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Thrombocytopenic Purpura (thrombocytopenic + purpura)
Kinds of Thrombocytopenic Purpura Selected AbstractsProof of an Association between Helicobacter pylori and Idiopathic Thrombocytopenic Purpura in Latin AmericaHELICOBACTER, Issue 3 2007Germán Campuzano-Maya Abstract Background:, Association between Helicobacter pylori and idiopathic thrombocytopenic purpura (ITP) has been found in Japan and in some European countries. It has also been shown that eradication of H. pylori can increase platelet counts in patients with ITP. The aims of this study were to determine the prevalence of H. pylori infection in patients with ITP in Colombia, and the effect of bacterial eradication on their platelet counts. Materials and methods:, Between December 1998 and April 2006, a total of 32 patients diagnosed with ITP were included in the study. Controls were age and sex matched. Results:,H. pylori infection in patients with ITP was significantly higher (p = .00006) than in control individuals (90.6% and 43.8%, respectively), as determined by 13C-urea breath test. A significant association between H. pylori infection and ITP was found (p < .0003), with an odds ratio (OR) of 13.15 (95%CI: 3.24,53.29). Multivariate analysis for the association between H. pylori and ITP showed an OR of 20.44 (95%CI: 3.88,107.49) for women and 19.28 (95%CI: 2.03,183.42) for individuals over 50 years. All 29 H. pylori -positive patients with ITP received eradication treatment. After a median follow up of 12.2 months, 80.8% had a recovery in platelet counts. Conclusions:, According to these results and others from different countries where H. pylori infection rates are high, patients with ITP should be initially tested for H. pylori status, and if present, infection should be eradicated before initiating a drastic conventional ITP treatment. An algorithm for the study and management of patients with ITP in the post- Helicobacter era is presented. [source] Extensive Brain Stem Lesions in Thrombotic Thrombocytopenic Purpura: Repeat Magnetic Resonance FindingsJOURNAL OF NEUROIMAGING, Issue 1 2005Sun Ah Park MD ABSTRACT The authors report on an unusual case of extensive brain stem lesions as a manifestation of thrombotic thrombocytopenic purpura (TTP). A 28-year-old woman developed rapidly progressive neurologic deficits 5 days after a cesarean delivery. Her condition had been normal after delivery. Initial magnetic resonance imaging (MRI) revealed extensive T2 hyperintense lesions involving the entire brain stem; only part of the pons showed hyperintense abnormalities in a concomitantly taken diffusion-weighted image. The hematologic evaluations and her clinical course revealed the diagnosis of TTP, so plasma exchange and methyl-prednisolone therapy were initiated. After 10 days of treatment, she developed neurologic improvement. A follow-up MRI on the 75th day revealed dramatically reduced brain stem lesions with only residual punctate lesions in the pons. Her remaining neurologic deficits were dysarthria, limb ataxia, and left hemiparesis. As demonstrated in this study, extensive brain stem involvement should be added as a possible neuroimaging feature of TTP. [source] Reticulated platelet counts correlate with treatment response in patients with idiopathic thrombocytopenic purpura and help identify the complex causes of thrombocytopenia in patients after allogeneic hematopoietic stem cell transplantationCYTOMETRY, Issue 4 2007Anna-Katharina Thomas-Kaskel Abstract Background: In thrombocytopenic conditions of unknown origin, quantification of reticulated platelets (RP) in the peripheral blood by flow cytometry has been shown to differentiate increased platelet (Plt) turnover from insufficient Plt production. Methods: We used a whole blood flow cytometry method combining thiazole orange and anti-CD41a-staining to assess RP in 71 healthy subjects, six with thrombocytopenic myelodysplastic syndrome (MDS), nine with liver cirrhosis, 14 patients with idiopathic thrombocytopenic purpura (ITP), and 12 patients who had undergone hematopoietic stem cell