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.     

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.     

Subcutaneous anti‐D treatment of idiopathic thrombocytopenic purpura in children

We investigated the effect of subcutaneous anti‐D IgG as platelet enhancing therapy in children with idiopathic thrombocytopenic purpura (ITP). Twenty‐three children were treated with subcutaneous anti‐D 50 µg/kg. The median platelet count increased from 7 × 10⁹ to 31 × 10⁹/L on day 3 (P < 0.01). The median decline in hemoglobin was 1.3 g/dl. Two children experienced minor fever and chills within 24 hr of treatment. Pain at the injection site was common but self‐limiting with no effect on activity level. These results suggest subcutaneous anti‐D IgG 50 µg/kg as an effective and well‐tolerated treatment option in childhood ITP. Pediatr Blood Cancer 2009; 53:1315–1317. © 2009 Wiley‐Liss, Inc.     

Neutropenia following IVIG therapy in pediatric patients with immune-mediated thrombocytopenia

It has been observed that some children with immune-mediated thrombocytopenia (ITP) who are treated with intravenous immunoglobulin (IVIG) experience a decline in their absolute neutrophil count (ANC). The aim of this study was to investigate the incidence of neutropenia following IVIG therapy in a large cohort of children with ITP. This retrospective comparative cohort study determined the incidence of neutropenia in 104 patients (110 treatment courses) admitted for ITP to the Children's Hospital of Philadelphia from January 2000 to October 2003. Post-treatment ANCs were compared between patients who received IVIG and patients who received anti-D immunoglobulin. The incidence of neutropenia in each group was analyzed using the Fisher exact test. Pretreatment ANCs were not significantly different between the two treatment groups (P = 0.72). Neutropenia (ANC < 1,500/microL), developed during 18 of 64 (28%) treatment courses with IVIG, compared with 0 of 46 (0%) treatment courses with anti-D immunoglobulin (P < 0.001). This study suggests that IVIG may cause neutropenia commonly in children with ITP. While this is likely to be a transient condition, its recognition may affect clinical decisions such as the need for a bone marrow examination.     

Initial Management of Childhood Acute Immune Thrombocytopenia: Single-Center Experience of 32 Years

Immune thrombocytopenia (ITP) is an acute self-limited disease of childhood, mostly resolving within 6 months irrespective of whether therapy is given or not. Treatment options when indicated include corticosteroids, intravenous immune globulin (IVIG), and anti-RhD immunoglobulin. We reviewed our 32 years’ experience for first-line therapy of acute ITP. Five hundred forty-one children (mean age: 5.3 years) diagnosed and treated for ITP were evaluated retrospectively. Among 491 acute ITP patients, IVIG was used in 27%, high-dose steroids in 27%, low-dose steroids in 20%, anti-D immunoglobulin G (IgG) in 2%, and no therapy in 22%. When the initial response (platelets >50 × 10⁹/L) to first-line treatment modalities were compared, 89%, 84%, and 78% patients treated by low-dose steroids, high-dose steroids, and IVIG responded to treatment, respectively (P > .05). Mean time to recovery of platelets was 16.8, 3.8, and 3.0 days in patients treated with low-dose steroids, high-dose steroids, and IVIG, respectively (P < .0001). Thrombocytopenia recurred in 23% of low-dose steroid, 39% of high-dose steroid, and in 36% of IVIG (P < .0001) treatment groups. Of 108 patients who were observed alone, 4 (3%) had a recurrence on follow-up and only 2 of these required treatment subsequently. Recurrence was significantly less in no therapy group compared with children treated with 1 of the 3 options of pharmacotherapy (P < .0001). Response rates were similar between patients treated by IVIG and low- and high-dose steroids; however, time to response was slower in patients treated with low-dose steroids compared with IVIG and high-dose steroids.     

