血浆可溶性P-选择素与儿童偏头痛关系的研究

目的 研究血浆可溶性P-选择素水平与儿童偏头痛的关系.方法 采用ABC-ELISA法检测偏头痛组、偏头痛病情控制组及健康对照组儿童可溶性P-选择素的水平、经颅多普勒(TCD)和外周血血小板计数,并进行P-选择素与血小板计数的相关性分析.结果 偏头痛组患儿血浆可溶性P-选择素水平明显高于病情控制组及健康对照组,差异有统计学意义(F=111.067,P<0.05);病情控制组与正常对照组比较,差异元统计学意义(P>0.05);TCD检测显示偏头痛组患儿脑血管痉挛发生率明显高于病情控制组患儿,差异有统计学意义(X2=23.08,P<0.05);血常规检查显示偏头痛组血小板计数高于病情控制组及健康对照组,差异有统计学意义(X2=22.22,P<0.05);病情控制组患儿血浆可溶性P-选择素水平降至正常,脑血管痉挛及血小板数升高的发生率均降至正常水平.血浆可溶性P-选择素水平与血小板计数呈正相关(r=0.996,P<0.01).结论 儿童偏头痛血浆可溶性P-选择素增高,脑血管痉挛发生率明显增高.血浆可溶性P-选择素与血小板计数呈正相关.血浆可溶性P-选择素可作为儿童偏头痛诊断、治疗及疗效评价的指标之一.     

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.     

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.     

婴幼儿特发性血小板减少性紫癜401例临床特点

目的 分析婴幼儿特发性血小板减少性紫癜(ITP)的临床特点,并比较婴儿与幼儿ITP的疗效.方法 收集401例婴幼儿ITP,均给予激素冲击治疗和静脉滴注免疫球蛋白治疗,疗效判定依据血小板计数的高低和出血症状的改善分为完全缓解、有效、无效.401例婴幼儿ITP按年龄分为婴儿组(≤1岁)和幼儿组(>1～3岁),按病程分为急性(病程≤6个月)和慢性(病程>6个月),对其临床资料进行回顾性分析,应用SPSS 12.0软件进行统计分析.结果 1.婴儿组与幼儿组均为男童比例高,但2组间性别比较差异无统计学意义(χ2=0.682,P>0.05).2.婴儿组入院时血小板中位计数低于幼儿组,差异有统计学意义(Z=2.668,P<0.05).3.婴儿组骨髓巨核细胞增高比例低于幼儿组,差异有统计学意义(χ2=16.322,P<0.001);婴儿组产板巨核细胞中位数低于幼儿组,差异有统计学意义(Z=2.065,P<0.05).4.婴儿组经治疗后血小板计数达到或超过100×109 L-1的时间短于幼儿组,差异有统计学意义(Z=3.542,P<0.001).5.急性患儿入院时血小板中位计数低于慢性患儿,差异有统计学意义(Z=2.100,P<0.05).6.输注血小板患儿的住院时间与未输注血小板患儿相比,差异无统计学意义(Z=1.385,P>0.05).结论 婴幼儿ITP中,男童比例高,大部分无明显诱因,以皮肤黏膜出血为主;婴儿入院时血小板中位计数较低,血小板上升至正常的时间较短,对治疗反应较幼儿好,幼儿急性ITP可以变为慢性;输注血小板并不能缩短ITP患儿的住院时间.     

Retrospective evaluation of children with immune thrombocytopenic purpura and factors contributing to chronicity

