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Idiopathic thrombocytopenic purpura (ITP),as a heterogeneous autoimmune disease,is the most common bleeding disorder,of which etiology and pathogenesis are still unclear. Health related quality of life are severely affected,especally in chronic patients . Studys on chronic idiopathic thrombocytopenic purpura pathogenesis ,both in China and abroad,have found that genetic factors may be involved in the occurrence and prognosis of the disease. The paper gives a review from the follow aspects,such as its risk factors,relationship between genetic polymorphisms or expression levels and chronic ITP susceptibility and seriousness,the role of epigenetic,researchs about chronic and refractory ITP,et al.
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Objective To observe the effect of expelling wind, cooling blood and invigorating kidney prescription on the marrow megakaryocyte differentiation maturity and TGF-β1 in patients with chronic idiopathic thrombocytopenic purpura (CITP). Methods Fifty CITP patients were randomly divided to three groups, including 20 patients administrated with the expelling wind, cooling blood and invigorating kidney prescription as TCM group, 15 patients with the same decoction and prednisone as combine group, and 15 patients with prednisone as western medicine group, 4 weeks as one treatment course. After 3 courses, the change of platelet counts, marrow megakaryocyte amount, platelet-produced megakaryocyte proportion and TGF-β1 level in the marrow were observed. Results The fine efficiency of TCM group (55.0%, 11/20) excelled western medicine group (20.0%, 3/15) significantly and close to that of combine group (60.0%, 9/15). Platelet counts of TCM group was raised obviously after treatment (P<0.01), better than that of western medicine group (P<0.05), and was similar to that of combine group. The marrow megakaryocyte amount of TCM group was effectively decreased and the platelet-produced megakaryocyte proportion was increased greatly (P<0.01), similar to that of combine group, which of western medicine group had no significant difference between pre-therapy and post-therapy. TGF-β1 of TCM group and combine group degraded obviously (P<0.05) compared with that before treatment, and was significant deviation compared with that of western medicine group (P<0.05). Conclusion The expelling wind, cooling blood and invigorating kidney prescription can effectively degrade the value of TGF-β1 of CITP patients, facilitate the maturity of the marrow megakaryocyte amount and raise the platelet counts.
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Objective To investigate clinical significance of transforming growth factor-beta 1 ( TGF-β1 ) in patients with chronic idiopathic thrombocytopenic purpura(CITP). Methods The serum level of TGF-β1 in 38 pa-tients with initial CITP were detected using enzyme-linked immunosorbent assay(ELISA). Results The serum level of TGF-β1 in initial patients with CITP was significantly higher than that of the controls [( 132.57±5.17) μg/L vs ( 76.81±4.42) μ/L] ( P <0.01 ). The serum level of TGF-β1 in those having good response after therapy was sig-nificantly lower than before treatment[(81.26±3.78)μg/L] (P <0.01 ). There was no difference in TGF-β1 be-tween nonremission [(123.49 ± 4.31 ) μg/L] and initial patients (P > 0.05 ). There was negative correlation between TGF-β1 and platelet count(r = -0. 342 ,P < 0.05 ) ,there was positive correlation between TGF-β1 and megakaryo-cyte count (r = 0.409, P < 0.01 ). Conclusions TGF-β1 partakes in the pathogenesis of CITP, the determination of which in patients with CITP is useful to judge the state of illness, which can be regarded as an assistant index of cur-ative effect.
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The understanding and treatment of Traditional Chinese Medicine(TCM) on chronic idiopathic thrombocytopenic purpura(CITP) is prosperous,while no conclusion is yielded.By digging out documentation and summarizing clinical experience,"Qi and Yin Discrimination","Viscera Discrimination"and "Meridians Discrimination"are concluded to formulate the idea that the treatment of CIPT should bebased on Qi and Yin in this article,which aims at identifying the understanding of TCM on CITP,and then guiding clinical practice to improve therapeutic effect.