Corticosteroid prophylaxis for neurologic complications of intravenous immunoglobulin G therapy in childhood immune thrombocytopenic purpura

To assess in a retrospective analysis if there is evidence suggesting corticosteroids can prevent the neurologic complications of intravenous immunoglobulin (IVIG) therapy in children with immune thrombocytopenic purpura (ITP). From March 1985 to September 1997, 112 children received IVIG (1 g/kg/day for one or two dosages) for the treatment of ITP. During the years 1990 to 1997, 23 children nonrandomly received a short course of prednisone (2 mg/kg/day during and for 3 days after the completion of IVIG therapy) as a prophylaxis against the neurologic complications of IVIG therapy. The authors analyzed the data of all 112 children and compared the incidence of neurologic complications in those who received prednisone prophylaxis with those who did not. The severity of the complications was assessed as follows: grade 1, headache only; grade 2, headache plus vomiting; grade 3, headache, vomiting, and fever; grade 4, headache, vomiting, fever, and meningeal signs (aseptic meningitis). Of the 23 children who received prednisone prophylaxis, 2 (8.7%) experienced headache and vomiting after the completion of prednisone prophylaxis. Of the 89 children without prednisone prophylaxis, 27 (30.3%) experienced neurologic symptoms of varying severity, including one patient with aseptic meningitis proven by examination of the spinal fluid. Twelve of these patients needed additional hospital care for the complications. Children receiving prednisone had a 78% lower risk of neurologic complications (OR = 0.22; CI = 0.05-0.90; P = 0.036). This retrospective study shows a short course of prednisone therapy, given during and until 3 days after the completion of IVIG infusion, is likely to decrease the incidence and severity of neurologic complications of IVIG in children with ITP.     

Intramuscular anti‐D in chronic immune thrombocytopenia children with severe thrombocytopenia

Nine patients with chronic immune thrombocytopenia and platelet counts <20 × 10⁹/L, with a median age of 7.8 (3.8–15.5) years, received three phases of 10 mcg/kg/dose of intramuscular anti‐D. Phase 1 was anti‐D daily for 5 days, followed by phase 2, anti‐D weekly for 12 weeks and withheld when platelet counts ≥20 × 10⁹/L, and then phase 3 was anti‐D once every 2 weeks for 24 weeks. According to the International Working Group criteria, in phase 1, 66.7% of patients responded to the treatment. In phases 2 and 3, 11.1% (0–41.7%) and 7.7% (0–33.3%) of total episodes of follow up, respectively, responded to the treatment. Therefore, intramuscular anti‐D given at a dose of 10 mcg/kg for 5 days is an alternative method to raise platelet counts in chronic immune thrombocytopenia children with severe thrombocytopenia where the intravenous form of anti‐D is not available.     

Diabetes and immune thrombocytopenic purpura: a new association with good response to anti‐CD20 therapy

Type 1 diabetes (T1D) is rarely a component of primary immune dysregulation disorders. We report two cases in which T1D was associated with thrombocytopenia. The first patient, a 13‐year‐old boy, presented with immune thrombocytopenia (ITP), thyroiditis, and, 3 wk later, T1D. Because of severe thrombocytopenia resistant to immunoglobulins, high‐dose steroids, and cyclosporine treatment, anti‐cluster of differentiation (CD20) therapy was introduced, with consequent normalization of thrombocytes and weaning off of steroids. Three and 5 months after anti‐CD20 therapy, levothyroxin and insulin therapy, respectively, were stopped. Ten months after stopping insulin treatment, normal C‐peptide and hemoglobin A1c (HbA1c) levels and markedly reduced anti‐glutamic acid decarboxylase (GAD) antibodies were measured. A second anti‐CD20 trial for relapse of ITP was initiated 2 yr after the first trial. Anti‐GAD antibody levels decreased again, but HbA1c stayed elevated and glucose monitoring showed elevated postprandial glycemia, demanding insulin therapy. To our knowledge, this is the first case in which insulin treatment could be interrupted for 28 months after anti‐CD20 treatment. In patient two, thrombocytopenia followed a diagnosis of T1D 6 yr previously. Treatment with anti‐CD20 led to normalization of thrombocytes, but no effect on T1D was observed. Concerning the origin of the boys' conditions, several primary immune dysregulation disorders were considered. Thrombocytopenia associated with T1D is unusual and could represent a new entity. The diabetes manifestation in patient one was probably triggered by corticosteroid treatment; regardless, anti‐CD20 therapy appeared to be efficacious early in the course of T1D, but not long after the initial diagnosis of T1D, as shown for patient two.     

