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Immune Thrombocytopenic Purpura (immune + thrombocytopenic_purpura)

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Medical Sciences	100%

Selected Abstracts

Immune thrombocytopenic purpura: epidemiology and implications for patients

EUROPEAN JOURNAL OF HAEMATOLOGY, Issue 2009
Marc Michel
Abstract The age-adjusted prevalence of immune thrombocytopenic purpura (ITP) is estimated to be 9.5 per 100 000 persons in the USA while its annual incidence is estimated to be 2.68 per 100 000 in Northern Europe (at a cut-off platelet count of <100 × 109/L). The mean age of adults at diagnosis in Europe is 50 yrs and the incidence of ITP increases with age. Both the treatments used to treat patients with ITP and the disease itself can impact on patient health-related quality of life (HRQoL). As the incidence of ITP in Europe rises, especially in the elderly, the number of patients with a decreased HRQoL is increasing. Literature searches and focus groups have aided the development of a conceptual model to assess HRQoL. In this model, low platelet counts and the associated symptoms of ITP in addition to the side effects of treatment are proposed as the main determinants of a negatively impacted HRQoL. Primary conceptual domains of HRQoL, affected in patients with ITP, include emotional health, functional health, work, social and leisure activities and reproductive health. As treatment benefits are likely to improve these domains, the conceptual model could be used for better management of patients, taking into account HRQoL. The short-form 36-item questionnaire (SF-36) and the ITP Patient Assessment Questionnaire (ITP-PAQ) are validated measures of HRQoL which can provide a comprehensive assessment of numerous factors to help evaluate decisions about patient management. Future clinical trials investigating treatment options for ITP should assess HRQoL using these validated questionnaires. [source]

Epidemiology and pathophysiology of immune thrombocytopenic purpura

EUROPEAN JOURNAL OF HAEMATOLOGY, Issue 2008
Terry Gernsheimer
Abstract Immune thrombocytopenic purpura (ITP) is characterized by the presence of antiplatelet antibodies and immune platelet destruction. The disorder has been described as having a predilection for young women over men. Bone marrow megakaryocytes appear morphologically and quantitatively normal, and early platelet kinetic studies were consistent with reduced platelet survival as the primary abnormality in ITP. During the last 10,20 yr, understanding of the kinetics of this disorder has evolved with evidence that platelet survival is not as abbreviated as previously thought. Thrombopoietin levels are only minimally elevated, if at all, suggesting marrow stimulation and platelet production may not be maximized. Megakaryocyte physiology appears to be altered in ITP, also suggestive of diminished platelet production. It appears both platelet survival and production are impaired in ITP. The epidemiology of ITP is reviewed here and the pathophysiology of ITP is reconsidered. [source]

Autoimmune thrombocytopenia: flow cytometric determination of platelet-associated autoantibodies against platelet-specific receptors

JOURNAL OF THROMBOSIS AND HAEMOSTASIS, Issue 1 2005
A. TOMER
Summary., Immune thrombocytopenic purpura (ITP) is an autoimmune disorder characterized by antibody-induced platelet destruction. Despite its clinical importance, the diagnosis of ITP is one of exclusion, thus, inevitably associated with potential difficulties. We here describe a feasible diagnostic method using the commonly available technique of flow cytometry. An antigen-specific assay for platelet-associated antibody was developed and tested in 62 adult patients with chronic ITP, 14 patients with thrombocytopenia of decreased production and 60 healthy controls. The method is based on flow cytometric (FCM) detection of autoantibodies reacting with specific platelet receptors immobilized on microbeads. The average fluorescence level in the ITP group calculated as a ratio to normal was 4.07 (range 0.8,31.0), in the non-ITP thrombocytopenic patients 0.9 (range 0.7,1.2), and in the healthy controls 1.0 (range 0.7,1.3). The average assay coefficient of variation was 0.218 [95% confidence interval (CI) 0.213, 0.221]. The difference between the ITP patients and both groups was highly significant (P < 0.001), using a stringent non-parametric analysis. A comparison of the FCM assay with the radioactive immunobead assay previously reported on the same cohort of patients showed significant correlation (R2 = 0.71, 95% CI 0.39, 0.53). The overall performance of the FCM assay in discriminating between ITP patients and normals was estimated by the receiver operating characteristic (ROC) plot, showing an area under the curve of 0.96 (maximal value 1.0), with standard error of 0.033. We conclude that the present FCM assay is clinically useful for routine diagnosis and follow-up of ITP. [source]