transplantation (HSCT). Results: Patients with MDS had normal, patients with liver cirrhosis had slightly elevated RP counts compared to healthy subjects. ITP patients had elevated RP counts, and RP >15% were associated with treatment response (P = 0.015). In 7/10 patients after HSCT, an increase of RP preceded Plt recovery, whereas in patients with secondary thrombocytopenia after normal regeneration, the assessment of RP allowed the differentiation between conditions with high Plt turnover, such as GvHD and microangiopathy, indicated by high RP counts, and graft failure, indicated by low RP counts. Conclusions: Our data provide the rationale for prospective studies on the diagnostic and prognostic value of RP counts in larger patient populations with ITP and after HSCT. © 2007 Clinical Cytometry Society [source] Eradication of Helicobacter pylori increases platelet count in patients with idiopathic thrombocytopenic purpura in JapanEUROPEAN JOURNAL OF CLINICAL INVESTIGATION, Issue 3 2005T. Inaba Abstract Background, The effect of Helicobacter pylori eradication on the platelet count in patients with thrombocytopenic purpura is controversial. In this multicentre study, we prospectively assessed the effect of H. pylori eradication therapy in idiopathic thrombocytopenic purpura patients. Materials and methods, Thirty-five consecutive patients with chronic idiopathic thrombocytopenic purpura (11 males and 24 females, a median age of 57) were assessed for H. pylori infection by use of a urea breath test. All patients received 1-week triple therapy (amoxicillin, clarithromycin, and lansoprazole) to eradicate H. pylori. At 6 months, idiopathic thrombocytopenic purpura patients with a platelet count recovery of greater than 100 × 109 L,1 were defined as idiopathic thrombocytopenic purpura responders. Results,Helicobacter pylori infection was observed in 25 (71%) of the 35 patients. All infected patients were cured. Eleven patients were identified as idiopathic thrombocytopenic purpura responders; 24 were considered nonresponders. Platelet counts improved by more than 100 × 109 L,1 in 11 (44%) of the 25 patients cured of H. pylori infection, while none of the 10 patients H. pylori -negative patients experienced the same improvement (P = 0·015). Univariate analysis showed that H. pylori infection and its eradication were significant factors associated with platelet recovery (P = 0·015). Conclusions,Helicobacter pylori infection played a role in the pathogenesis of idiopathic thrombocytopenic purpura in approximately 30% of all patients assessed and 45% of the patients with H. pylori infection. Eradication of H. pylori in idiopathic thrombocytopenic purpura patients led to improved disease activity. [source] Corticosteroid side-effects and risk for bleeding in immune thrombocytopenic purpura: patient and hematologist perspectivesEUROPEAN JOURNAL OF HAEMATOLOGY, Issue 3 2009Jacqueline 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 patientsEUROPEAN JOURNAL OF HAEMATOLOGY, Issue 2009Marc 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] Aberrant increase in the immature platelet fraction in patients with myelodysplastic syndrome: a marker of karyotypic abnormalities associated with poor prognosisEUROPEAN JOURNAL OF HAEMATOLOGY, Issue 1 2009Naomi Sugimori Abstract Objectives:, Some patients with myelodysplastic syndrome (MDS) show a marked increase in the percentage of immature platelet fraction (IPF%) despite the absence of severe thrombocytopenia. To determine the significance of such an unbalanced increase in the IPF%, we investigated the IPF% and other laboratory findings of 51 patients recently diagnosed with MDS. Method:, Subjects consisted of 80 healthy males, 90 healthy females, and 51 patients with MDS and 20 patients with idiopathic thrombocytopenic purpura (ITP). The IPF and IPF% were determined using a Sysmex XE-2100 system loaded with IPF Master software (XE IPF Master, Sysmex). Platelet counts were measured simultaneously. Results:, IPF% and platelet counts of these patients ranged from 1.1% to 25.1% (median, 5.3%) and from 6 to 260 × 109/L (median, 71 × 109/L), respectively. Twelve patients showed platelet counts more than 50 × 109/L with 10% or more IPF%. All of the 12 patients had chromosome abnormalities including monosomy 7 and complex abnormalities involving 7 or 5q. In the other 39 patients who did not show the aberrant IPF% increase, chromosomal abnormalities were seen only in seven patients and none of them had chromosome 7 abnormalities. The IPF% of two patients increased to more than 10% in association with the appearance of monosomy 7. Conclusions:, These findings suggest that a high IPF% in MDS patient may be a marker for karyotypic abnormalities with a poor prognosis, including chromosome 7 abnormalities. [source] Rituximab therapy in adult patients with relapsed or refractory immune thrombocytopenic purpura: long-term follow-up resultsEUROPEAN JOURNAL OF HAEMATOLOGY, Issue 3 2008Marta 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 purpuraEUROPEAN JOURNAL OF HAEMATOLOGY, Issue 2008Terry 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] Interferon-, +874A/T and interleukin-4 intron3 VNTR gene polymorphisms in Chinese patients with idiopathic thrombocytopenic purpuraEUROPEAN JOURNAL OF HAEMATOLOGY, Issue 3 2007Xiaoli Chen Abstract Objectives:, The polarization of Th1/Th2 towards Th1 contributes to the pathogenesis of idiopathic thrombocytopenic purpura (ITP). Cytokines may play crucial roles in the pathogenesis of ITP. The purpose of this study was to investigate whether the interferon (IFN)-, +874(A/T) and interleukin-4 (IL-4) variable number of tandem repeats (VNTR) in intron3 polymorphisms may be responsible in part for genetic susceptibility to ITP. Methods:, Genotyping of IFN-, +874A/T and IL-4 intron3 VNTR was performed in 196 patients with ITP and 128 healthy individuals by polymerase chain reaction sequence-specific primers and direct PCR respectively. Results:, There was no association between IFN-, +874A/T and IL-4 intron3 VNTR polymorphism and ITP risk when all patients, as a group, were analyzed. When the patients were subdivided into two groups: childhood ITP and adult ITP, no statistical differences were found in the genotype and allele frequencies of IFN-, +874A/T and IL-4 intron3 VNTR between the two groups and the controls. Similar results were observed between acute childhood ITP, chronic childhood ITP, acute adult ITP or chronic adult ITP and the controls. Conclusion:, These polymorphisms were distributed similarly between the patients with ITP and the controls, demonstrating that these two candidate gene polymorphisms are not attributed to ITP susceptibility. [source] Acquired thrombotic thrombocytopenic purpura as the presenting symptom of systemic lupus erythematosus.EUROPEAN JOURNAL OF HAEMATOLOGY, Issue 5 2005Successful treatment with plasma exchange, immunosuppression, report of two cases Abstract:, Thrombotic thrombocytopenic purpura (TTP) is a rare but life-threatening syndrome characterized by platelet aggregation causing occlusive microangiopathy. It has been described as a complication in systemic lupus erythematosus (SLE). Recent research indicated that genetic or autoantibody-induced deficiency of the metalloprotease ADAMTS13 plays a key role in the pathogenesis of TTP. Here we report two uncommon cases of TTP as the first presenting symptom of SLE. Both patients were treated with combined plasma exchange and immunosuppressive therapy, and recovered completely. Although TTP and SLE have several clinical findings in common, and both disorders may coexist more frequently than we currently assume, features of one disease should not mislead to reject the alternative disorder. [source] Ursodeoxycholic acid treatment of idiopathic thrombocytopenic purpura with liver dysfunctionEUROPEAN JOURNAL OF HAEMATOLOGY, Issue 5 2003Michiaki Koike Abstract: Ursodeoxycholic acid (UDCA) is known to reduce immunoglobulin from B cells and cytokine production from T cells. We found that UDCA increased the platelet count in two idiopathic thrombocytopenic purpura (ITP) patients who have liver dysfunction. UDCA was tolerated and did not cause diarrhea in the amounts used. Further investigation is needed to evaluate the effectiveness of UDCA in ITP patients. [source] Fulminant thrombotic thrombocytopenic purpura in two patients with systemiclupus erythematosus and phospholipid autoantibodiesEUROPEAN JOURNAL OF HAEMATOLOGY, Issue 6 2000M. Osman Musa No abstract is available for this article. [source] Plasma-soluble Fas (APO-1, CD95) and soluble Fas ligand in immune thrombocytopenic purpuraEUROPEAN JOURNAL OF HAEMATOLOGY, Issue 4 2000Chie 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 macrothrombocytopeniaEUROPEAN JOURNAL OF HAEMATOLOGY, Issue 3 2000F. 