¿Cuál es el pronóstico de la trombopenia inmunitaria crónica?

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.     

Prognostic implications for direct platelet-associated IgG in childhood idiopathic thrombocytopenic purpura

To determine the value of the direct platelet associated IgG (PAIgG) level as a prognostic indicator in childhood idiopathic thrombocytopenia purpura (ITP), 18 children with ITP were studied. Ten of the 18 had PAIgG levels measured at diagnosis, before any therapy. Of these 10 patients, six (Group I) had an acute course, with a mean initial platelet count of 15 X 10(9)/liter and a mean initial PAIgG level of 330.9 fg/plt. Four patients (Group II) had a chronic course, with a mean initial platelet count of 11 X 10(9)/liter and a mean initial PAIgG level of 38.3 fg/plt. There was no significant difference between the mean initial platelet count of Groups I and II (p greater than 0.10), but the initial PAIgG levels in those patients with an acute course were significantly higher than the levels in those patients with a chronic course (p less than 0.05). Of the original 18 patients, nine were splenectomized for chronic thrombocytopenia, with normalization of the platelet count in all instances. Of these splenectomized patients, five had platelet counts and PAIgG levels measured before and after splenectomy. All five had normal PAIgG levels following splenectomy. The PAIgG level is a good prognostic indicator for the clinical course of childhood ITP. A high PAIgG level suggests an acute course while a modestly elevated level suggests a chronic course. The PAIgG level normalizes in remission after splenectomy.     

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。     

High-Dose Intravenous Immunoglobulin as Single Therapy in a Child with Autoimmune Hemolytic Anemia

Between 1975 and 1992 450 children with idiopathic thrombocytopenic purpura (ITP) were diagnosed, and of those 100 (22%) developed the chronic form of the disease. Approximately half the patients with chronic ITP presented with mild to moderate hemorrhagic manifestations at the onset of purpura (30 cases) andlor later during the course of the disease (25 cases). The incidence of intracranial hemorrhage was 1 %, and the mortality rate due to overwhelming septicemia after splenectomy was also 1%. Overall one-third of the patients received no therapy; two-thirds of them went into spontaneous remission within 8 months to 8 years from the onset of ITP. Steroids given in conventional or high doses (51 cases) achieved a transient (if any) rise in platelet count, but in no case were steroids curative. Remission related to intravenous immune globulin (IVIG) therapy was noticed in 38.5% of the children (10 of 26) after variable courses. The response rate to splenectomy was 95.0%. Ultimately the long-term outcome in children with chronic ITP was as follows: remission, 58 cases (spontaneous, 30; after IVIG therapy, 10; after splenectomy, 18); hemostatic platelet values, 22 cases (spontaneous, 16; after IVIG, 5; after splenectomy, I). Thirteen children were lost in follow-up, and 7 remain thrombocytopenic but asymptomatic. These data indicate that chronic ITP in childhood runs a benign course in most cases and may remit with or without therapy euen several years from onset. Therefore, therapeutic intervention has to be individvalized, and splenectomy, which is not always safe, should be reserved for problematic cases that fail to respond to conventional therapeutic modalities.     

Clinical characteristics of chronic idiopathic thrombocytopenia in Chinese children

PURPOSES: Clinical course and treatment outcome of childhood chronic ITP are quite variable in the literature. We report in the current paper our observation on the clinical behavior of chronic ITP in Chinese children. PATIENTS AND METHODS: We performed a retrospective review (Jan. 1990 to Dec. 2000) of children having low platelet count (plt <150 x 10(9)/L) for more than 6 months without identifiable cause. The indication for treatment was plt < or =20 x 10(9)/L. Remission is defined as plt > or =150 x 10(9)/L. RESULTS: Thirty-four children were identified within these 11 years. Their median age at diagnosis was 6.7 years (range from 0.4 to 16.8 years). The M:F ratio was 16:18. Bone marrow aspiration was performed in 30/34 cases. The median plt count at presentation was 24 x 10(9)/L (range 2 to 135 x 10(9)/L). Fourteen of 34 (41%) children eventually achieved durable remission. The chance of remission at 5 years was 66.62% with a median follow-up time of 5.86 years (range 0.72 to 10.41 years). Concerning therapy, 17/34 (50%) required no treatment while for the remaining 17, treatment included steroid (n = 16), IVIG (n = 7) or splenectomy (n = 3). In spite of temporary improvement in most, treatment induced prolonged complete remission (plt >150 x 10(9)/L) in only 2 patients. Twenty of 31 tested had abnormal immune marker(s) at presentation but none evolved into specific autoimmune disease later on. There was no correlation between the remission status, response to treatment, and the presence of autoimmune markers. CONCLUSION: About half of our chronic ITP patients achieved remission within 5 years. Medical treatment does not seem to alter the natural course of the disease but induced a transient response in most cases. Positive autoimmune markers are common among chronic ITP patients and have no significance in predicting outcome.     