ObjectiveImmune thrombocytopenic purpura (ITP) is the most common cause of acquired thrombocytopenia children. The aim of this retrospective study is to describe presenting features and clinical characteristics of ITP and evaluate clinical course, treatment modalities, and complications and determine the effects of preceding infection history, age, gender, treatment modality, and admission platelet count on chronicity.MethodTwo hundred and eleven patients who were diagnosed ITP and followed-up in Department of Pediatric Hematology, Ankara Children Hematology Oncology Education and Research Hospital between January 2008 and September 2012 were included. Age of the patients, gender, date of admission, date of diagnosis, complaint in the application, previous infection and laboratory tests were recorded.ResultsMean age of the patients on diagnosis was 5.4 ± 4.1 years. The female/male ratio was 1.03. The clinical courses were determined as acute or chronic in 72% and 28% of patients respectively. Mean age at diagnosis was significantly higher in chronic ITP (p < 0.01). Chronic course was significantly higher in female patients (p < 0.05). The most frequent complaint was bruises on the skin (68%). The most common physical examination findings were petechiae, purpura and ecchymosis (89%). Patients with a history of past infection (53.6%) and who had serologically positive infection (15.6%) frequently had acute course (p < 0.01). The most common serologically positive infection was Rubella. The mean platelet count was significantly higher in chronic ITP (p < 0.01). In the initial treatment of patients admitted in the acute phase, megadose methylprednisolone (MDMP) was used in 31% of patients, intravenous immune globulin (IVIG) in 55% of patients and anti-D in 2% of patients while 12% did not receive any treatment. There were no significant differences between the recurrence rate and treatment modality (p > 0.05).ConclusionIn our study, in females and in patients without any history of past infection, platelet count >20 × 10⁹/L and initial diagnosis age > 10 years were found to increase the probability of chronic disease, which is compatible with the literature.     

Evaluation of clinical characteristics, diagnosis and management in childhood immune thrombocytopenic purpura: a single center's experience

Diagnostic evaluation and management in childhood immune thrombocytopenic purpura (ITP) are controversial. We reviewed the files of 162 children with ITP to evaluate clinical characteristics, response to treatment and outcome. History of antecedent infection, vaccination and serologic evidence for acute viral infection were present in 48%, 5% and 17% of the patients, respectively. At diagnosis, two-thirds of the patients had a platelet count of <10,000/microl but only 10% had major bleedings. Intracranial hemorrhage was seen in two patients (1.2%) with a mortality rate of 0.6%. Sixteen percent developed chronic ITP. The rate of platelet recovery with mega-dose methylprednisolone (30 mg/kg/d for 3 and 20 mg/kg/d for 4 days) was similar to that obtained with intravenous immunoglobulin or oral prednisolone. Four of seven patients with ITP responded to splenectomy. These data show that mode of treatment has no effect on the clinical course and prognosis of childhood ITP.     

特发性血小板减少性紫癜患儿血小板生成素及其受体基因转录水 …

目的 探讨急性特发性血小板减少性减少性紫癜（ＡＴＴＰ）患儿血小板生成素（ＴＰＯ）及血小板ＴＰＯ受体ｃ－ＭＰＬ ｍＲＮＡ基因转化水平变化的意义。方法 采用ＥＬＩＳＡ法检测血清ＴＰＯ、血小板相关抗体（ＰＡＩｇＧ）水平;半定量ＲＴ－ＰＣＲ法检测外周血血小板ｃ－ＭＰＬ ｍＲＮＡ的相对量。结果 ＡＴＴＰ患儿初治时血浆ＴＰＯ水平增高,血小板ｃ－ＭＯＬ ｍＲＮＡ较低;以大剂量甲基强的松龙冲击治疗后,位随血小板计     

Eltrombopag (thrombopoietin‐receptor agonist) and plasmapheresis as rescue therapy of acute post‐renal transplant immune thrombocytopenia in a child with Schimke immuno‐osseous dysplasia—case report

SIOD is rare disorder related to SMARCAL1 or SMARCAL2 gene mutation, including (among other comorbidities) T‐cell immunodeficiency, nephrotic syndrome, and renal failure. Up to 22% of primary patients may develop various autoimmune disorders. We report the case of 11‐year‐old male with SIOD, who presented ITP at 2 years after renal transplantation with decrease in platelet count (from normal) to 56 000/μL and then (gradually) to 2000/μL. There was no effect of iv. methylprednisolone/dexamethasone. As the presence of antibodies against GPIIb/IIIa, GPIb, and GPIaIIa platelet glycoproteins was confirmed, patient was given 50 g of IVIG and then was put on plasmapheresis; however, both showed poor direct effect. As we were afraid to give rituximab (due to expected overimmunosuppression), we prescribed the oral TPO‐receptor agonist (eltrombopag). Patient responded after 17 days of therapy, to the final dose of 50 mg/d (approx. 2 mg/kg). The antiplatelet antibodies disappeared after four plasmapheresis. Overall, the therapy was continued for 7 weeks and was stopped at platelet count of 433 000/μL. Platelet count remained stable in 8‐month follow‐up. Combination of plasmapheresis and TPO‐receptor agonist was effective in post‐renal transplant acute ITP in patient with SIOD.     