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] Concomitant multiple sclerosis and idiopathic thrombocytopenic purpuraEUROPEAN JOURNAL OF NEUROLOGY, Issue 8 2010M. A. Sahraian First page of article [source] Successful treatment of idiopathic thrombocytopenic purpura by Chinese herbal medicine EK-49 and ascorbic acid in an elderly patient developing chronic subdural hematomaGERIATRICS & GERONTOLOGY INTERNATIONAL, Issue 1 2007Atsushi Hirano Treatment of idiopathic thrombocytopenic purpura can present difficult therapeutic choices. An 88-year-old woman was admitted for treatment of a right subdural hematoma. She had a history of chronic thrombocytopenia, with follow-up evaluations by a local physician. Platelet count on admission was 5 × 103/µL, with a high serum concentration of platelet-associated immunoglobulin G. Bone marrow examination showed a marked increase in megakaryocytes. The patient was diagnosed with idiopathic thrombocytopenic purpura accompanied by chronic subdural hematoma. An increase in platelet count and gradual resolution of the subdural hematoma were obtained with prednisolone and azathioprine administration, but platelet count decreased when steroid tapering was attempted. Ultimately we administered the Chinese herbal medicine EK-49 and ascorbic acid, with a gradual increase in platelet count and no adverse effects. Some elderly patients with chronic subdural hematoma can be treated non-invasively. Further, a combination of EK-49 and ascorbic acid may be an effective treatment for refractory idiopathic thrombocytopenic purpura. [source] Proof of an Association between Helicobacter pylori and Idiopathic Thrombocytopenic Purpura in Latin AmericaHELICOBACTER, Issue 3 2007Germán Campuzano-Maya Abstract Background:, Association between Helicobacter pylori and idiopathic thrombocytopenic purpura (ITP) has been found in Japan and in some European countries. It has also been shown that eradication of H. pylori can increase platelet counts in patients with ITP. The aims of this study were to determine the prevalence of H. pylori infection in patients with ITP in Colombia, and the effect of bacterial eradication on their platelet counts. Materials and methods:, Between December 1998 and April 2006, a total of 32 patients diagnosed with ITP were included in the study. Controls were age and sex matched. Results:,H. pylori infection in patients with ITP was significantly higher (p = .00006) than in control individuals (90.6% and 43.8%, respectively), as determined by 13C-urea breath test. A significant association between H. pylori infection and ITP was found (p < .0003), with an odds ratio (OR) of 13.15 (95%CI: 3.24,53.29). Multivariate analysis for the association between H. pylori and ITP showed an OR of 20.44 (95%CI: 3.88,107.49) for women and 19.28 (95%CI: 2.03,183.42) for individuals over 50 years. All 29 H. pylori -positive patients with ITP received eradication treatment. After a median follow up of 12.2 months, 80.8% had a recovery in platelet counts. Conclusions:, According to these results and others from different countries where H. pylori infection rates are high, patients with ITP should be initially tested for H. pylori status, and if present, infection should be eradicated before initiating a drastic conventional ITP treatment. An algorithm for the study and management of patients with ITP in the post- Helicobacter era is presented. [source] Successful use of dapsone in refractory pregnancy-associated idiopathic thrombocytopenic