NK细胞在儿童免疫性血小板减少症发病和治疗中的意义

目的：探讨自然杀伤细胞（NK细胞）在儿童免疫性血小板减少症（ITP）的发病和治疗中的意义。方法采用流式细胞仪分别检测62例新诊断ITP患儿、43例持续性ITP患儿、21例慢性ITP患儿和51例对照组儿童外周血NK细胞百分比;并观察单独使用标准剂量静脉注射用人免疫球蛋白（IVIG）对NK细胞百分比正常及减少的新诊断的ITP患儿的疗效。结果新诊断ITP患儿、持续性ITP、慢性ITP患儿NK细胞百分比均较正常对照组儿童显著降低（P＜0.05）,但三组ITP患儿NK细胞百分比差异无显著性（P ！0．05）;NK细胞百分比正常的新诊断ITP患儿单独使用IVIG有效率为92．86％（26/28）,NK细胞百分比降低的新诊断ITP患儿单独使用IVIG有效率仅为14．70％（5/34）。结论 NK细胞表达变化与ITP发病存在一定关系,同时NK细胞百分比正常的新诊断ITP患儿可首选IVIG治疗。     

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.     

Syndrome of inappropriate anti‐diuretic hormone in Kawasaki disease

Background: The pathogenesis of hyponatremia in acute Kawasaki disease (KD) remains unclear. A recent case report of KD complicated by syndrome of inappropriate anti‐diuretic hormone (SIADH) led us to determine the prevalence of SIADH in acute KD patients. Methods: Subjects were 39 Japanese KD patients (2–84 months of age, 25 males and 14 females) treated with intravenous immunoglobulin (IVIG), 2 g/kg/day and oral aspirin. SIADH was defined when hyponatremic patients (serum sodium concentration <135 mEq/L) had decreased serum osmolality <280 mOsm/kg H₂O, elevated urine sodium concentration >20 mEq/L and elevated urine osmolality >100 mOsm/kg H₂O without dysfunctions of renal, thyroid or adrenal gland. We also studied the relation between clinical course of SIADH and the amount of infused fluid during IVIG. Results: Before IVIG, 27 patients (69%) had hyponatremia and 11 (28% of total; 41% of hyponatremic patients) had SIADH while after IVIG, 13 (33%) hyponatremia and four (10%; 31% of hyponatremic patients) SIADH. Among 11 patients with SIADH before IVIG, SIADH improved in 10 after IVIG, but hyponatremia persisted in five. Significant correlation was observed between serum sodium concentration after IVIG and infusion amount in SIADH patients (r=?0.64, P= 0.03), but not in non‐SIADH patients. Conclusions: This is the first report to show that SIADH is common as a cause of hyponatremia in acute KD and hence careful management of water and sodium is warranted.     

