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1.
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.  相似文献   

2.
《Current Paediatrics》2002,12(4):298-303
The most common cause of thrombocytopenia in childhood is immune. The diagnosis must be carefully considered, as there are no specific diagnostic tests. Most children have an acute disease with spontaneous remission within a few weeks. Although the platelet count may be very low, bleeding symptoms are rarely severe, and most often restricted to the skin and mucous membranes. Most children do not require active treatment, but can be managed with good advice, ongoing support and a 24-h contact point. Children with significant bleeding problems may be treated with oral steroids, reserving intravenous immunoglobulin for emergencies or to cover injuries and surgery. About 20% of children continue with thrombocytopenia beyond 6 months (chronic ITP), but expectant management can continue, treatment is rarely required. Splenectomy is rarely required and should be reserved for the very rare child with serious bleeding persisting beyond 6 or 12 months.  相似文献   

3.
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.  相似文献   

4.
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.  相似文献   

5.
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.  相似文献   

6.
ObjectivesTo analyse the characteristics of children with chronic ITP (chronic immune thrombocytopenia) in the Hospital Infantil Universitario Niño Jesús (HIUNJ) between 2003 and 2008. To also evaluate whether clinical variables as age, gender, initial platelet count, and treatment have any prognostic significance on the outcome of ITP.Patients and methodsData were retrospectively collected from 288 patients diagnosed with «Purpura and other haemorrhagic illnesses». Forty-two out of these 288 satisfied the criteria for «chronic ITP».ResultsTen patients out of 42 (23.8%) achieved remission with splenectomy, and 25 (almost 60%) achieved it without splenectomy (14 were complete remissions and 11 were partial remissions). Eight patients (almost 20% of patients with chronic ITP) had spontaneous remissions between 6 and 12 months from initial diagnosis. None of the clinical variables analysed were related to the outcome of the disease and the prognosis of the disease.ConclusionsAlmost 60% of children with chronic ITP achieve remission without treatment regardless of age, gender, initial treatment or platelet count. Splenectomy is one of the treatments with best results; however the high spontaneous recovery rate in children with cITP, the low percentage of bleeding, and the generally benign outcome should encourage delaying this as long as possible. As it is possible to have a remission between 6 and 12 months from the initial diagnosis, the term «chronic» should be reserved for patients with ITP lasting more than 1 year.  相似文献   

7.

1 Background

Immune thrombocytopenia (ITP) during childhood spontaneously remits in up to 80% of children. Predictors of remission are not well understood.

2 Procedure

We analyzed data from Intercontinental Cooperative ITP Study Group (ICIS) Registry II, a large prospective cohort of children with ITP, to investigate factors that might predict remission.

3 Results

In ICIS Registry II, 705 patients had data collected through 12 months following diagnosis, with 383 patients having data available at 24 months as well. Younger age and pharmacologic treatment at diagnosis were significantly associated with disease resolution at 12 and 24 months (P < 0.0001 for both) as was bleeding at diagnosis (P < 0.0001 and P = 0.0213, respectively). Gender and platelet count at diagnosis were not significantly correlated with remission. In the multivariable analysis, remission at 12 months was associated with younger age, higher bleeding grade at diagnosis, and treatment with a combination of intravenous immunoglobulin (IVIG) and corticosteroids at diagnosis. Only younger age and treatment with IVIG and steroids in combination at diagnosis were associated with remission at 24 months. Patients <1 year of age had the highest odds of achieving remission at both 12 months (OR 4.7, 95% CI: 2.0–10.6) and 24 months (OR 7.0, 95% CI: 2.3–20.8).