purpuraINTERNAL MEDICINE JOURNAL, Issue 1 2000R. LUSH No abstract is available for this article. [source] Dyspnoea after antiplatelet agents: the AZD6140 controversyINTERNATIONAL JOURNAL OF CLINICAL PRACTICE, Issue 3 2007V. L. Serebruany Summary Recent randomised studies suggest that experimental oral reversible platelet P2Y12 receptor inhibitor, AZD6140, causes dyspnoea. This also raises similar concerns about the parent compound, and another adenosine triphosphate (ATP) analogue (AR-69931MX or cangrelor), which is currently in Phase 3 trial in patients undergoing coronary interventions. We analysed package inserts, and available clinical trials safety data for antiplatelet agents with regard to the incidence of dyspnoea. We found that dyspnoea is a very rare complication of the presently approved platelet inhibitors, mostly caused by underlying disease, rather than antiplatelet therapy per se. The main reasons for respiratory distress after oral (AZD6140), and intravenous (cangrelor) agents may be the development of mild asymptomatic thrombotic thrombocytopenic purpura, fluid retention and dyspnoea because of the reversible nature of these drugs. Also, these agents are ATP analogues, which rapidly metabolise to adenosine, a well-known bronchoprovocator causing dyspnoea as well. In summary, dyspnoea is seldom considered, there are no treatment algorithms when it does occur, plausible mechanisms exist and despite these plausible mechanisms, the true cause of dyspnoea in these exposed individuals is unknown. Additional pulmonary function testing, immunological investigations and platelet receptor studies are urgently needed to determine the cause of dyspnoea after AZD6140, and to point out how such serious adverse reactions can be prevented, or at least minimised, raising potential concerns about this drug. [source] Two generations with familial thrombotic thrombocytopenic purpuraINTERNATIONAL JOURNAL OF CLINICAL PRACTICE, Issue 1 2006R. G. Rodrigues Summary Thrombotic thrombocytopenic purpura (TTP) is a rare multi-system disease characterised by the pentad of microangiopathic haemolytic anaemia, thrombocytopenia, renal dysfunction, fever and neurologic changes. A hereditary form of recurrent familial TTP has been described, which usually presents in adolescence or early adulthood and can lead to recurrent or chronic relapsing TTP. Genetic analyses of patients with familial TTP have linked the disease to chromosome 9q34, and an increased incidence is seen in people with HLA-B40 group antigens. We describe here an 11-year-old Egyptian girl with no significant past medical history who presented with new onset of bruising, petechial rash, fatigue and fevers and was diagnosed with familial TTP. Further testing revealed that both the patient and her father had the HLA-B40 group antigen and also had ADAMTS-13 von Willebrand factor-cleaving protease deficiency as well as factor-H deficiency. [source] Conjugation of methotrexate to immunoglobulins kills macrophages by Fc receptor mediated uptake?INTERNATIONAL JOURNAL OF LABORATORY HEMATOLOGY, Issue 3 2008X. WANG Summary The aim of this study was to conjugate methotrexate (MTX) with intravenous immunoglobulin (IVIG) and investigate whether the conjugate produce selective cytotoxicity on macrophages to provide a new strategy for the management of idiopathic thrombocytopenic purpura. MTX was bound to IVIG via human serum albumin as an intermediary. The binding activity of the Fc fragment of the conjugate was assayed by flow cytometry. The selective cytotoxicity of the conjugate was determined by trypan blue exclusion. After conjugating, the binding activity of the conjugate to Fc receptors did not diminish when compared with IVIG. In vitro, the conjugate showed significantly higher cytotoxicity to macrophages than Hela cells. The conjugate of IVIG and MTX showed potent and selective cytotoxicity to macrophages in vitro. [source] Quantification of red blood cell fragmentation by the automated hematology analyzer XE-2100 in patients with living donor liver transplantationINTERNATIONAL JOURNAL OF LABORATORY HEMATOLOGY, Issue 5 