大剂量短疗程泼尼松治疗儿童急性免疫性血小板减少症的疗效观察

目的观察大剂量短疗程泼尼松(Pred)疗法对儿童急性免疫性血小板减少症(ITP)的疗效。方法 162例ITP患儿根据治疗方法不同随机分为大剂量静脉丙种球蛋白+甲基泼尼松龙组(IVIG+MP)、静脉丙种球蛋白组(IVIG)、甲基泼尼松龙组(MP)与Pred组。IVIG+MP组41例,采用IVIG(1g/kg,共1次)+MP[10 mg/(kg.d),每3天减半量,共9 d]冲击治疗,继之口服Pred[1.5～2.0 mg/(kg.d)],并逐渐减量维持治疗;IVIG组39例,采用丙种球蛋白(1 g/kg,共1次)冲击治疗,继之口服Pred[1.5～2.0 mg/(kg.d)]并逐渐减量维持治疗;MP组40例,采用MP[10 mg/(kg.d),每3天减半量,共9 d]冲击治疗,继之口服Pred[1.5～2.0 mg/(kg.d)]并逐渐减量维持治疗;Pred组42例,采用口服Pred[4 mg/(kg.d),共4 d]治疗后停药,无减量维持治疗。比较各组治疗前后血小板数、治疗有效率、不良反应发生率及药费支出。结果各治疗组治疗前后血小板数及治疗有效率差异无显著性,IVIG+MP组、IVIG组、MP组治疗不良反应发生率及药费支出均高于Pred组。结论大剂量短疗程Pred疗法治疗儿童急性ITP能有效提升血小板计数,有效率与IVIG及MP冲击治疗相比差异无显著性,不良反应少,花费低。     

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.     

Clinical course of children with immune thrombocytopenic purpura treated with intravenous immunoglobulin G or megadose methylprednisolone or observed without therapy

The authors compared the prognosis in 50 children with acute immune thrombocytopenicpurpura (ITP) who received intravenous immunoglobulin G (IVIG), megadose methylprednisolone (MDMP), or no therapy. Twenty-six children were observed with no therapy, 12 children received IVIG, and 12 children received MDMP. The percentage of the patients whose platelet counts increased at a level of > 20 x 10(9)/L and > 50 x 10(9)/L at 3 days after starting therapy was significantly higher in both IVIG and MDMP groups than in the no therapy group (p < .01), but there was no significant difference at 10 and 30 days after initiation between the 3 groups (p > .05 in each comparison). This result suggested that therapy does not increase the rate of recovery but shortens the duration of thrombocytopenia in the first days. Management derision in ITP is made on clinical condition rather than on platelet count and no treatment options is to be preferred even in the face of mucosal bleeding. If the patient has extensive bleeding and the decision is to treat, both IVIG and MDMP are equally effective in providing a safe platelet level early on.     

CLINICAL COURSE OF CHILDREN WITH IMMUNE THROMBOCYTOPENIC PURPURA TREATED WITH INTRAVENOUS IMMUNOGLOBULIN G OR MEGADOSE METHYLPREDNISOLONE OR OBSERVED WITHOUT THERAPY

The authors compared the prognosis in 50 children with acute immune thrombocytopenic purpura (ITP) who received intravenous immunoglobulin G (IVIG), megadose methylprednisolone (MDMP), or no therapy. Twenty-six children were observed with no therapy, 12 children received IVIG, and 12 children received MDMP. The percentage of the patients whose platelet counts increased at a level of > 20 &#50 10 9 /L and > 50 &#50 10 9 /L at 3 days after starting therapy was significantly higher in both IVIG and MDMP groups than in the no therapy group ( p < .01), but there was no significant difference at 10 and 30 days after initiation between the 3 groups ( p > .05 in each comparison). This result suggested that therapy does not increase the rate of recovery but shortens the duration of thrombocytopenia in the first days. Management decision in ITP is made on clinical condition rather than on platelet count and no treatment options is to be preferred even in the face of mucosal bleeding. If the patient has extensive bleeding and the decision is to treat, both IVIG and MDMP are equally effective in providing a safe platelet level early on.     

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。     

A cost-utility analysis of treatment for acute childhood idiopathic thrombocytopenic purpura (ITP)