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Idiopathic thrombocytopenic purpura is a common hemorrhagic disease of child,and the chronic type of it is easier to recur.This paper introduces Professor SHI Yu-min's experience in treating chronic idiopathic thrombocytopenic purpura with methods of strengthening kidney,benefiting qi and activating blood flow combining with tonification and regulation.In addition,this paper expounds Prof.SHI's characteristics and laws in herbal prescription.And a case is presented.
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PURPOSE: Chronic idiopathic thrombocytopenic purpura (CITP) is an autoimmune disease caused by autoantibodies reacting to certain antigens, and platelet glycoprotein (GP) IIb/IIIa and GP Ib/IX complexes are thought to be some of those antigens. However, the clinical significance of anti-GP autoantibodies in CITP patients is unknown. In this study, we investigated the clinical correlation between the presence of circulating autoantibodies against GP IIb/IIIa and GP Ib/IX, and disease activity. MEHTODS: From December 1997 to June 1998, 20 CITP patients were enrolled in this study. Autoantibodies against GP IIb/IIIa and GP Ib/IX in patient's sera during treatment were detected by immunoblotting, and their platelet counts at the initial evaluation and 6 month follow-up were compared according to the presence or the absence of antibodies. RESULTS: Autoantibodies to GP antigens were found in 40% (8/20) of the patients. Seven patients were positive for GP IIb/IIIa; 4 for GP Ib/IX and 3 for both. GP autoantibody-positive patients had lower mean platelet counts than GP autoantibody-negative patients at initial evaluation (133,000/microliter vs 172,000/microliter, P>0.05) and at 6 month follow-up (154,000/microliter vs 192,000/microliter, P>0.05). Detection of GP autoantibodies related more with active disease than with remission at initial evaluation (45.5%(5/11) vs 33.3%(3/9), P>0.05) and at 6 month follow-up (50.0%(5/10) vs 30.0% (3/10), P>0.05). There was no detection of GP-specific antibodies in 3 splenectomized patients. CONCLUSION: It is hard to conclude on our data alone that the presence of GP autoantibodies correlates with disease status in CITP, although it seems to associate with lower platelet counts.
Subject(s)
Humans , Antibodies , Autoantibodies , Autoimmune Diseases , Blood Platelets , Follow-Up Studies , Glycoproteins , Immunoblotting , Platelet Count , Purpura, Thrombocytopenic, IdiopathicABSTRACT
PURPOSE: Chronic idiopathic thrombocytopenic purpura (CITP) is an autoimmune disease caused by autoantibodies reacting to certain antigens, and platelet glycoprotein (GP) IIb/IIIa and GP Ib/IX complexes are thought to be some of those antigens. However, the clinical significance of anti-GP autoantibodies in CITP patients is unknown. In this study, we investigated the clinical correlation between the presence of circulating autoantibodies against GP IIb/IIIa and GP Ib/IX, and disease activity. MEHTODS: From December 1997 to June 1998, 20 CITP patients were enrolled in this study. Autoantibodies against GP IIb/IIIa and GP Ib/IX in patient's sera during treatment were detected by immunoblotting, and their platelet counts at the initial evaluation and 6 month follow-up were compared according to the presence or the absence of antibodies. RESULTS: Autoantibodies to GP antigens were found in 40% (8/20) of the patients. Seven patients were positive for GP IIb/IIIa; 4 for GP Ib/IX and 3 for both. GP autoantibody-positive patients had lower mean platelet counts than GP autoantibody-negative patients at initial evaluation (133,000/microliter vs 172,000/microliter, P>0.05) and at 6 month follow-up (154,000/microliter vs 192,000/microliter, P>0.05). Detection of GP autoantibodies related more with active disease than with remission at initial evaluation (45.5%(5/11) vs 33.3%(3/9), P>0.05) and at 6 month follow-up (50.0%(5/10) vs 30.0% (3/10), P>0.05). There was no detection of GP-specific antibodies in 3 splenectomized patients. CONCLUSION: It is hard to conclude on our data alone that the presence of GP autoantibodies correlates with disease status in CITP, although it seems to associate with lower platelet counts.
