血小板特异性抗体的测定与特发性血小板减少性紫癜的关系

特发性血小板减少性紫癜 (ＩＴＰ)发生的主要原因是抗血小板自身抗体引起的血小板破坏 ,因而血小板自身抗体的检测对ＩＴＰ的诊断及发病机理的研究有重要价值。本研究用改良的血小板抗原单抗特异性固相化法 (ｍｏｎｏｃｌｏｎａｌａｎｔｉｂｏｄｙｓｐｅｃｉｆｉｃｉｍｍｏｂｏｌｉｚａｔｉｏｎｏｆｐｌａｔｅｌｅｔａｎｔｉｇｅｎｓ ,ＭＡＩＰＡ)测定了ＩＴＰ患儿和非免疫性血小板减少症患儿血清中的抗ＧＰＩＩｂ/ＩＩＩａ和抗ＧＰＩｂ/Ｉｘ自身抗体 ,并同时测定这些患儿的血小板表面相关抗体 (ＰＡＩｇＧ) ,将二者作比较 ,试图评价血小板…     

特发性血小板减少性紫癜患儿巨细胞病毒感染的初步研究

为探讨特发性血小板减少性紫癜（ＩＴＰ）与人巨细胞病毒（ＨＣＭＶ）活动性感染的关系，用聚合酶链反应（ＰＣＲ）技术检测了４４例临床确诊为特发性血小板减少性紫癜患儿外周血白细胞中ＨＣＭＶ－ＤＮＡ，同时作病毒分离及ＨＣＭＶ－ＩｇＧ、ＩｇＭ测定。结果表明：ＨＣＭＶ－ＤＮＡ阳性者１４例，阳性率为３２％；而病毒分离阳性者８例，阳性率为１８％；ＨＣＭＶ－ＩｇＧ、ＩｇＭ阳性率分别为３９％和２３％。提示：（１）部分ＩＴＰ患儿发病时有ＨＣＭＶ活动性感染；（２）ＰＣＲ技术特异、敏感、快速，对ＩＴＰ患儿的ＨＣＭＶ活动性感染的早期快速诊断有重要作用。     

小剂量静注丙种球蛋白治疗儿童免疫性血小板减少性紫癜的疗效观察

为寻求应用静脉丙种球蛋白治疗免疫性血小板减少性紫癜的最佳方法,将我院自１９９６年１１月以来收住的ＩＴＰ患儿随机分为两组：Ａ组应用小剂量ＩＶＩＧ,即０．４ｇ／连用２天;Ｂ组应用大剂量ＩＶＩＧ,即０．４ｇ／连用５天。结果显示：小剂量ＩＶＩＧ与大剂量ＩＶＩＧ治疗ＩＴＰ的疗效相当。小剂量ＩＶＩＧ可以替代大剂量ＩＶＩＧ作为ＩＴＰ患儿的急救方法。     

抗-D抗体治疗特发性血小板减少性紫癜的进展

特发性血小板减少性紫癜 (ＩＴＰ)是一种自身免疫性疾病 ,常规治疗包括肾上腺皮质激素、静脉用免疫球蛋白(ＩＶＩＧ)、脾脏切除及其它免疫抑制剂。抗 Ｄ抗体的临床应用增加了ＩＴＰ的治疗方法。1　抗 Ｄ抗体作用机制1983年Ｓａｌａｍａ等[1] 认为 :静脉或肌肉注射抗 Ｄ抗体后 ,Ｒｈ Ｄ阳性的红细胞表面即覆盖抗 Ｄ抗体 ,被抗 Ｄ抗体致敏的红细胞与网状内皮系统(ＲＥＳ)上Ｆｃ受体 (ＦｃＲ)优先结合 ,出现以下两种结果 :①间接抑制ＲＥＳ生物活性 ;②阻断了ＦｃＲ与血小板 血小板相关抗体复合物结合 ,从而使血小板 -血小板相关抗体复合…     

大剂量氢化考的松治疗急重型特发性血小板减少性紫癜

大剂量氢化考的松治疗急重型特发性血小板减少性紫癜空军４６３医院（１１００４２）王虹特发性血小板减少性紫癜（ＩＴＰ）是一种原因未明的自身免疫性疾病，伴有血小板相关抗体，主要为ＩｇＧ或ＩｇＭ升高，能特异地与巨核细胞相结合，抑制区核细胞产生血小板，使循环血...     