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.     

重症肺炎合并脓毒症患儿炎症因子及凝血指标与危重症评分相关性分析

目的 探讨重症肺炎合并脓毒症患儿炎症因子和凝血指标与危重症评分的相关性。方法 选择2010年1月至2012年11月在福建省妇幼保健院PICU入住24 h以上,符合重症肺炎合并脓毒症诊断的患儿为研究对象。根据小儿危重病例评分法分为极危重组（<70分）、危重组（~80分）和非危重组（>80分）。检测炎症因子（血WBC、PLT和CRP、IL-6）和凝血指标（D-二聚体和可溶性P-选择素）水平,采用多元线性逐步回归分析炎症因子、凝血指标与危重症评分的相关性。结果 101例患儿进入分析,男47例,女54例。非危重组53例,危重组42例,极危重组6例。①随着危重症评分分值降低,IL-6、D-二聚体和可溶性P-选择素水平逐渐增高,组间两两比较差异均有统计学意义(P均<0.05);CRP水平亦随危重症评分降低而逐渐增高,在非危重组和危重组间差异有统计学意义（P<0.05）;PLT计数则随危重症评分降低呈降低趋势,组间两两比较差异均有统计学意义(P均<0.05);血WBC在各组间差异均无统计学意义(P均﹥0.05)。②IL-6、可溶性P-选择素和D-二聚体水平与危重症评分呈正相关,PLT计数与危重症评分呈负相关,血WBC和CRP与危重症评分无相关性。结论 IL-6、可溶性P-选择素、D-二聚体和PLT水平与儿童重症肺炎合并脓毒症的严重程度相关。     

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.     

Platelet-associated immunoglobulin G in childhood idiopathic thrombocytopenic purpura

Platelet-associated IgG was studied in children with acute and chronic ITP and in patients with thrombocytopenic SLE, using the microtiter solid-phase radioimmunoassay. Of the children with acute ITP, 85% had elevated PAIgG levels. The degree of elevation of PAIgG at onset of disease did not correlate with the development of chronicity. Of the children with acute ITP, clinically and hematologically indistinguishable from the rest, 15% had normal PAIgG values. All of 22 children with chronic ITP had elevated PAIgG values. Although there was good correlation between the platelet count and the PAIgG value in children with chronic ITP, the association was not as striking in those with acute ITP; thus, factors in addition to the level of PAIgG may contribute to the thrombocytopenia in the latter group. Patients with SLE and thrombocytopenia had higher values of PAIgG than would be predicted from the platelet count; the PAIgG value is probably not the only factor determining the degree of immune thrombocytopenia.     

Prevalence and Clinical Significance of Antithyroid Antibodies in Children with Immune Thrombocytopenic Purpura

Background and objective: To determine the prevalence and the clinical significance of thyroid autoantibodies and their influence on treatment response in children with idiopathic thrombocytopenic purpura (ITP). Patient and Method: We retrospectively analyzed the antithyroglobulin (anti-TG) and antithyroid peroxidase (anti-TPO) antibodies from the records of 151 ITP patients who were admitted to the Pediatric Hematology Department of Gaziantep University between 2009 and 2012. Results: Anti-TPO and/or anti-TG was found positive in 38 (36.8%) of 103 patients whose thyroid autoantibody levels were measured. The comparison of positivity ratios of autoantibodies between acute and chronic ITP patients showed no significant difference. However, the separate comparison of each group of ITP patients with control group showed significantly high positivity ratios of autoantibodies in ITP patients. The initial mean platelet count of anti-TPO positive patients at diagnosis was significantly less than that of the negative patients (P = .008). One month after treatment, platelet count of anti-TPO positive patients was significantly less than that of the negative patients (P = .01). Moreover, the mean platelet counts of anti-TPO positive patients were significantly less than those of the negative patients after intravenous immunoglobulin treatment (P < .001). Conclusion: We demonstrated that the thyroid-autoimmune-diseases-related autoantibodies are frequently found in childhood ITP. Although no recommendation is found in international guidelines regarding screening for thyroid autoantibodies in patients with ITP, in view of the high incidence of antithyroid antibodies and their potential negative effect on treatment response, screening these patients for such antibodies would be recommended.     