Cost and mortality associated with hospitalizations in patients with immune thrombocytopenic purpura,

AMERICAN JOURNAL OF HEMATOLOGY, Issue 10 2009
Mark D. Danese
Immune thrombocytopenic purpura (ITP) is associated with low platelet counts and, consequently, a high risk of adverse events leading to hospitalization. However, there are few data on the clinical and economic burden of hospitalizations for ITP. The Nationwide Inpatient Sample (NIS) database of discharges, a stratified 20% sample of all United States (US) community hospitals across all payers, was used to evaluate discharges in ITP patients. We developed nationally representative numbers of discharges in ITP patients from 2003 to 2006 based on diagnosis codes. Using appropriate weights for each NIS discharge, we created national estimates of average cost, length of stay, and in-hospital mortality for specific groups of ITP-related hospitalizations. Approximately 129,000 discharges occurred between 2003 and 2006 in ITP patients. The average cost associated with all discharges in 2008 dollars was 16,476, with a 6.4-day length of stay and in-hospital mortality of 3.8%. In contrast, the average cost of all hospitalizations in the US population during the same period was 10,039, the average length of stay was 4.8 days, and in-hospital mortality was 2.5%. Mortality risk was higher for ITP patients than for the standard US population adjusted for age and gender, with a relative mortality ratio of 1.5 (95% CI: 1.4,1.6). On the basis of a nationally representative sample of US discharge records from 2003 to 2006, hospitalization with ITP represents an economically and clinically important event. ITP was associated with higher costs, longer stays, and more in-hospital deaths on average than all other hospitalized patients combined. Am. J. Hematol. 2009. © 2009 Wiley-Liss, Inc. [source]

Corticosteroid side-effects and risk for bleeding in immune thrombocytopenic purpura: patient and hematologist perspectives

EUROPEAN JOURNAL OF HAEMATOLOGY, Issue 3 2009
Jacqueline A. Guidry
Abstract Objectives:, The purpose of this study was to examine hematologist and patient perspectives about the side-effects of the corticosteroid treatment of immune thrombocytopenic purpura (ITP) and their perspectives about the patient's risk for bleeding. The specific aim was to compare patient and hematologist perspectives and, if a difference was documented, the implications of that difference. We hypothesized that patients with ITP may have more concern about corticosteroid side-effects and less concern about serious bleeding than hematologists. Methods:, We surveyed 80 patients in the Oklahoma ITP Registry and all 83 hematologists in Oklahoma about the occurrence and severity of 18 corticosteroid side-effects and risks for serious bleeding. Results:, Response rates were 80% (patients) and 71% (hematologists). Responses of patients and hematologists were significantly different from each other regarding both the frequency of severe corticosteroid side-effects and the risk of serious bleeding. For 13 of the 18 corticosteroid side-effects, patients reported more frequent occurrence of severe symptoms than hematologists (P < 0.05); physicians reported more frequent occurrence for one side-effect (P < 0.05). Conversely, 69% and 93% of hematologists reported being very worried about serious bleeding when responding to two case scenarios describing patients with platelet counts of 10 000/,L and 5000/,L (P < 0.05), compared with only 16 (31%) of 51 patients whose lowest platelet count had been <10 000/,L. Conclusion:, Awareness of the different opinions about corticosteroid side-effects and risk for bleeding between ITP patients and hematologists may improve management decisions. [source]

Immune thrombocytopenic purpura: epidemiology and implications for patients

Rituximab therapy in adult patients with relapsed or refractory immune thrombocytopenic purpura: long-term follow-up results