4 Conclusions

Younger age, bleeding severity at diagnosis, and initial treatment with a combination of corticosteroids and IVIG are associated with remission at 12 months in the ICIS Registry II. Patients <1 year of age have the highest likelihood of remission. The relationship of bleeding and treatment at diagnosis requires further study to clarify whether these are independent predictors of remission.  相似文献   

8.
Chronic immune thrombocytopenic purpura (ITP), defined as a platelet count of below 150 x 109/L persisting for more than 6 months from onset of illness, occurs in approximately 20% to 25% of children with acute-onset ITP. A small subset of these patients (approximately 5%) will manifest symptomatic, severe thrombocytopenia (platelet counts <20 x 109/L) at 1 year or longer following diagnosis, and may require splenectomy. Complete/partial response rates following splenectomy in children with primary chronic ITP are of the order of 70% to 75%; response rates are lower in children with secondary ITP and those with complex autoimmune cytopenias (e.g., Evans syndrome). Laparoscopic splenectomy is increasingly preferred over open splenectomy. Patients should be immunized with the pneumococcal, Haemophilus type b and meningococcal vaccines before splenectomy; the duration of postsplenectomy antibiotic prophylaxis using penicillin or an equivalent antibiotic is controversial but should be at least until 5 years of age and for a minimum of 1 year postsplenectomy. Some experts advocate life-long antibiotic prophylaxis. Treatment of postsplenectomy failures is a challenge; partial/complete remission rates are low, and multimodality therapy may be more efficacious than monotherapy. The presence of an accessory spleen should be sought and removal considered if present. The role of newer treatment modalities such as anti-CD 20 remains to be established.  相似文献   

9.
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.  相似文献   

10.
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.  相似文献   

11.
OBJECTIVE: To retrospectively review our institutional experience of adolescents with idiopathic thrombocytopenic purpura (ITP). STUDY DESIGN: Medical record review of all patients diagnosed with ITP between the ages of 10 and 18 years seen at our center from January 1976 to March 2000. RESULTS: Data were collected from 126 patients. Of the evaluable 110 cases, 63 (57%) satisfied the criteria for chronic ITP, 30 (27%) for acute ITP, and 17 (15%) were uncertain. Sex distribution and mean ages were similar in all 3 groups. Platelet count at presentation was higher in patients with chronic ITP. Splenectomy was performed in 24 patients, with 17 (77%) of 22 having normal platelet counts at last follow-up. Outcome for the nonsplenectomized patients with chronic ITP included normalization of platelet count (n = 4), minimal or no bleeding without treatment (n = 29), treatment for ongoing symptoms (n = 5), and unknown (n = 1). Two patients died, 1 from intracranial hemorrhage and 1 from Escherichia coli sepsis and pulmonary hemorrhage. CONCLUSIONS: Patients 10 to 18 years of age with ITP are more likely than younger children to have chronic disease. Many patients with ITP recover without drug therapy or need for splenectomy. ITP in adolescents shares features of both childhood and adult ITP.  相似文献   

12.
Childhood immune thrombocytopenic purpura: diagnosis and management   总被引:1,自引:0,他引:1  
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.  相似文献   

13.
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.  相似文献   

14.
To date, immunoglobulin preparations and corticosteroids are the most important therapeutic principles in idiopathic thrombocytopenic purpura (ITP). Initial treatment with high-dose immunoglobulins (HD-IgG) as well as the following prednisone therapy, however, were only of short lasting success in three children with newly diagnosed ITP. Recurrent bleeding tendency tempted to combine both drugs. A desired longterm elevation of platelet counts, however, was not achieved. Administration of Anti-Rho (D) to two of the patients was also disappointing. Splenectomy which could not be postponed any longer has already led to 17 and 21 months lasting remissions in 2 patients, respectively. The third patient who required monthly single doses of HD-IgG for 6 months after surgery, is in a partial remission since 27 months now. All types of drug administration--alone, consecutive or even combined--must be considered to defer splenectomy. As experienced in our patients, however, splenectomy still seems to be justified in case even such individually adapted therapeutic approaches fail.  相似文献   

15.
Coronavirus disease 2019 (COVID-19) vaccines rarely cause de novo immune thrombocytopenia (ITP) but may worsen preexisting ITP in adults. Whether severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) vaccines impact platelet counts and bleeding in children, adolescents, and young adults (C-AYA) with preexisting ITP is unknown. We report here the very limited effect of COVID-19 vaccination on platelet counts and bleeding in a single-center series of 2 C-AYA with ITP. No patient experienced worsening bleeding and only one child had a significant decrease in platelet count which improved spontaneously to her baseline without intervention. SARS-CoV2 vaccination was safe in C-AYA with ITP in this small cohort.  相似文献   

