosteroids reduces subsequent morbidity. METHODS: Centres participating in a Nordic ITP study were divided according to whether they had treated more than 2/3, from 1/3 to 2/3, or less than 1/3 children within 14 days of diagnosis. The course of disease from 15 days to 6 months after diagnosis was compared for children managed at the three centre categories. The comparison was restricted to children in whom at least one platelet count <20x10(9)/l was measured, numbering 156, 143 and 84 in the three different categories, respectively. RESULTS: The three groups of children were clinically similar but were managed with initial treatment rates of 89%, 57% and 14%, respectively. By day 15, the platelet count had stabilised to >20x10(9)/l in 67%, 67% and 52% (p<0.05) and to >150x10(9)/l in 38%, 29% and 29% (p<0.20). At 1 month after diagnosis there was no difference in recovery rates. Chronic ITP developed in 27%, 22% and 25% in the three groups. During follow-up, one or more disease-related events occurred in 23%, 22% and 19%, with no difference in the average numbers of episodes with mucosal bleeding. Treatment courses were administered to 19%, 13% and 11%, respectively. CONCLUSION: Active treatment policies accelerated platelet recovery in children with short-lasting ITP but did not avert the development of chronic ITP and did not cause a reduction in morbidity during follow-up.     

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.     

Idiopathic thrombocytopenic purpura: a 10-year natural history study at the childrens hospital of alabama

Childhood idiopathic thrombocytopenic purpura (ITP) is a common disorder. However, single-institution, long-term, natural history data are limited. The objective of this paper is to review presenting features, response to therapy, and natural history of ITP treated at a single pediatric academic medical center. A retrospective chart review was made for all children (ages birth-18 years) diagnosed with ITP (ICD 287.3) and treated at the Childrens Hospital of Alabama/University of Alabama at Birmingham between 1993 and 2003. Four hundred nine patients were identified (49% male, 51% female; mean age: 5.85 years; range: 1 month-17 years). There was no seasonal variation of presentation. The mean platelet count was 19k (0-120k). Bone marrow aspiration (BMA) was performed in 72% but altered the diagnosis or therapy in no patient. Treatment consisted of corticosteroids in 256 (92% response), intravenous immunoglobulin (IVIG) in 125 (87% response), Win-Rho D in 58 (91% response), and no therapy in 71 (100% response). Response was defined as increase in platelet count to > 50k. There was no difference in response to any therapy. No patients died. One patient presented with a CNS hemorrhage at presentation, responded to therapy, and survived. Twenty-three of 409 patients (6%) experienced clinical bleeding requiring hospitalization or blood transfusion. Chronic ITP (persistence > 6 months) was noted in 99 patients (24%). Chronic patients presented at an older age (7.8 vs 5.2 years for acute only, p<0.001), and with higher platelet counts (27k vs 17k, p<0.001). The risk of chronic ITP was partially predicted by presenting platelet count > 50k and age > 10 years, or both; 50% of patients presenting with these features developed chronic ITP vs 24% overall rate. Splenectomy was curative in 30/31 (97%) patients. There was no postsplenectomy sepsis. Of 99 patients with chronic ITP, 25 responded to splenectomy, 37 resolved at a mean of 20.3 months after diagnosis (7-96 months), 36 had persistent mild thrombocytopenia (50k-125k), and 1 failed to respond to any treatment including splenectomy. Overall, 91% of cases resolved with therapy or observation. ITP is a common pediatric disease presenting at any age with low morbidity and mortality. Most cases can be managed by pediatricians without hematology referral. Several equally successful therapeutic options exist. Chronic cases present at an older age with higher platelet counts. Up to 50% of cases of chronic ITP will resolve with ongoing follow-up. The overall prognosis in childhood ITP is excellent.     

选择性脾切除术治疗儿童ITP的疗效探讨

目的探讨近年来儿童慢性特发性血小板减少性紫癜(ITP)患者行选择性脾切除术的有效性及安全性。方法收集1986年～2000年新华医院及上海儿童医学中心行选择性脾切除术治疗ITP的患儿资料,以术后血小板计数的稳定最低值判断疗效,回顾性研究其相关因素。结果16例慢性ITP患儿行选择性脾切除术,其中9例男孩,7例女孩。治愈7例(43.75%),好转5例(31.25%)。术后随访未有感染并发症发生。治愈患儿的术后血小板峰值均超过400×109/L,而其余患儿中仅2例超过400×109/L,经Fisher精确检验,两组间有显著差异(P<0.01)。结论选择性脾切除术是治疗儿童慢性ITP安全有效的方法。脾切除术后的疗效与术后血小板最高峰值相关,术后高的血小板计数峰值将提示着良好的预后。     