Subject(s)
Humans , Antibodies , Autoantibodies , Autoimmune Diseases , Blood Platelets , Follow-Up Studies , Glycoproteins , Immunoblotting , Platelet Count , Purpura, Thrombocytopenic, IdiopathicABSTRACT
PURPOSE: The aim of this study is to investigate the usefulness of responsiveness to high dose intravenous immune globulin G (IVIG) or oral prednisone therapy as preoperative predictors for splenectomy response in patients with chronic idiopathic thrombocytopenic purpura (ITP). METHODS: We reviewed retrospectively the charts of 23 patients who were admitted to Yonsei Medical Center, Wonju Christian Hospital, Ajou Medical College Hospital and Pochon CHA General Hospital under the diagnosis of chronic ITP and plenectomized from January 1990 to April 1999, below the age of 20. All of the patients had been treated with high dose IVIG and, or oral prednisone. The responses to the treatments were classified according to Berchtold and McMillan's criteria (1) complete response (CR) 50 103/L. RESULTS: Of 23 patients, 12 boys and 11 girls, the mean age at operation was 12.4 years (5.4~19.4 years), the mean duration from diagnosis to splenectomy was 47 months (6~173 months) and mean follow up was 33 months (3~95 months). Of the 6 patients with responses to oral prednisone, 5 had responses to splenectomy and of the 12 patients with reponses to IVIG, 10 had responses to splenectomy at 3 months. All of 14 patients with no response to oral prednisone and 4 patients with no response to IVIG were responsed to splenectomy at 3 months. Four patients relapsed during follow up and there was no serious complication following splenectomy. CONCLUSION: We concluded that a positive response to oral prednisone or IVIG may be associated with a positive response to subsequent splenectomy and splenectomy is an effective and safe treatment.
Subject(s)
Female , Humans , Diagnosis , Follow-Up Studies , Hospitals, General , Immunoglobulins, Intravenous , Prednisone , Purpura, Thrombocytopenic, Idiopathic , Retrospective Studies , SplenectomyABSTRACT
BACKGROUND: Idiopathic thromboycytopenic purpura(ITP) is one of the most common acquired bleeding disorders in childhood. The entity of chronic ITP is defined as the persistence of thrombocytopenia for more than 6 months from initial presentation. There are the controversies regarding the diagnosis and management of this disease. The aim of this study is to compare the responses to managements and clinical symptoms of children with chronic ITP. METHODS: We retrospectively analyzed patients with chronic ITP diagnosed and managed between Jan., 1992 and June, 1997. Data from 33 cases were collected. The length of follow-up ranged from 6 months to 5 years. Statistical analysis was done using Jandel Sigmastat software(version 2.0). RESULTS: 1) Mean age is 5.4+/-4.3 years. The ratio of female to male is 0.65. 2) Most common clinical symptoms at presentation were petechiae(81.8%) and mucosal bleeding(42.4%). The patients who presented only petechiae and purpurae were 18 cases(54.5%), both petechiae and mucosal bleeding were 9 cases(27.3%). 3) The platelet count was 43,015+/-39,912/mm3. Bone marrow examinations showed normal or increased megakaryocyte, no abnormal cells and normal cellularity. 4) The prolonged complete response lasting more than 6 months without maintenance therapy was attained in 15.2% of the patients. A continuous complete response was observed in 6.1% to prednisone and IV-gamma-globulin. A prolonged complete response to dexamethasone was in 23.8% and continuous complete response was in 14.3%. The splenectomy was underwent in 3 patients. All of them had a prolonged complete response. One of six patients who were treated with combination chemotherapy had a prolonged complete response. CONCLUSION: This analysis of chronic ITP patients suggests that splenectomy remains the most effective treatment and the next one is high dose dexamethasone therapy. But the number of patients was not enough, more attention should be given to the management of chronic idiopathic thrombocytopenic pupura(ITP).