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.     

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 chronic immune thrombocytopenic purpura: unresolved issues

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.     

儿童慢性特发性血小板减少性紫癜的临床转归

目的总结儿童慢性特发性血小板减少性紫癜(cITP)的临床经过及主要临床特点,以期对儿童cITP的应对策略有所帮助。方法回顾性分析58例儿童慢性ITP的自发缓解情况与主要临床特点。结果(1)58例cITP患儿中,33例(56.9%)获得自发缓解,中位缓解时间1年;预计累计自发缓解率在诊断后6~12个月、2、3和4年内分别为40%,47%,47%,55%,无死于出血者。(2)儿童cITP的起病年龄、性别、前驱感染史、病初PLT、抗核抗体及病初对药物治疗的反应均与疾病转归无关(P≥0.05)。(3)急、慢性ITP患儿的最低血小板计数分布基本相同(P=0.156),轻、中度出血的分布没有差别(P=0.329),但cITP重度出血多于急性ITP,分别为13.8%和1.9%。结论儿童cITP中的部分病例也呈现自限性经过,起病时的临床表现及早期药物治疗对疾病转归无明显影响。     

Platelet antibody in prolonged remission of childhood idiopathic thrombocytopenic purpura

Evaluations were performed in 20 patients with childhood idiopathic thrombocytopenic purpura (ITP) who remained in remission longer than 12 months. The mean duration of follow-up from diagnosis was 39 months (range 17 to 87 months). Eleven patients (four girls) in group 1 had an acute course of ITP, defined as platelet count greater than 150 X 10(9)/L within 6 months of diagnosis. Nine patients (five girls) in group 2 had a chronic course, defined as platelet count less than 150 X 10(9)/L for greater than or equal to 1 year or requiring splenectomy in an attempt to control hemorrhagic symptoms. Mean age at diagnosis and duration of follow-up were similar for both groups. Platelet count and serum (indirect) platelet-associated IgG (PAIgG) levels were normal in all 20 patients at follow-up. Both direct and indirect PAIgG levels were measured using a 125I-monoclonal anti-IgG antiglobulin assay. All had normal direct PAIgG levels, except for one patient in group 1 who had a borderline elevated value of 1209 molecules per platelet. These data suggest that the prevalence of elevated platelet antibodies is low during sustained remission without medication in patients with a history of childhood ITP. These data may be relevant for pregnant women with a history of childhood ITP, with regard to the risk of delivering an infant with thrombocytopenia secondary to transplacental passage of maternal platelet antibody.     

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.     

Chronic childhood idiopathic thrombocytopenia purpura: long-term follow-up

An understanding of the natural history of childhood chronic idiopathic thrombocytopenia purpura (ITP) could contribute to a rational therapeutic approach to its treatment, which remains controversial. In our retrospective study of 92 children with ITP, 22 had a chronic course and were followed for 3–14 years (median 8.6 years). Treatment, when indicated, was individualized: 4 patients (18.2%) did not receive any treatment, 14 (63.6%) received steroids only, while 4 (18.2%) were treated with steroids and one of the following: high-dose gamma globulin (4 patients), splenectomy (2 patients) or immunosuppressive therapy (2 patients). During follow-up, 14 patients (63.6%) achieved complete remission, 5 (22.7%) partial remission and only 3 (13.5%) remained severely thrombocytopenic, with minimal bleeding tendency. Eleven patients (50%) responded to the initial prednisone course (1–5mg/kg/day), but showed a marked decrease in platelet count when steroids were tapered off. In view of the high rates of complete and partial remission and the mild course of the few non-responding patients, it is suggested that with adequate supportive therapy, follow-up problems and fatalities can be kept to a minimum. We believe that aggressive therapy, such as splenectomy, should be reserved for the rare symptomatic and severely thrombocytopenic patient.     