BACKGROUND: The primary objective in the treatment of acute pediatric idiopathic thrombocytopenic purpura (ITP) is to rapidly increase the platelet count. METHODS: We built a decision analytic model to evaluate the cost-utility of four commonly used treatment strategies: intravenous immunoglobulin G (IVIG) 0.8 g/kg, anti-D 75 mcg/kg, methylprednisolone (30 mg/kg for 3 days), and prednisone (4 mg/kg/day for 4 days). In our baseline model, all children were hospitalized upon presentation, and discharged once the platelet count reached > or =20,000. We performed a literature search to estimate time to platelet count > or =20,000 for each strategy, as well as the probability of side effects. We obtained cost data and quality of life measures from institutional and published data sources. RESULTS: Total cost of one-time treatment for a 20 kg child was US dollars 786 with prednisone, US dollars 1,346 with methylprednisolone, US dollars 2,035 with anti-D, and US dollars 2,492 with IVIG. The strategies of IVIG and methylprednisolone were less effective and more expensive than anti-D and prednisone, respectively. Although anti-D caused the most rapid rise in platelet counts, the incremental cost-utility ratio (costs incurred by using anti-D instead of prednisone divided by health benefit of using anti-D instead of prednisone) was US dollars 7,616 per day of severe thrombocytopenia avoided, primarily due to the much higher medication cost of anti-D. Utilizing an outpatient model, the cost difference between anti-D and prednisone was even more striking. CONCLUSIONS: The clinical benefit of anti-D is offset by a substantial cost increase. Although often overlooked in favor of newer agents, a brief course of high-dose prednisone is an inexpensive and effective treatment for acute ITP.     

儿童免疫相关性疾病的常规免疫学指标研究

目的比较特发性血小板减少性紫癜(ITP)、过敏性紫癜(HSP)及皮肤黏膜淋巴结综合征(KD)的常规免疫学指标变化及静脉注射免疫球蛋白(IVIG)的应用。方法用流式细胞仪测T细胞亚群,免疫比浊法测免疫球蛋白(Ig)及补体。常规加IVIG治疗。结果ITP患儿CD3 CD4 T细胞降低并IgG升高;HSP患儿IgA升高伴CD3 T细胞增高。KD患儿CD3 CD8 T细胞降低。IVIG可明显改善3种疾病临床症状,尤其有助KD患儿冠状动脉损害发生率减少。结论T细胞亚群、lg 应作为ITP、HSP及KD的常规检查项目。早期应用IVIG对改善预后及缩短病程有重要意义。     

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.     

A randomized and comparative study of intravenous immunoglobulin and mega dose methylprednisolone treatments in children with acute idiopathic thrombocytopenic purpura

The most common cause of mortality in childhood acute idiopathic thrombocytopenic purpura (ITP) is intracranial hemorrhage (ICH), which occurs in about 0.1% of children with platelet counts below 20,000/microl. Forty-two children (1-13 years) with ITP and platelet counts < or = 20,000/microl were randomly divided into two groups. Twenty patients received mega-dose methylprednisolone (MDMP) in a dosage of 30 mg/kg/d for three days and 20 mg/kg/d for four days. Twenty-two patients received intravenous immunoglobulin (IVIG) in a dosage of 1 g/kg/d two days. Platelet counts of the patients were determined at diagnosis, at 2, 4, 7, 14, 30, 60, 90, 120, 150, and 180 days and at three-month intervals after the 6th month. The mean platelet counts of both groups gradually increased and peaked on the 7th day (p > 0.05). There were no significant differences between the mean platelet counts of patients, in the two groups on treatment days 0, 2, 4, 7, and 14. The mean time for achievement of platelet counts above 20,000/microg in the MDMP group and the IVIG group was 4.1 and 2.9 days (p < 0.05) and above 50,000/microl was 5.0 and 5.2 days (p > 0.05), respectively. The percentages of patients with platelet counts above 20,000/microl at the 2nd day of the treatment were 50% in the MDMP group, and 86% in the IVIG group (p < 0.05). No significant differences were observed in the mean platelet counts of the two groups treatment days 30, 60, 90, 120 and 180 (p > 0.05). Chronic ITP developed in five patients (25%) in the MDMP group, and in four patients (18%) in the IVIG group (p > 0.05). Intravenous immunoglobulin (IVIG) (1 g/kg/d for 2 days) and MDMP treatments (30 mg/kg/d for 3 days, 20 mg/kg/d for 4 days, perorally) are equally effective in the treatment of acute ITP. Because of its nonbiologic source, lower cost, fewer side effects and oral use, we prefer oral preparations of MDMP in the treatment of childhood ITP.