Post‐varicella thrombocytopenic purpura

Aims: The aim of the study was to characterize the clinical course of post‐varicella idiopathic thrombocytopenic purpura (ITP) and to asses the risk of acquiring ITP after varicella infection. Methods: A retrospective study of all children diagnosed with ITP in a tertiary medical centre during 1998–2008. Findings were compared with the Intercontinental Childhood ITP Study Group database. The risk of acquiring ITP after a varicella infection was assessed. Results: Ten children were diagnosed with post‐varicella ITP. The incidence of post‐varicella ITP was 1.9% amongst children diagnosed with ITP and 1.1% amongst children hospitalized for varicella. ITP was diagnosed, on average, 8.5 days after the onset of the varicella rash. The female‐to‐male ratio was 1:1.5. The average minimal platelet count was 9.5 × 10⁹ platelets/L. Post‐varicella ITP had an acute course in 80% of cases and a chronic course in the remaining 20%. Bleeding episodes occurred in three patients. During the follow‐up period, 11 patients with previously diagnosed ITP developed varicella. The infection had no apparent affect on the platelet count of the children with acute ITP, but caused a relapse in 71% of the patients with chronic ITP. Conclusions: Post‐varicella ITP has similar clinical features and course to non‐varicella associated ITP. The calculated risk of ITP as a complication of varicella infections is approximately 1:25 000.     

Long-term outcome of chronic idiopathic thrombocytopenic purpura in children

OBJECTIVES: Children with chronic idiopathic thrombocytopenic purpura (ITP) generally have a favorable outcome, but it is not known whether there are any prognostic factors to predict outcome. The objectives of this study were to assess the spontaneous remission rate and the prognostic significance of age, gender, initial platelet count, initial treatment, and response to treatment. METHODS: In this retrospective review of 62 consecutive children with chronic ITP, 37 were girls and 27 were 10 years of age or older (median age 9 years; range, 0.75-19). RESULTS: Thirty-five patients (56%) achieved spontaneous remission (remission without splenectomy), 30 of them (48%) within 4 years from diagnosis. Twenty-eight (45%) were complete remissions (platelet counts of >/=100,000) and 7 (11%) were partial remissions (50,000-99,000). There was no significant difference in the spontaneous remission rate between the younger (<10 years) and older children (55.8% vs. 57.1%, P = 0.95) or between boys and girls (56% vs. 56.7%, P = 0.98). Similarly, platelet count at initial diagnosis, initial therapy, or response to initial therapy did not have any prognostic significance. All 14 patients who underwent splenectomy achieved complete remission. CONCLUSIONS: More than 50% of children with chronic ITP achieve spontaneous remission. Age, gender, platelet count at initial diagnosis, initial treatment, and response to initial treatment do not have any prognostic significance toward the outcome of chronic ITP.     

Management of immune thrombocytopenic purpura: A prospective study of 147 children from the regional network RHémaP

AIM: Assessment of the impact of guidelines from a regional pediatric network to standardize the management of childhood immune thrombocytopenic purpura (ITP). MATERIALS AND METHODS: Consensus guidelines were drawn up in centers of the pediatric network for hematological diseases, RHémaP, and a cohort of children referred for ITP in these centers was set up. A 1-year follow-up was recorded for each patient over a 43-month period. RESULTS: We report data from a cohort of 147 children. At diagnosis, we recorded severe thrombocytopenia (median=8G/l) and 141 children had hemorrhagic symptoms (96%). Only 23 children had a bone marrow aspiration (BMA) at diagnosis (16.3%), which meant a high level of implementation of the RHémaP recommendations (96%) since indications of BMA were limited to rare indications. For 135 children (91.8%), treatment fulfilled the RHémaP guidelines that were mainly based on the platelet count: 121 received intraveinous immunoglobulin (IVIG) and 14 were not treated. Among those who received IVIG, 110 were good responders (91%) at the 96-h evaluation (platelet count greater than 20G/l), nine (7.4%) were poor responders, and 1 died of intracranial hemorrhage. At 6 months, chronic ITP was observed in 40 children (32.8%). Chronic ITP was associated with a higher platelet count at diagnosis and an older age (p<10(-3) and p=10(-3), respectively). CONCLUSION: The practices recorded over a 43-month period in our cohort fulfilled the RhémaP guidelines and we conclude that we managed to standardize regional practices for children with ITP. We observed conventional epidemiological characteristics in this cohort. Older children and higher platelet count at diagnosis were significantly associated with higher frequency of chronic ITP.     

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.     

Childhood idiopathic thrombocytopenic purpura in the Nordic countries: epidemiology and predictors of chronic disease

AIM: To describe the epidemiology of idiopathic thrombocytopenic purpura (ITP) in the Nordic countries, to define clinical subgroups and to investigate factors predicting chronic disease. METHODS: A prospective registration was done from 1998 to 2000, including all children with newly diagnosed ITP aged 0-14 y and at least one platelet count <30 x 10(9)/l. RESULTS: 506 children were registered and 423 followed for 6 mo. The incidence was 4.8/10(5) per year. Most children were aged 0-7 y (78%), with a predominance of boys, while patients aged 8-14 y had equal representation of the two sexes. There were seasonal variations determined by variations in postinfectious cases with sudden onset. The platelet count was <10 x 10(9)/l in 58%, but bleeding manifestations were mild or moderate in 97%. The insidious form (symptoms for more than 2 wk) was more frequent in older children and girls, showed little seasonal variation, had milder manifestations and ran a chronic course in more than half the cases. Intracranial haemorrhages did not occur in the first 6 mo after diagnosis. Chronic ITP developed in 25%. The strongest predictor of chronic disease was insidious onset of symptoms (OR 5.97). CONCLUSION: In the Nordic countries, ITP mainly affects children aged 0-7 y, with a winter bulk of postinfectious cases superimposed on a steady occurrence of non-infectious cases. Clinically, it may be useful to distinguish between children with sudden versus insidious onset of symptoms rather than between different age groups.     