EUROPEAN JOURNAL OF HAEMATOLOGY, Issue 3 2008
Marta Medeot
Abstract Objective:, To evaluate the long-term activity and toxicity profile of rituximab in adult patients with idiopathic immune thrombocytopenic purpura (ITP). Patients and methods:, Twenty-six patients with active and symptomatic ITP relapsed or refractory received weekly infusions of rituximab 375 mg/m2 for 4 wk. Median time from diagnosis to rituximab was 34.5 months. The following parameters of efficacy and toxicity were considered: complete response (CR) and partial response (PR), relapse rate, relapse-free survival (RFS), therapy-free survival (TFS), short- and long-term toxicity. Results:, CR and PR were 14/26 (54%) and 4/26 (15%), respectively. Median time of observation was 56.5 months (range 39,77). Nine of the 18 responding patients relapsed after a median of 21 months (range 8,66); 9/26 patients (35%) maintained the response, with a median follow-up of 57 months (range 39,69), and 11/26 (42%) did not necessitate further therapy; estimated 5 yr RFS and TFS were 61% and 72%, respectively. Younger age and shorter interval from diagnosis to rituximab appeared indicators of better outcome. Rituximab administration was associated with two episodes of short-term toxicity, with one case of serum sickness syndrome; no infectious or other significant long-term complications were documented. Conclusion:, Rituximab therapy may achieve long-lasting remission in nearly one-third of patients with relapsed or refractory ITP, with a good safety profile. [source]

Epidemiology and pathophysiology of immune thrombocytopenic purpura

Plasma-soluble Fas (APO-1, CD95) and soluble Fas ligand in immune thrombocytopenic purpura

EUROPEAN JOURNAL OF HAEMATOLOGY, Issue 4 2000
Chie Yoshimura
Abstract: We investigated the levels of various cytokines and soluble factors in ITP patients, in order to determine the influence of these factors on the pathogenesis of ITP. We found increases in IL-2, IL-6, IFN-,, and M-CSF levels in ITP patients compared with those in healthy individuals. On lymphocyte phenotype analysis, we found no clear difference in total T cell population (CD2+ CD19, cells) or cytotoxic T cell frequency (CD8+ CD11b, cells) between these two groups. The frequency of helper/inducer T cells (CD4+ CD8, cells) was decreased in ITP patients. There was a significant increase in activated T cells (CD3+ HLA-DR+ cells) in ITP patients. Furthermore, frequencies of NK cells of potent activity (CD16+ CD56+ cells) were significantly elevated in ITP patients. Seventeen of the 54 ITP patients (31.5%) had elevated levels of sFas, and 11 of the 54 patients (20.4%) of sFasL. In addition, a significant increase of sFasL was observed in sFas-positive ITP patients, and in these patients the sFasL level was correlated with that of sFas (r=0.687, p<0.01). We found significant increases in IL-2 and sIL-2R levels in sFas-positive ITP patients. For other factors examined, however, there were no differences in level between sFas-positive and-negative ITP patients. Percentages of activated T cells (CD3+ and HLA-DR+ cells) and NK cells (CD16+ and CD56+ cells) were significantly higher in sFas-positive ITP patients than in sFas-negative ITP patients. These findings suggests that the pathogenesis of ITP includes alteration of the Fas/FasL pathway. [source]

Indirect study of thrombopoiesis(TPO, reticulated platelets, glycocalicin)in patients with hereditary macrothrombocytopenia

EUROPEAN JOURNAL OF HAEMATOLOGY, Issue 3 2000
F. Fabris
To better understand the pathogenesis of thrombopoiesis in this hereditary thrombocytopenic disorder, we determined the percentage of reticulated platelets (RP), plasma glycocalicin (GC) and thrombopoietin (TPO) levels in 29 patients with CHMT, 23 patients with immune thrombocytopenic purpura (ITP), and 17 patients with thrombocytopenia secondary to decreased bone marrow megakaryocytes (hypoplasia). The % RP was similar in CHMT (2.27±1.33) and hypoplasia (1.98±1.35) patients and markedly lower than that in ITP patients (8.80±7.97; p<0.001), suggesting that the production of new platelets is reduced in CHMT. Plasma GC was within the normal range (0.84±0.16 ,g/mL) both in patients with CHMT (0.63±0.20 ,g/mL) and ITP (0.82±0.90 ,g/mL), while it was significantly decreased in patients with hypoplasia (0.16±0.04 ,g/mL; p<0.001). When the GC value was normalized for platelet count, the GC index was normal in CHMT patients (2.05±1.1) and in patients with hypoplasia (0.85±0.10) while it was significantly increased in ITP patients (10.88±18.00; p<0.001); thus, patients with CHMT seem to have a normal platelet turnover. TPO was significantly increased in CHMT (195±72 pg/ml) as compared with normal (80±53 pg/ml; p<0.002); however, the mean level was not as high as in ITP patients (345±167 pg/mL; p<0.001). This finding suggests that CHMT syndrome is not secondary to a defective production of TPO and that megakaryocyte mass is nearly normal. [source]