Randomized trial of high-dose methylprednisolone versus intravenous immunoglobulin for the treatment of acute idiopathic thrombocytopenic purpura in children

BACKGROUND: Idiopathic thrombocytopenic purpura (ITP) is an acquired disorder characterized by immune-mediated platelet destruction. The authors performed a prospective, randomized trial comparing intravenous immunoglobulin (IVIG) with high-dose intravenous methylprednisolone in the treatment of children with acute ITP. The primary aim of the study was to compare the rate of platelet increase produced by either intervention. A decision to treat was based on the clinical presentation and not an arbitrary platelet count. In general, enrolled patients exhibited extensive bruising and platelet counts less than 10 x 10 /L (10,000/microL). PATIENTS AND METHODS: Seventy-seven consecutive patients, for whom the attending hematologist determined acute treatment was warranted, were studied. Forty-two patients received IVIG (1 g/kg/dose x2) and 35 received methylprednisolone (30 mg/kg/dose x3). Patients who exhibited an increase in platelet count of more than 50,000/microL after the first IVIG dose or the second methylprednisolone dose did not receive the second IVIG dose or the third methylprednisolone dose, respectively. Patients' ages ranged from 6 months to 15 years. Platelet counts were evaluated at presentation, 24, 48, 72 hours, 1 week, and 2 to 4 weeks. RESULTS: Eighty percent of patients treated with IVIG and 60% of patients treated with methylprednisolone demonstrated an increase in platelet count of 50,000/microL or more within 48 hours. Both IVIG and methylprednisolone therapy increased platelet counts significantly above pretreatment values. In the methylprednisolone group, the mean baseline platelet count was 4,600/microL, which rose to 14,000/microL after 24 hours, 38,000/microL after 48 hours, and 65,000/microL after 72 hours. The IVIG group had a mean baseline platelet count of 4,200/microL, which rose to 32,000/microL after 24 hours, 69,000/microL after 48 hours, and 146,000/microL after 72 hours. When compared with methylprednisolone, IVIG therapy produced a greater rise in platelet counts at 24, 48, and 72 hours, with no difference at 1 week or later time points. No serious bleeding was noted in either treatment group. CONCLUSIONS: Both IVIG and methylprednisolone produce a significant early rise in platelet count that is somewhat greater with IVIG. However, the higher platelet counts produced by IVIG may not justify the additional cost and potential risks of this agent.     

特发性血小板减少性紫癜患儿血清微小病毒B_(19)检测及意义

目的探讨人微小病毒B19(HPVB19)感染与儿童特发性血小板减少性紫癜(ITP)发病的关系。方法采用酶联免疫法(ELISA)对46例ITP患儿利30例健康儿童的血清标本进行HPVB19-IgM、IgG及血小板相关抗体检测。结果46例ITP患儿血清中HPVB19抗体总刚性率43.48%(20/46),30例健康儿童HPVB19-IgM、IgG均为阴性,2组间差异有统计学意义(P<0.01);ITP组中急性型与慢性型之间HPVB19抗体总阳性率差异有统计学意义(P<0.05);病毒感染刚性患儿的血小板相关抗体明显高于病毒感染阴性患儿,差异有统计学意义(P<0.01)。结论ITP患儿血清中HPVB19抗体总刚性率高,尤其是急性型;HPVB19感染后可导致血小板相关抗体升高而致血小板减少。     

Cytokines in idiopathic thrombocytopenic purpura (ITP)

Most research in idiopathic thrombocytopenic purpura (ITP) has focused on characterization of the autoantibodies directed against platelet antigens resulting in enhanced platelet elimination by macrophages. This report summarizes the current knowledge of cytokine pattern found in individuals with ITP. Serum assessment has demonstrated increased levels of interleukin (IL)-2 and interferon-gamma (IFN-7), while IL-4 was significantly decreased. In addition, thrombopoietin (TPO) has been found in normal levels while IL-11 has been reported to be elevated. These data indicate that ITP is associated with a Th1 type of T helper cytokine response, while that of type Th2 is downregulated. Initially, megakaryocytes are found at normal levels in bone-marrow aspirates, explaining the unchanged production of TPO. The increase in IL-11 may be reflected by the increased number of platelets being produced per megakaryocyte. However, there is little information on these events in immunocompetent sites such as bone marrow, spleen and lymph nodes.     