2005S. BANNO Summary The fragmented red cell (FRC) is a useful index for diagnosing and determining the severity of thrombotic thrombocytopenic purpura (TTP), thrombotic microangiopathy (TMA) and other similar conditions, as it is found in peripheral blood in patients with these diseases. The FRC expression rate has conventionally been determined by manual methods using smear samples. However, it is difficult to attain accurate quantification by such methods as they are time consuming and prone to a great margin of error. With cases of living donor liver transplantation, the current study examined the possibility of using a multi-parameter automated hematology analyzer, the XE-2100 (Sysmex Corporation) for FRC quantification. While there was a notable correlation between the manual and automated measurements, the manual measurement resulted in higher values. This suggested remarkable variations in judgment by individuals. The FRC values had a significant correlation with the reticulocyte count, red blood cell distribution width (RDW), fibrin/fibrinogen degradation products (P-FDP) and lactate dehydrogenase (LDH) among the test parameters, and this finding was consistent with the clinical progression in patients. The automated method can offer precise measurements in a short time without inter-observer differences, meeting the requirement for standardization. The determination of FRC count (%) by the XE-2100 that enables early diagnoses and monitoring of TTP or TMA will be useful in the clinical field. [source] Current management of adult idiopathic thrombocytopenic purpura in practice: a cohort study of 201 patients from a single center,INTERNATIONAL JOURNAL OF LABORATORY HEMATOLOGY, Issue 2 2004J. Zimmer Summary To define usefulness and response to therapy and outcome in adults with idiopathic thrombocytopenic purpura (ITP) in clinical practice. We retrospectively reviewed a cohort of 201 consecutive patients with ITP, diagnosed between 1985 and 1994. In particular, we analyzed the therapies used, their response rates, prognostic indicators of response and outcome. In 62 patients, with minor bleeding episodes and a mean (±SD) platelet count of 88 ± 23 × 109/l, no treatment was used and chronic ITP was diagnosed in 59%. A total of 139 patients, with bleeding episodes in 71.2% cases and a mean platelet count of 20 ± 13 × 109/l, received at least one treatment. Three patients died (1.5% of the series). Corticosteroids were used in 118 patients, with an initial response rate of 82.2% and a long-term complete response (CR) of only 22.9%. Intravenous immunoglobulin was used in 26 patients, with an initial transient response in more than 60%. A splenectomy was performed in 55 patients, with an initial response rate of 92.5% and a long-term CR in 60%. Young age and prior response to corticosteroids were significant predictors of a durable response to splenectomy. Danazol was given in 37 patients, with a favorable response in 73% of cases. Our results illustrate the guidelines of the American Society of Hematology. Patients with moderate thrombocytopenia do not require treatment. In severe cases, splenectomy is the only treatment giving durable cures in a significant proportion of patients. Despite frequent chronicity, ITP is life-threatening only in a minor subset of patients. [source] Excellent response of refractory life-threatening thrombotic thrombocytopenic purpura to cyclosporine treatmentINTERNATIONAL JOURNAL OF LABORATORY HEMATOLOGY, Issue 1 2004M. Itälä Summary The introduction of plasma exchange has significantly improved the outcome of thrombotic thrombocytopenic purpura (TTP), and survival has increased from 10 to 80,90%. TTP refractory to plasma exchange therapy, however, is still a therapeutic challenge. We describe here a patient who partially responded to plasma exchange therapy, but remained totally dependent on plasma infusions. Several attempts to discontinue plasma therapy repeatedly lead to relapses. TTP did not response to vincristine, either. After 3 months treatment with plasma therapy, cyclosporine was started. Plasma therapy could be