An acquired inhibitor to the GPVI platelet collagen receptor in a patient with lupus nephritis

JOURNAL OF THROMBOSIS AND HAEMOSTASIS, Issue 9 2009
P. NURDEN
Summary.,Background: GPVI is a major platelet collagen signaling receptor. In rare cases of immune thrombocytopenic purpura (ITP), autoantibodies to GPVI result in receptor shedding. Objectives: To investigate a possible pathogenic role of plasma anti-GPVI antibody located in a woman with lupus nephritis. Methods: Measured were (i) platelet aggregation to collagen and convulxin, (ii) platelet GPVI expression (flow cytometry and western blotting), (iii) plasma soluble GPVI (sGPVI, dual antibody ELISA), and (iv) plasma anti-GPVI antibody (ELISA using recombinant sGPVI). Results: In 2006 and early 2007, the patient had a normal platelet count but a virtual absence of platelet aggregation to collagen and convulxin. Her platelets responded normally to other agonists including cross-linking ITAM-dependent Fc,RIIA by monoclonal antibody, IV.3. Flow cytometry and western blotting showed a platelet deficiency of GPVI. Plasma sGPVI levels were undetectable whereas ELISA confirmed the presence of anti-GPVI antibody. Sequencing revealed a normal GPVI cDNA structure. The patient's plasma and the isolated IgG3 fraction activated and induced GPVI shedding from normal platelets. A deteriorating clinical condition led to increasingly strict immunosuppressive therapy. This was globally associated with a fall in plasma anti-GPVI titres, the restoration of platelet GPVI and the convulxin response, and the loss of her nephrotic syndrome. Conclusions: Our results show that this patient acquired a potent anti-GPVI IgG3 antibody with loss of GPVI and collagen-related platelet function. Further studies are required to determine whether anti-GPVI antibodies occur in other lupus patients with nephritis. [source]

Compromised ITAM-based platelet receptor function in a patient with immune thrombocytopenic purpura

JOURNAL OF THROMBOSIS AND HAEMOSTASIS, Issue 7 2008
E. E. GARDINER
Summary.,Background:,Receptors on platelets that contain immunoreceptor tyrosine-based activation motifs (ITAMs) include collagen receptor glycoprotein (GP) VI, and Fc,RIIa, a low affinity receptor for immunoglobulin (Ig) G. Objectives:,We examined the function of GPVI and Fc,RIIa in a patient diagnosed with immune thrombocytopenic purpura (ITP) who had unexplained pathological bruising despite normalization of the platelet count with treatment. Methods and Results:,Patient platelets aggregated normally in response to ADP, arachadonic acid and epinephrine, but not to GPVI agonists, collagen or collagen-related peptide, or to Fc,RII-activating monoclonal antibody (mAb) 8.26, suggesting ITAM receptor dysfunction. Plasma contained an anti-GPVI antibody by MAIPA and aggregated normal platelets. Aggregating activity was partially (,60%) blocked by Fc,RIIa-blocking antibody, IV.3, and completely blocked by soluble GPVI ectodomain. Full-length GPVI on the patient platelet surface was reduced to ,10% of normal levels, and a ,10-kDa GPVI cytoplasmic tail remnant and cleaved Fc,RIIa were detectable by western blot, indicating platelet receptor proteolysis. Plasma from the patient contained ,150 ng mL,1 soluble GPVI by ELISA (normal plasma, ,15 ng mL,1) and IgG purified from patient plasma caused Fc,RIIa-mediated, EDTA-sensitive cleavage of both GPVI and Fc,RIIa on normal platelets. Conclusions:,In ITP patients, platelet autoantibodies can curtail platelet receptor function. Platelet ITAM receptor dysfunction may contribute to the increased bleeding phenotype observed in some patients with ITP. [source]