Duration and morbidity of chronic immune thrombocytopenic purpura in children: five-year follow-up of a Nordic cohort

Aim: To describe the clinical course, morbidity and platelet recovery in an unselected Nordic cohort of children with chronic Immune Thrombocytopenic Purpura (ITP). Methods: Prospective 5‐year follow‐up of 96 children with ITP lasting more than 6 months, with reporting of hospital admissions, severity of bleeding episodes and stabilization of platelet counts above 20, 50 and 150 × 10⁹/L. Results: The estimated 5‐year recovery rate was 52%; exclusion of 12 splenectomized children did not change the estimate. Events eliciting admission to hospital occurred in 39 (41%). Major haemorrhages occurred in eight children (8%), including a nonfatal intracranial haemorrhage in one child (1%). The overall admission rate was 0.4/year of thrombocytopenia, decreasing during follow‐up as thrombocytopenia converted to milder degrees. Early recovery within 2 years of diagnosis occurred in 35%, was associated with low morbidity and was more likely in young children with abrupt onset of symptoms. Conclusion: In a Nordic cohort of children with chronic ITP, one half had recovered 5 years after diagnosis, more than half never required hospitalization and <10% experienced serious bleeding episodes, always with a platelet count <20 × 10⁹/L. Aggressive management can be restricted to the minority of children with continuing severe thrombocytopenia and frequent, clinically significant bleeding events.     

Inflammatory bowel disease associated with immune thrombocytopenic purpura in children.

OBJECTIVE: Previous reports suggest an association between inflammatory bowel disease (IBD) and immune thrombocytopenic purpura (ITP) in adults. To date, only five children with both diseases have been described. The aim of the study was to describe the characteristics of children with IBD and ITP. METHODS: Cases were obtained from the pediatric gastroenterology community by means of the pediatric gastroenterology internet bulletin board in June 1999. Eight cases were submitted from seven medical centers. Medical records were reviewed by two pediatric gastroenterologists and a pediatric hematologist. RESULTS: The age range of the patients was 2.1 to 16.5 years, with a mean age of 9.6 +/- 5.2 years. Four children had ulcerative colitis, three had Crohn disease, and one had indeterminate colitis. All had colonic involvement of IBD. Of eight patients, three had IBD first, three had ITP first, and two had both simultaneously. At ITP diagnosis, platelet count was less than 10,000/mL in five children, 17,000/mL in one child, and 50,000 to 60,000/mL in two children. Of the three children diagnosed with ITP first, two initially had rectal bleeding at the time of ITP diagnosis. Bone marrow evaluations, performed in six of eight children, were consistent with ITP. Six of the eight children had chronic ITP, including three children who were 5 years of age or younger. Therapy for ITP included steroids (n = 6), intravenous immunoglobulin (n = 6), Rh o (D) intravenous immunoglobulin (n = 2), and splenectomy (n = 1). CONCLUSIONS: The authors describe the largest pediatric case series of children with IBD and ITP. More than 50% of the children had the chronic form of ITP. Most patients responded to conventional therapy for ITP and IBD.     

Corticosteroids versus intravenous immune globulin for the treatment of acute immune thrombocytopenic purpura in children: a systematic review and meta-analysis of randomized controlled trials

OBJECTIVE: To compare the effectiveness of corticosteroids with intravenous immune globulin (IVIG) for the initial treatment of children with acute immune thrombocytopenic purpura (ITP). STUDY DESIGN: A systematic review and meta-analysis of randomized controlled trials comparing corticosteroids with IVIG. Studies were identified from eight electronic databases, meeting abstracts, expert consultation, and hand-searched reference lists. Two authors independently reviewed potentially eligible studies and extracted data. The number of patients with a platelet count >20,000/mm3, 48 hours after treatment initiation, was the primary outcome. Relative risks (RR) and risk differences were pooled using a random effects model, and numbers needed to treat (NNT) were calculated. RESULTS: A total of 1248 abstracts were reviewed, 55 articles were retrieved, and 10 studies were included. The RR (steroids vs IVIG) of achieving a platelet count >20,000/mm3 at 48 hours was 0.74 (95% CI: 0.65, 0.85), and the NNT was 4.55 (95% CI: 3.23, 7.69). CONCLUSION: Children treated with corticosteroids for acute ITP are 26% less likely to have a platelet count >20,000/mm3 after 48 hours of therapy, when compared with children treated with IVIG. Given the importance of low platelets in the pathogenesis of intracranial hemorrhage (ICH), this difference may hold important clinical implications.