discontinued after 3 weeks on cyclosporine, and serum LDH and blood platelet count were gradually normalized during 2 months. Cyclosporine was tapered off after 6 months treatment, and the patient has stayed in remission ever since. We conclude that cyclosporine is a worthwhile treatment option in patients with refractory TTP. [source] Severe epistaxis in brucellosis-induced isolated thrombocytopenia: A report of two casesINTERNATIONAL JOURNAL OF LABORATORY HEMATOLOGY, Issue 6 2000A. Sevinc Brucellosis can present initially with its haematological findings including anaemia, leukopenia, and thrombocytopenia and may mimic primary haematological diseases. We present two patients with complaints of severe epistaxis and isolated thrombocytopenia which was initially diagnosed as idiopathic thrombocytopenic purpura but which was finally attributed to brucellosis. Their platelet count reverted to normal within 2,3 weeks of initiating antibrucellosis treatment with recovery from the disease. [source] Thrombotic thrombocytopenic purpura: Results of the patients with thrombotic microangiopathies across Japan by ADAMTS13 analysis during 1998,2008ISBT SCIENCE SERIES: THE INTERNATIONAL JOURNAL OF INTRACELLULAR TRANSPORT, Issue n2 2009Y. Fujimura Background, Thrombotic microangiopathies (TMAs) are pathological conditions, characterized by generalized microvascular occlusion by platelet thrombi, thrombocytopenia, and microangiopathic hemolytic anemia. Two typical phenotypes of TMAs are hemolytic-uremic syndrome (HUS) and thrombotic thrombocytopenic purpura (TTP). Severe deficiency of plasma ADAMTS13 activity (ADAMTS13:AC) is more specific for TTP but not for HUS. Materials & Methods, Since 1998, our laboratory has functioned as a nationwide referral center for TMAs by analyzing ADAMTS13. Of 1564 tested patients from 426 hospitals, 919 were positive for TMAs. Levels of ADAMTS13:AC and the ADAMTS13-neutralizing autoantibodies (ADAMTS13:INH) in these patients were determined by chromogenic act-ELISA and/or by classic von Willebrand factor multimer assay. Results, TMA patients consisted of two groups, those with severe (less than 3% of normal control) and those with non-severe deficiency of ADAMTS13:AC. Additionally, both groups were divided into congenital (n = 65) and acquired (n = 854) TMAs. Of the congenital TMA patients, 41 had ADAMTS13:AC deficiency due to gene mutations, while the remaining 24 had the disease of unknown etiology. The 854 patients with acquired TMAs could be largely grouped into three categories: idiopathic TTP (n = 284), idiopathic HUS (n = 106), and secondary TMAs (n = 464). The secondary TMAs were observed in heterogeneous patient groups and were associated with drugs, connective tissue diseases, malignancies, transplantation, pregnancy, E. coli O157:H7 infection, and other factors. All of the patients with acquired severe ADAMTS13:AC deficiency were positive for ADAMTS13:INH. Conclusion, Although TMAs are highly heterogeneous pathological conditions, one third of TMA patients have severe deficiency of ADAMTS13:AC. Platelet transfusions to such patients are contraindicated. Thus, rapid ADAMTS13:AC assays will be prerequisite in medical facilities where TMA patients are treated. [source] Infection frequently triggers thrombotic microangiopathy in patients with preexisting risk factors: A single-institution experienceJOURNAL OF CLINICAL APHERESIS, Issue 2 2010Kenneth W. Douglas Abstract Thrombotic microangiopathies are rare conditions characterized by microangiopathic hemolytic anemia, microthrombi, and multiorgan insult. The disorders, which include hemolytic uremic syndrome and thrombotic thrombocytopenic purpura, are often acute and life threatening. We report a retrospective analysis of 65 patients presenting to our institution from 1997 to 2008 with all forms of thrombotic microangiopathy. Therapeutic plasma exchange was a requirement for analysis and 65 patients were referred to our institution; 66% of patients were female and median age at presentation was 52 years. Bacterial infection was the most commonly identified etiologic factor and in the