Identification of an epitope on glycoprotein IIb-IIIa that is recognized by HLA-DRB1*0405-restricted CD4+,superior' T cells from a patient with immune thrombocytopenic purpura

JOURNAL OF THROMBOSIS AND HAEMOSTASIS, Issue 2 2004
J. Yamanouchi
No abstract is available for this article. [source]

Acute renal failure in a patient with antiphospholipid syndrome and immune thrombocytopenic purpura treated with eltrombopag,

AMERICAN JOURNAL OF HEMATOLOGY, Issue 9 2010
C. John Sperati
No abstract is available for this article. [source]

The incidence of immune thrombocytopenic purpura in children and adults: A critical review of published reports,

AMERICAN JOURNAL OF HEMATOLOGY, Issue 3 2010
Deirdra R. Terrell
Reports of the incidence of ITP are few and their methodology is variable. Accurate estimates of the incidence of immune thrombocytopenic purpura (ITP) are important to understand the medical and public health impact of the disease. To critically review all published reports on the incidence of ITP in children and adults, all articles identified on the Medline database (searched January 1, 1966-August 7, 2009) that reported data on the incidence of ITP were retrieved. Articles which directly estimated the incidence of ITP were selected for review. Eight articles reported the incidence of acute ITP in children. After review, four were determined to have the strongest estimates, based on the method of patient identification and study design. The lowest incidence estimate in these four studies was 2.2 per 105 children/year (95% confidence interval 1.9, 2.4) and the highest incidence estimate was 5.3 per 105 children/year (95% confidence interval 4.3, 6.4). Three studies reported the incidence of ITP in adults. The estimate from the article with the strongest methodology reported an incidence estimate of 3.3 per 105 adults/year. The current strongest estimate of the incidence of acute ITP in children is between 1.9 and 6.4 per 105 children/year; for adults the current strongest estimate of the incidence of ITP is 3.3 per 105 adults/year. An important limitation of these studies is that they are primarily from Europe and may not be generalizable to all regions. Am. J. Hematol. 2010. © 2009 Wiley-Liss, Inc. [source]

Cost and mortality associated with hospitalizations in patients with immune thrombocytopenic purpura,

Repeated courses of rituximab in chronic ITP: Three different regimens,

AMERICAN JOURNAL OF HEMATOLOGY, Issue 10 2009
Aisha Hasan
This study investigated responses to retreatment with rituximab in chronic immune thrombocytopenic purpura (ITP) patients. Treatment with rituximab in chronic ITP patients induces long-lasting responses in ,30% of patients but even these patients may relapse. Twenty patients who had achieved a response to rituximab and relapsed were retreated with rituximab (375 mg/m2× 4); this data was analyzed retrospectively. Subsequently, 16 patients were prospectively randomized to receive rituximab with cyclophosphamide, vincristine and prednisone (R-CVP) or double dose rituximab (DDR). Retreatment with standard dose rituximab demonstrated responses similar to initial rituximab treatment in 15 of 20 patients. Neither of the two more intensive regimens (R-CVP, DDR) induced responses in any patient who had previously failed to respond to rituximab nor induced substantially longer-lasting responses among previous responders. No additional toxicity was noted with the DDR regimen, whereas R-CVP was not well tolerated. These results suggest that retreatment with standard dose rituximab induces similar responses in 75% of previously responding patients and is well tolerated. Neither combining rituximab with CVP nor doubling the dose of rituximab increased the response rate. Am. J. Hematol., 2009. © 2009 Wiley-Liss, Inc. [source]

Postvaccination hyperhemolysis coinciding with remission of Epstein Barr virus (EBV)-associated immune thrombocytopenic purpura (ITP),,

AMERICAN JOURNAL OF HEMATOLOGY, Issue 9 2009
Christine M. Cserti-Gazdewich
No abstract is available for this article. [source]

A case of immune thrombocytopenic purpura presenting with recurrent intracranial hemorrhage