Subject(s)
Child , Female , Humans , Male , Bone Marrow Examination , Dexamethasone , Diagnosis , Drug Therapy, Combination , Follow-Up Studies , Hemorrhage , Megakaryocytes , Platelet Count , Prednisone , Purpura , Purpura, Thrombocytopenic, Idiopathic , Retrospective Studies , Splenectomy , ThrombocytopeniaABSTRACT
BACKGROUND: Chronic idiopathic thrombocytopenic purpura is an autoimmune disorder caused by sequestration of antibody-sensitized platelets in the reticuloendothelial system. However, uncertainty as to the specificity, frequency and clinical significance of such antibodies still remains. So, we tried to further clarify the above uncertainty in childhood chronic idiopathic thrombocytopenic purpura. METHODS: We analyzed sera from 29 patients. Twenty six patients were chronic ITP who were admitted or followed up to the Department of Pediatrics, Severance Hospital, Yonsei University Medical College from August 1996 to March 1997 by employing a modified antigen-capture ELISA(MACE), flow cytometry and electrophoresis(SDS-PAGE) and immuno-blotting(IB) assays. Three patients with ITP less than 6 months after onset of ITP were included to know the possibility to differrentiate between acute ITP and chronic ITP in this study. RESULTS: 1) Glycoprotein(GP)-specific antibodies were found in 28% (8/29) of patients, with 2 patients having antibodies directed solely to Gp II b/III a, no patients holding antibodies specific only for GPI b/I X and 6 possessing antibodies against both anti-GP I b/I X and Gp II b/III a antigen. 2) The detection rate of GP-specific antibodies of flow cytometry was about 10%. The positivity of anti-GPI b/I X antibodies by MACE and immunoblotting was 14% (4/29), respectively, the positivity of anti-Gp II b/III a antibodies by MACE and immunoblotting was, 21 % (6/29) respectively. The concordance rate between two assays(MACE and IB) was 79% (23/29). None of the three methods was good enough to stand alone. 3) Serum antibodies were not more frequently detected in active(p=1.0) or non-splenectomized(p=.54) chronic ITP patients. 4) No association was found between antibody specificity(anti-GPI b/I X, anti-Gp II b/ III a) and platelet counts(p : .87). CONCLUSION: We conclude that in korean childhood chronic ITP, antibodies against both anti-GPI b/I X and Gp II b/III a antigen were predominant antibody. But, the longterm follow-up in more cases is needed to further clarify the clinical significance of antral-platelet antibody in chronic ITP should be assessed.
Subject(s)
Humans , Antibodies , Blood Platelets , Flow Cytometry , Follow-Up Studies , Immunoblotting , Mononuclear Phagocyte System , Pediatrics , Purpura, Thrombocytopenic, Idiopathic , Sensitivity and Specificity , UncertaintyABSTRACT
Objective To investigate the changes of protein kinase C(PKC)activity in peripheral blood T lymphocytes in children with chronic idiopathic thrombocytopenic purpura(CITP)and the relationship between PKC activity,T lymphocytes activation and thrombocytes decrease.Methods Collecting sterile peripheral blood from CITP children(n=30)and healthy children(n=30),T lymphocytes were isolated and purified by the T cell segregation enrichment column,the PKC activity was detected by non-radioactive assay.Soluble interleukin-2 receptor(sIL-2R),which was T cell activated marker,was determined by enzyme-linked immunoabsorbent assay(ELISA),platelet was counted by cell counting meter.Results Compared with healthy children,PKC activity was significantly enhanced in CITP children[(0.94?0.23)mmol/(min?L)vs(0.50?0.17)mmol/(min?L),t= 8.42 P