multivariate model was the only significant variable associated with survival outcome (odds ratio 5.1, 95% confidence interval, 1.2,21.7). As infection can be considered a common trigger event for thrombotic microangiopathy, patients with hepatobiliary sepsis may benefit from elective cholecystectomy. We conclude that bacterial infection frequently triggers TTP and other thrombotic microangiopathies in patients with preexisting risk factors and propose a model for the development of these syndromes. © J. Clin. Apheresis 2010. © 2010 Wiley-Liss, Inc. [source] Predictors of complications in therapeutic plasma exchangeJOURNAL OF CLINICAL APHERESIS, Issue 6 2009Carsten P. Bramlage Abstract Plasma exchange (PE) is used for blood purification to modulate proteins involved in pathological processes. As the number of patients receiving PE treatment and the heterogeneity of the underlying diseases is steadily increasing, we evaluated the most frequent complications and analyzed causes leading to adverse reactions. 883 PE procedures in 113 patients between the years 2000 to 2006 were retrospectively analyzed with respect to complications. Additionally, underlying diseases and settings of PE procedure were analyzed to identify high-risk patients and respective PE settings. A total of 226 adverse reactions were recorded (25.6% of all PE procedures). Most complications were mild (n = 121, 13.7%) or moderate (n = 98, 11.0%). In seven cases (n = 7, 0.7%), severe, life-threatening adverse events were induced by PE either due to severe allergic reactions (n = 4, 0.5%) or to sepsis (n = 3, 0.3%). Patients with neurologic diseases had a significantly higher risk to develop complications compared to those with internal diseases (P = 0.013). This was due to a higher rate of PE associated adverse events (in particular hypotension) and complications associated with vascular access. Among patients from internal medicine those with hemolytic uremic syndrome (HUS) and thrombotic thrombocytopenic purpura (TTP) had the highest risk to develop complications. Patients with neurological diseases compared to those with medical conditions and patients with HUS/TTP compared to those with other diseases had a higher risk to develop complications. However, severe adverse events are rare. Thus, PE seems to be a safe and recommendable procedure. J. Clin. Apheresis, 2009. © 2009 Wiley-Liss, Inc. [source] Effect of blood group on idiopathic thrombotic thrombocytopenic purpuraJOURNAL OF CLINICAL APHERESIS, Issue 4 2009Lara Zuberi Abstract Thrombotic thrombocytopenic purpura (TTP) is a condition caused by deficiency of ADAMTS13 resulting in accumulation of ultra large Von Willebrand factor multimers (ULVWF), leading to micro thrombi in multiple organs. The varying susceptibilities of blood group antigens to ADAMTS13 have been demonstrated. A and B antigens are protective of VWF; and VWF purified from blood group O individuals has been shown to be cleaved faster by ADAMTS13 compared to VWF from blood group AB individuals. We proposed that there may be a difference in the incidence of blood groups in TTP patients compared with the general population. We felt this to be important for a life-threatening disease with poorly understood epidemiology. We report a retrospective analysis of 74 patients presenting from 1993 to 2008 with idiopathic TTP. We studied the incidence across various blood groups and also estimated the recurrence and mortality in each group. The incidence of various blood groups were as follows: O 36%, A 36%, B 25%, and AB 2%, compared with expected frequencies in the Detroit area: O 44%, A 33% B 20%, and AB 3%. There was a trend of lower than expected frequency of blood group O. There were 24 recurrences and 14 deaths, uniform across blood groups. We hypothesized that there may be an association between blood groups and the risk of TTP; however the differences in our study were not statistically significant. Recurrence and disease specific mortality did not appear to be impacted by blood group. J. Clin. Apheresis 2009. © 2009 Wiley-Liss, Inc. [source] | |||||||||||||||||||||||||||||||