PEDIATRICS INTERNATIONAL, Issue 1 2005
Ali Bay
No abstract is available for this article. [source]

Fatal case of bilateral internal jugular vein thrombosis following IVIg infusion in an adolescent girl treated for ITP

AMERICAN JOURNAL OF HEMATOLOGY, Issue 4 2008
Pui-Ying Iroh Tam
Intravenous immunoglobulin (IVIg) is often used as therapy in immune-mediated diseases and is generally considered a safe therapeutic agent. However, thrombotic complications such as myocardial infarction and deep vein thrombosis have been reported, although primarily in older adults. We describe a 13-year-old girl who received one dose of IVIg for immune thrombocytopenic purpura and developed fatal bilateral jugular venous thromboses. This is the first known case of IVIg-associated thrombosis in an adolescent and alsothe first report describing internal jugular vein thrombosis associated with IVIg infusion. We identify additional risks that may potentiate the agent's thrombotic risk. Am. J. Hematol., 2008. © 2007 Wiley-Liss, Inc. [source]

Antiphospholipid antibodies (APLA) in immune thrombocytopenic purpura (ITP) and antiphospholipid syndrome (APS)

AMERICAN JOURNAL OF HEMATOLOGY, Issue 6 2006
Carlos J. Bidot
Abstract Antiphospholipid antibodies (APLA) are associated with anti-phospholipid syndrome (APS), a thrombotic disorder, but they are also frequently detected in immune thrombocytopenic purpura (ITP), a bleeding disorder. To investigate possible differences of APLA between these two disorders, we assayed IgG and IgM APLA by ELISA in 21 patients with ITP and 33 with APS. The APLA reacting against two protein target antigens, ,2 -glycoprotein 1 (,2GP1) and FVII/VIIa, and four phospholipids [cardiolipin (CL), phosphatidylcholine (PC), phosphatidylserine (PS), and phosphatidylethanolamine (PE)] as well as lupus anticoagulant (LA) were analyzed. We made the following observations: (i) IgG and IgM antibodies to ,2GP1 and IgM antibodies to FVII/VIIa were more common in APS than ITP, P < 0.05, while IgG antibodies against the phospholipids (aCL, aPC, aPS, aPE) were more common in ITP than APS, P < 0.05; (ii) multiple APLA ,3 antigens) were more frequent in APS than ITP, P < 0.05; (iii) LA was frequently associated with APS but was absent in ITP; (iv) APLA is quite common in ITP: two-thirds were positive for at least one APLA. In summary, APLA are prevalent in ITP but their profile differs from APS. In APS, antibodies were predominantly against ,2GP1 and 80% had positive LA, while in ITP the APLA reacted most often with the phospholipids without LA. The difference in APLA may result in opposite clinical manifestations in two disorders. Am. J. Hematol. 81:391,396, 2006. © 2006 Wiley-Liss, Inc. [source]

Acute renal failure after intravenous anti-D immune globulin in an adult with immune thrombocytopenic purpura

AMERICAN JOURNAL OF HEMATOLOGY, Issue 4 2003
Nancy S. Chun
Abstract Intravenous anti-D immune globulin (anti-D IGIV) is indicated for the treatment of immune thrombocytopenic purpura (ITP) in nonsplenectomized patients who are Rh(D)-positive. Recent reports have described episodes of intravascular hemolysis (IVH) and acute renal failure (ARF) after anti-D IGIV. We report the first adult patient with ITP who required and received dialysis after IVH and ARF complicating treatment with anti-D IGIV. Whether the transfusion of 2 units of Rh(D)-positive red cells, indicated for the resulting anemia, exacerbated the IVH and renal failure is unclear. Three weeks after the administration of anti-D IGIV (13 days after two hemodialysis treatments), the patient's renal function had returned to normal. This case highlights the infrequent but potentially serious side effects of anti-D IGIV and the need to monitor a patient's renal function closely if there is evidence of IVH after infusion of anti-D IGIV. If red cell transfusion is indicated, we recommend the use of Rh(D)-negative red cell products. Am. J. Hematol. 74:276,279, 2003. © 2003 Wiley-Liss, Inc. [source]