丙种球蛋白治疗新生儿血小板减少性紫癜20例

我们 1997～ 1999年对新生儿血小板减少性紫癜 (ＩＴＰ)采用静脉注射丙球蛋白 (ＩＶＩＧ)治疗 ,并与 1970～ 1996年新生儿ＩＴＰ对比 ,报告如下。资料与方法一、临床资料　随意抽取 1970～ 1996年新生儿ＩＴＰ 2 0例为对照组 ,1997～ 1999年新生儿ＩＴＰ 2 0例为治疗组 ;按1998年 6月特发性血小板减少性紫癜诊疗建议 (修订草案 )诊断[1] 。排除同族免疫性血小板减少及新生儿暂时性血小板减少。治疗组男 12例 ,女 8例 ;日龄未满 2 8ｄ。对照组男 14例 ,女 16例 ;日龄未满 2 8ｄ。二、治疗方法　治疗组用ＩＶＩＧ 40 0ｍｇ/ (ｋｇ·ｄ) (长春…     

小儿特发性血小板减少性紫癜血小板相关抗体,T细胞亚群检测及其？…

本文对１９８９年９月至１９９５年２月收治的１１２例小儿特发性血小板减少性紫癜的血小板相关性抗体、Ｔ细胞亚群检测及其临床意义进行了探讨。结果表明：急、慢型无论是ＯＫＴ３＾＋及ＯＫＴ４＾＋均低于正常对照组，且有显著性差异，Ｐ值均〈０．０５；而ＯＫＴ８＾＋与正常对照组相比则较高，Ｐ值〈０．０２５。但是，急、慢两型相比均未见统计学上显差性差异。本文的检测血小板抗体ＩｇＧ、ＩｇＭ及ＩｇＡ，显示均有所增高尤以     

静注免疫球蛋白及地塞米松治疗特发性血小板减少性紫癜临床观察

探讨大剂量地塞米松（ＤＥＸ）及静注免疫球蛋白（ＩＶＩＧ）治疗特发性血小板减少性紫癜（ＩＴＰ）的疗效及其对血液粘稠度的影响。以ＤＥＸ、ＩＶＩＧ、ＩＶＩＧ加ＤＥＸ治疗ＩＴＰ患儿,观察其血小板数变化,并于治疗前后各抽取静脉血一次。结果显示：１．平均血小板计数升至正常时间：单纯ＤＥＸ治疗组为７天,单纯ＩＶＩＧ组及ＩＶＩＧ加ＤＥＸ组为４天;平均计数峰值：单纯ＩＶＩＧ组及ＩＶＩＧ加ＤＥＸ组高于ＤＥＸ组,但单纯ＩＶＩＧ组与ＩＶＩＧ加ＤＥＸ组之间无显著差异。２．ＩＴＰ患儿治疗后骨髓成熟产板巨核细胞数比例由１．５６±２．５３％升至１３．３６±６．６８％,原幼巨核细胞数比例由４０．２０±１．８７％降至１３．３９±９．２８％,差异非常显著,治疗后各组间骨髓巨核细胞数比例无显著差异。３．ＩＴＰ患儿全血粘度值、血浆粘度值治疗前后三组均无显著变化。结论：ＩＴＰ患儿ＩＶＩＧ治疗较ＤＥＸ治疗血小板上升迅速,上升幅度大。１ＶＩＧ治疗的同时加大剂量ＤＥＸ治疗,未能进一步使血小板升高。     