16.
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.  相似文献   

17.
Platelet-associated IgG (PAIgG) was quantitated in 33 children with immune thrombocytopenia and platelet counts less than 100 X 10(9)/liter using a simple radial immunodiffusion (RID) assay. Elevated PAIgG levels were found in 76% (16/21) of children with acute idiopathic thrombocytopenic purpura (ITP), 88% (7/8) of children with chronic ITP, and all four children studied with systemic lupus erythematosus and thrombocytopenia. Normal PAIgG values were found in children with the following disorders: malignancy and chemotherapy-related thrombocytopenia; ITP in remission (platelet counts greater than 150 X 10(9)/liter); various nonimmune hematologic disorders and juvenile rheumatoid arthritis, these children having normal platelet counts. In children with acute ITP, elevated PAIgG values at initial presentation fell to within the normal range when clinical remission occurred. The RID assay can be easily established in most hematology laboratories and has the advantage that solubilized "test" platelets used in the assay can be stored frozen prior to analysis. We conclude that this simple technique is of value in the evaluation of childhood thrombocytopenic states and yields results comparable to those reported using more complex antiplatelet antibody assays.  相似文献   

18.
PURPOSE: To ascertain characteristics of children with immune thrombocytopenic purpura (ITP) and intracranial hemorrhage (ICH). METHODS: The authors identified 75 published cases of ICH in children with ITP by review of the literature from 1954 to 1998. Data pertaining to the ICH was recorded for age, gender, time from diagnosis of ITP (to ICH), platelet count, head trauma or arteriovenous malformation, concomitant medications, associated infections, other bleeding manifestations, prior treatment, and outcome.RESULTS Sixty-two cases represented 6 months to 20 years of age; 65% of patients were female. The median time from the diagnosis of ITP to ICH was 32 days (range 0 days to 8 years). Fifty of 69 ICH cases (72%) occurred within 6 months of diagnosis, but only 7 (10%) occurred within 3 days of diagnosis. The platelet count was less than 10000/microL in 71.4% of the cases. Treatment prior to the ICH was primarily steroids but also included intravenous immune globulin (IVIG), splenectomy, and others (interferon, azathioprine, or vincristine). There was no difference in mortality of patients before (56%) or after (54%) 1980. CONCLUSIONS: A very low platelet count appears permissive but not sufficient for ICH to occur in children with ITP. ICH occurs more commonly in acute ITP but can occur years after diagnosis. A significant number of patients develop an ICH despite having already initiated steroid treatment of ITP.  相似文献   

19.
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.  相似文献   

20.
Background: Idiopathic thrombocytopenic purpura (ITP) is an autoimmune disorder with a variable clinical course. Methods: A retrospective analysis was carried out of ITP patients presenting to a pediatric hematology‐oncology department during a period of 20 years, with a focus on treatment and outcome. Results: One hundred and twenty‐four cases were recorded (mean patient age, 8.4 years). Forty‐nine children (39.5%) had platelet counts <10 000/µL at diagnosis. No episode of severe bleeding was observed. Peak incidence was observed during spring and summer. Respiratory infections proceeded in 58% of cases. Treatment consisted of i.v. immunoglobulin (IVIG) in 93 children at four dosing schedules. Sixteen children received corticosteroids, 10 children received anti‐D immunoglobulin and 14 received no treatment. Recovery was observed in 67% of children on IVIG and in 50% on anti‐D globulin. Eight patients did not respond initially and received corticosteroids. Three children with refractory thrombocytopenia received anti‐CD20 (rituximab). Fourteen children (11%) had persistent/chronic disease. In 10 of them recovery was observed in 13 months–8 years. Splenectomy was performed in six children with resistant/chronic disease. Conclusion: ITP has a benign course in the majority of cases. Anti‐D globulin can effectively be used as an alternative first‐line treatment. Rituximab can successfully be used in refractory cases, while splenectomy has currently limited indications.  相似文献   

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