Single-port laparoscopic splenectomy: The first three cases

ASIAN JOURNAL OF ENDOSCOPIC SURGERY, Issue 1 2010
Y.K. You
Abstract In the past two decades, laparoscopic surgery has replaced open surgery in most abdominal surgeries, including splenectomies for which it has become the standard. Single-port laparoscopic surgery is a newly emerging surgical technique that decreases postoperative scarring and parietal trauma. Herein we report on three cases of splenectomy in which single-port laparoscopic surgery technique was applied. Between October 2008 and January 2009, a 13-year-old male suffering from grade-III splenic trauma and two females, aged 33 and 61, respectively, and both diagnosed with immune thrombocytopenic purpura, underwent single-port laparoscopic splenectomies. Preoperative and postoperative management, including vaccination, was performed in a routine manner. A 3.5 cm transverse incision at the anterior axillary line at umbilicus level was used as a single-port entry point. The entire procedure took 195, 125 and 133 minutes, respectively. All patients recovered and were discharged without any complications. [source]

Complement activation on platelets correlates with a decrease in circulating immature platelets in patients with immune thrombocytopenic purpura

BRITISH JOURNAL OF HAEMATOLOGY, Issue 4 2010
Ellinor I. B. Peerschke
Summary The role of the complement system in immune thrombocytopenic purpura (ITP) is not well defined. We examined plasma from 79 patients with ITP, 50 healthy volunteers, and 25 patients with non-immune mediated thrombocytopenia, to investigate their complement activation/fixation capacity (CAC) on immobilized heterologous platelets. Enhanced CAC was found in 46 plasma samples (59%) from patients with ITP, but no samples from patients with non-immune mediated thrombocytopenia. Plasma from healthy volunteers was used for comparison. In patients with ITP, an enhanced plasma CAC was associated with a decreased circulating absolute immature platelet fraction (A-IPF) (<15 × 109/l) (P = 0·027) and thrombocytopenia (platelet count < 100 × 109/l) (P = 0·024). The positive predictive value of an enhanced CAC for a low A-IPF was 93%, with a specificity of 77%. The specificity and positive predictive values increased to 100% when plasma CAC was defined strictly by enhanced C1q and/or C4d deposition on test platelets. Although no statistically significant correlation emerged between CAC and response to different pharmacological therapies, an enhanced response to splenectomy was noted (P < 0·063). Thus, complement fixation may contribute to the thrombocytopenia of ITP by enhancing clearance of opsonized platelets from the circulation, and/or directly damaging platelets and megakaryocytes. [source]

Single nucleotide polymorphisms of the inflammatory cytokine genes in adults with chronic immune thrombocytopenic purpura

BRITISH JOURNAL OF HAEMATOLOGY, Issue 6 2004
Takashi Satoh
Summary Single nucleotide polymorphisms (SNPs) of inflammatory cytokine genes were examined in 84 adult Japanese patients with chronic immune thrombocytopenic purpura (ITP) and 56 race-matched healthy controls. The SNPs examined were within the genes encoding tumour necrosis factor (TNF)- , (,238 G/A and ,308 G/A), TNF- , (+252 G/A), and interleukin (IL)-1, (,511 C/T and +3953 T/C). Of these SNPs, the frequency of the TNF- , (+252) G/G phenotype was significantly higher in ITP patients than in healthy controls (21% vs. 7%, P = 0·04, odds ratio = 3·6, 95% confidence interval 1·1,11·1), while no significant association was detected for the other SNPs. The distribution of the TNF- , (+252) phenotype was not associated with human leucocyte antigen class II alleles or the therapeutic response in ITP patients. The frequency of circulating anti-glycoprotein IIb/IIIa antibody-producing B cells was significantly higher in ITP patients with the TNF- , (+252) G/G phenotype than in those with the G/A or A/A phenotype (11·9 ± 4·9 vs. 6·8 ± 4·9 and 3·7 ± 2·8 per 105 peripheral blood mononuclear cells; P = 0·02 and P < 0·001, respectively). These findings suggest that the SNP located at TNF- , (+252) contributes to susceptibility to chronic ITP by controlling the autoreactive B-cell responses to platelet membrane glycoproteins. [source]