静注免疫球蛋白及地塞米松治疗特发性血小板减少性紫癜临床观察

探讨大剂量地塞米松及静注免疫球蛋白治疗特发性血小板减少性紫癜（ＩＴＰ）的疗效及其对血液粘稠度的影响。以ＤＥＸ、ＩＶＩＧ、ＩＶＩＧ加ＤＥＸ治疗ＩＴＰ患儿,观察其血小板数变化,并于治疗前后各抽取静脉血一次。结果显示：１。平均血小板计数升至正常时间：单纯ＤＥＸ治疗组为７天,单纯ＩＶＩＧ组及ＩＶＩＧ加ＤＥＸ组为４天;平均计数峰值;单纯ＩＶＩＧ组及ＩＶＩＧ加ＤＥＸ组高于ＤＥＸ缄,但单纯ＩＶＩＧ组与ＩＶＩＧ加     

EBV血清阳性对ITP患儿IVIG疗效的影响

免疫性血小板减少性紫癜（ＩＴＰ）常由于急性病毒感染而引起。患有急、慢性ＩＴＰ的患儿大约８０％在应用静脉免疫球蛋白（ＩＶＩＧ）治疗的最初一周效果很好。ＩＶＩＧ通过中和脾和肝脏内的巨噬细胞的Ｆｃ受体而起作用。急性ＥＢ病毒（ＥＢＶ）感染可能与几种免疫介质的异常有关,包括血小板减少。该文旨在评诂 ＥＢＶ感染对ＩＶＩＧ治疗ＩＴＰ疗效的影响。 病人和方法以１９８９年２月～１９９５年１２月期间在某大学医院儿科协血液肿瘤病房诊断为ＩＴＰ的患者为研究对象。初期仅单独应用ＩＶＩＧ。调查表格项目包括病史、ＥＢＶ血清学、…     

Platelet-Associated IgG in Children - Values and Evaluation of PAIgG in Various Thrombocytopenias -

Platelet-associated IgG (PAIgG) levels were measured in 60 children with ITP (46-chronic, 14-acute) using Fab-anti Fab radioimmunoassay method described by McMillan et al. In some patients platelet binding IgG in serum (PBIgG) was also determined at the same time. Patients with ITP had significantly greater PAIgG levels than 30 normal subjects and 13 non-immune thrombocytopenic controls. Elevated PAIgG values did not correlate with parameters of platelet size (mean platelet volume; MPV and percentage of large platelet; PLP) and so these data indicated that high levels of PAIgG in ITP were not due to nonspecific adhesion of serum IgG to megathrombocytes usually increased in this disorder, but due to specific immunological reaction. PBIgG IgG values were also elevated in patients with pretreated chronic ITP, but high levels remained even after successful splenectomy. Furthermore, serial determination of PAIgG values were obtained in some patients with chronic ITP who underwent splenectomy and with acute ITP who achieved spontaneous remission. PAIgG returned to normal levels when thrombocytopenia disappeared. PAIgG seems to be the most reproducible indicator which reflects transition of the clinical picture in this disorder.     

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.     

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.     

特发性血小板减少性紫癜患者血小板抗体及淋巴细胞亚群的研究

目的探讨血小板相关抗体(PAIgG)和T淋巴细胞亚群的变化,在特发性血小板减少性紫癜(ITP)免疫发病机制中的作用、临床意义。方法采用间接免疫荧光法测定30例ITP患者及20例正常对照组的PAIgG,20例ITP患儿治疗后复查PAIgG。同时采用流式细胞仪直接免疫荧光法检测外周T血淋巴细胞亚群。结果ITP组PAIgG阳性率为80%,正常对照组为20%(P<0.001),ITP组PAIgG明显高于正常对照组(P<0.001),20例ITP患儿治疗后复查PAIgG,其数值明显下降,差异有显著性(P<0.001)。T淋巴细胞亚群中,ITP组CD3、CD4、CD4/CD8显著低于正常对照组(P<0.01),CD8则显著高于正常对照组(P<0.01)。结论抗血小板相关抗体对提高ITP的诊断、疗效及预后的判断有一定的实用价值,T淋巴细胞亚群的变化能较好的反映ITP的病理机制。     

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.     