Pulsed high-dose dexamethasone therapy in children with chronic idiopathic thrombocytopenic purpura

The effectiveness of pulsed high-dose oral dexamethasone therapy in children with refractory chronic idiopathic thrombocytopenic purpura (ITP) is evaluated. Thirteen children with severe chronic ITP were enrolled in the study from an outpatient pediatric hematology clinic (ages 2-14 years), 5 boys and 7 girls. They did not maintain a response to other forms of therapy (IVIg, Anti-D, conventional steroids, danazol) and one girl relapsed after splenectomy. Dexamethasone was administered orally at a dosage of 40 mg/M2/day (maximum 40 mg/day) for 4 consecutive days. The cycle was repeated once a month for 6 months. The immediate response to therapy was excellent as the mean platelet count at day 1 was 15 x 10(9)/L, while mean platelet count at day 4 was 158 x 10(9)/L. At the end of 6 cycles 3 patients maintained a platelet count of >150 x 10(9)/L and 4 patients showed partial response. At the end of the first year and second year (12 and 24 months after onset of treatment) 3 patients still had complete response, 3 patients had partial response, and 7 patients were failures. Six of the failures underwent splenectomy and one was shifted to dapsone, had no response, and refused splenectomy. Side effects were tolerable. They included bloating, nausea, vomiting, insomnia, anxiety, and depression, and transient glucosuria; however, they were not severe enough to discontinue the cycles. Mean duration of illness prior to start of dexamethasone was not significantly different in between responders and nonresponders. Dexamethasone given orally in high doses is an effective drug in achieving short-term platelet responses. Long-term remission is obtained in nearly half the patients with well-established chronic ITP. Its effectiveness in almost half the patients, minimal side effects, and low cost indicate that this treatment should be considered in patients with chronic ITP who do not tolerate the disease well before considering splenectomy.     

Guidelines for therapy of idiopathic thrombocytopenic purpura (ITP) in childhood

The term "idiopathic thrombocytopenic purpura (ITP)" comprises a rather heterogeneous group of diseases with different etiology and pathogenesis. 80% of the cases are acute forms following viral infections. Within the first 6 months the spontaneous remission rate is higher than 80%. The remaining patients develop an intermittent or chronic form of the disease. Lethal complications, commonly cerebral hemorrhage, are rare (less than 2%). Following a review of the established and the recent experimental therapeutic approaches, the authors try to give comprehensive therapeutical guide-lines for the management of the various clinical forms of the disease. If there is only a minor bleeding diathesis, it is recommended to withhold therapy for 2 to 3 weeks, irrespective of the platelet count. If no spontaneous remission occurs, we suggest therapy with corticosteroids. In case of failure of this therapy, infusions of high-dose immunoglobulins are advisable. Short-lasting successes may even be prolonged with further infusions (once a week or at longer intervals)--thus postponing splenectomy. In addition, a combination of high-dose immunoglobulins and corticosteroids may be effective. Following pneumococcal vaccination and penicillin prophylaxis, splenectomy should be performed not earlier than one year after diagnosis of ITP and only in children older than 5 years. If splenectomy does not lead to recovery of the platelet count, immunoglobulin infusions--possibly combined with corticosteroids--may be repeated. Finally, if all the above proposed therapeutic actions have failed, immunosuppressive drugs (azathioprime or vincristine) may be necessary.(ABSTRACT TRUNCATED AT 250 WORDS)     

Intracranial hemorrhage in immune thrombocytopenic purpura: a retrospective analysis

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.     

PULSED HIGH-DOSE DEXAMETHASONE THERAPY IN CHILDREN WITH CHRONIC IDIOPATHIC THROMBOCYTOPENIC PURPURA

The effectiveness of pulsed high-dose oral dexamethasone therapy in children with refractory chronic idiopathic thrombocytopenic purpura (ITP) is evaluated. Thirteen children with severe chronic ITP were enrolled in the study from an outpatient pediatric hematology clinic (ages 2-14 years), 5 boys and 7 girls. They did not maintain a response to other forms of therapy (IVIg, Anti-D, conventional steroids, danazol) and one girl relapsed after splenectomy. Dexamethasone was administered orally at a dosage of 40 mg/M2/day (maximum 40 mg/day) for 4 consecutive days. The cycle was repeated once a month for 6 months. The immediate response to therapy was excellent as the mean platelet count at day 1 was 15 &#50 10 9 /L, while mean platelet count at day 4 was 158 &#50 10 9 /L. At the end of 6 cycles 3 patients maintained a platelet count of >150 &#50 10 9 /L and 4 patients showed partial response. At the end of the first year and second year (12 and 24 months after onset of treatment) 3 patients still had complete response, 3 patients had partial response, and 7 patients were failures. Six of the failures underwent splenectomy and one was shifted to dapsone, had no response, and refused splenectomy. Side effects were tolerable. They included bloating, nausea, vomiting, insomnia, anxiety, and depression, and transient glucosuria; however, they were not severe enough to discontinue the cycles. Mean duration of illness prior to start of dexamethasone was not significantly different in between responders and nonresponders. Dexamethasone given orally in high doses is an effective drug in achieving short-term platelet responses. Long-term remission is obtained in nearly half the patients with well-established chronic ITP. Its effectiveness in almost half the patients, minimal side effects, and low cost indicate that this treatment should be considered in patients with chronic ITP who do not tolerate the disease well before considering splenectomy.     