Relationships among bleeding severity,health‐related quality of life,and platelet count in children with immune thrombocytopenic purpura

Important outcomes for children with immune thrombocytopenic purpura (ITP) include health‐related quality of life (HRQOL) and bleeding severity. A HRQOL instrument for children with ITP, the Kids' ITP Tools (KIT), was recently validated. Secondary analysis of the KIT database was performed to determine relationships among platelet count, bleeding severity and HRQOL. Bleeding severity grade correlated with platelet count in chronic ITP but not in acute ITP. Platelet count and bleeding severity failed to have any statistically significant correlations with the KIT scores. These findings suggest that relationships among outcome measures in children with ITP, using currently available instruments, remain poorly defined. Pediatr Blood Cancer 2009;53:652–654. © 2009 Wiley‐Liss, Inc.     

Combination therapy for refractory idiopathic thrombocytopenic purpura in adolescents

PURPOSE: To investigate combined immunosuppressive therapy with vincristine, methylprednisolone, and prolonged cyclosporine in adolescents with refractory idiopathic thrombocytopenic purpura (ITP). PATIENTS AND METHODS: Ten adolescent patients with ITP refractory to previous medical management, including gluco-corticosteroid, intravenous immunoglobulin or anti-Rh (D) IgG, or splenectomy, were treated with combination immunosuppressive therapy at the University of Michigan between 1997 and 2001. Therapy consisted of weekly doses of vincristine 1.5 mg/m intravenous push (IVP) (maximum dose 2 mg), weekly methylprednisolone 100 mg/m IVP, and cyclosporine (CSA) 5 mg/kg orally twice daily (goal: CSA trough of 100-200 mg/mL). Vincristine and methylprednisolone were given weekly until the platelet count was greater than 50,000/mm for a minimum of 2 doses and a maximum of 4 doses. CSA was continued until the platelet count was normal for 3 to 6 months. RESULTS: Seven patients had continuous complete responses (platelet count normal after cessation of CSA), a median of 13 months (9-37 months) since completion of therapy. One patient had a partial response (platelet count 80-120 x 10 /L off CSA for 3 months). Two patients were nonresponders (platelet count <40 x 10 /L), one of whom had all therapy discontinued after 2 weeks due to peripheral neuropathy. The median time to response was 7 days (range 7-67 days). CSA was administered for a median of 4 months (range 0.5-19 months). CONCLUSIONS: A combination immunosuppressive approach that includes prolonged cyclosporine therapy may be promising for refractory ITP and is associated with sustained disease remissions in some 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.     

Treatment of childhood idiopathic thrombocytopenic purpura with Rhesus antibodies (anti-D)

We have recently reported that a rise of platelet numbers in ITP can be induced by blockade of the RES with antibody-coated red blood cells. We now present a collaborative study in which 15 Rhesus-positive children with ITP (nine boys and six girls aged 1–15 years) were treated with low-dose anti-D. Ten patients had chronic ITP (duration 6–47 months), five had acute ITP. Doses of 28–50 g anti-D/kg bodyweight per course were given intravenously. In all patients clinical signs of bleeding ceased and platelet counts were elevated. An excellent, good or fair response with platelet increments of >100, 50–100, or 20–50×10⁹/l, respectively, was observed in 19, 7, and 12 out of 45 courses in chronic ITP, and in 4, 1, and 2 out of 8 courses in acute ITP. The platelet increase (>40×10⁹/l) persisted for 10 to over 360 days in chronic ITP. There were no untoward side reactions. Haemoglobin values remained stable in all patients but laboratory signs of mild, compensated haemolysis ensued. The direct antiglobulin test became positive in all cases due to anti-D IgG. Previous therapy of patients with chronic ITP included high-dose immunoglobulins and prednisone. These regimens were both effective but remissions were short. We conclude that anti-D therapy is an effective and safe form of treatment in childhood ITP.Abbreviations ITP idiopathic thrombocytopenic purpura - IgG immunoglobulin G - RES reticuloendothelial system - RBC red blood cells - Rh Rhesus - DAT direct antiglobulin test