Evaluation of a simple immunodiffusion technique for quantitation of platelet-associated immunoglobulin G in childhood immune thrombocytopenias

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.     

Agenesis of the Corpus Callosum and Neural Crest Tumors

Hematologic abnormalities, including thrombocytopenia, are seen in HIV infection. Mi have previously reported elevated platelet-associated IgG (PAIgG) in thrombocytopenia in children associated with human immunodeficiency virus (HIV). In this study we prospectively monitored 40 HIV-infected infants and children to determine the significance of elevated PAIgG levels as they relate to thrombocytopenia. We also examined platelet eluatesfor the presence of HIV antibody and antigen. Of 16 patients with thrombocytopenia, 15 (93.7%) had elevated PAIgG. Of 24 patients with normal platelet counts, 21 (87.5%) had elevated PAIgG. On follow-up, none of the children with normal platelet counts and elevated PAIgG levels developed thrombocytopenia. Examination of the platelet eluates was negative for HIV antibody or P24 antigen. Although the sensitivity of an elevated PAIgG level in predicting thrombocytopenia is 93%, its specificity is only 13%. Elevated PAIgG levels are therefore not causally related to the development of thrombocytopenia in children.     

Platelet-Associated IgG in Pediatric HIV Infection

Hematologic abnormalities, including thrombocytopenia, are seen in HIV infection. Mi have previously reported elevated platelet-associated IgG (PAIgG) in thrombocytopenia in children associated with human immunodeficiency virus (HIV). In this study we prospectively monitored 40 HIV-infected infants and children to determine the significance of elevated PAIgG levels as they relate to thrombocytopenia. We also examined platelet eluatesfor the presence of HIV antibody and antigen. Of 16 patients with thrombocytopenia, 15 (93.7%) had elevated PAIgG. Of 24 patients with normal platelet counts, 21 (87.5%) had elevated PAIgG. On follow-up, none of the children with normal platelet counts and elevated PAIgG levels developed thrombocytopenia. Examination of the platelet eluates was negative for HIV antibody or P24 antigen. Although the sensitivity of an elevated PAIgG level in predicting thrombocytopenia is 93%, its specificity is only 13%. Elevated PAIgG levels are therefore not causally related to the development of thrombocytopenia in children.     

Platelet-associated immunoglobulin G in childhood idiopathic thrombocytopenic purpura

Childhood ITP is an acquired hemorrhagic disorder with a heterogeneous clinical course. We measured PAIgG levels in 20 children with ITP (7 acute, 13 chronic). Both groups had significantly greater PAIgG values than age-matched normal subjects and thrombocytopenic controls (P less than 0.001). In addition, PAIgG values in chronic ITP were significantly lower than those in acute ITP (P less than 0.003). Serial PAIgG values were obtained in some patients; most returned to normal in association with clinical recovery. The measurement of PAIgG is useful in the diagnosis and follow-up of childhood ITP. PAIgG values may assist in differentiating acute and chronic disease in children.     

小儿特发性血小板减少性紫癜血小板相关抗体、T细胞亚群检测及其临床意义

本文对1989年9月至1995年2月收治的112例小儿特发性血小板减少性紫癜的血小板相关性抗体、T细胞亚群检测及其临床意义进行了探讨。结果表明：急、慢型无论是OKT3及OKT4+均低于正常对照组，且有显著性差异，P值均＜0．05；而OKT8+与正常对照组相比则较高，P值＜0．025。但是，急、慢两型相比均未见统计学上显差性差异。本文的检测血小板抗体lgG、IgM及IgA，显示均有所增高尤以PAIgG最明显，阳性率达85．7％，与文献报道近似。经治疗10例血小板动态变化，PAIg随着血小板的恢复均下降至正常，呈负相关.