Alpha-interferon therapy induces improvement of platelet counts in children with chronic idiopathic thrombocytopenic purpura

PURPOSE: To investigate alpha-interferon (IFN) therapy for children with chronic idiopathic thrombocytopenic purpura (ITP). PATIENTS AND METHODS: Patients with refractory ITP lasting more than 12 months from diagnosis were included if they had platelet counts <50 x 10(9)/L and had received no treatment during the past month. Patients received IFN (3 x 10(6) U/m2 per dose), three times per week for 4 weeks; if partial (<150 x 10(9)/L) or no response was obtained, the same dose was continued for another 8 weeks. In patients with favorable response and subsequent decrease to pre-treatment values, an additional 4 weeks of treatment could be administered. RESULTS: Fourteen patients (ages 4-20 y) receiving 17 IFN courses were included. Mean initial platelet count was 29 +/- 15 x 10(9)/L. A significant increase was achieved during 14 of 17 courses (82.4%). All but two responses were transitory, and platelets returned to initial values after IFN discontinuation (mean 44 +/- 26 days). Considering the best response achieved by each patient, we observed: 1) 10 patients who achieved a sustained improvement of platelet count throughout the treatment period, decreasing to initial values after therapy was stopped; 2) one patient who achieved platelet count >150 x 10(9)/L, remaining with normal platelets at 18 months; 3) one patient who achieved platelet count >150 x 10(9)/L, remaining with platelets between 100 and 140 x 10(9)/L at 48 months; 4) one patient who had no response; and 5) one patient in whom therapy worsened the thrombocytopenia. A mild to moderate flu-like syndrome and a moderate decrease of the absolute neutrophil count were the only side effects observed. CONCLUSION: Interferon therapy induces a significant increase of platelet count and seems to be a valid alternative therapy to attempt the achievement of prolonged remission in refractory ITP, to defer splenectomy in younger children, or to improve platelet count before planned splenectomy.     

Cost-effectiveness of splenectomy versus intravenous gamma globulin in treatment of chronic immune thrombocytopenic purpura in childhood

Infusions of intravenous gamma-globulin (IVGG) are an effective, nontoxic therapy for chronic idiopathic thrombocytopenic purpura (ITP) that would be more widely accepted if the therapeutic agent were not so expensive. The costs and outcomes of managing such children with splenectomy and IVGG were modeled with Markov processes. Children unresponsive to one treatment were considered to have received the alternative. The model accounted for spontaneous remissions, therapeutic responses, traumatic events, episodes of sepsis, and operative deaths. For a 10-year-old child with chronic ITP, the strategy of initial treatment with splenectomy had associated costs of $17,000 and a 97.9% ten-year survival rate, whereas the strategy of initial treatment with IVGG had associated costs of $21,000 but a 98.6% survival rate. Each additional life saved by employing the IVGG strategy cost $540,000, or $8,000 per year for a life expectancy of 70 years. Sensitivity analyses demonstrated that for older children the IVGG strategy continued to result in improved survival rates but was more costly than the splenectomy strategy. For younger children, the IVGG strategy dominated, with improved survival rates and lower costs.     

Chronic idiopathic thrombocytopenic purpura in children: case reports of spontaneous recovery without splenectomy

Children with chronic idiopathic thrombocytopenic purpura generally show a favorable outcome with a high spontaneous recovery rate even many years after the initial diagnosis. In this retrospective study, 5 out of 12 children with chronic ITP achieved a spontaneous recovery. A careful follow-up appears to be adequate for most of the patients, reserving splenectomy to the rare severely affected patients.     

Idiopathic thrombocytopenic purpura diagnosed during the second decade of life

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.