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Idiopathic Thrombocytopenic Purpura (idiopathic + thrombocytopenic_purpura)
Kinds of Idiopathic 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] Recurrent idiopathic thrombocytopenic purpura in childhoodPEDIATRIC BLOOD & CANCER, Issue 2 2008Maria Vranou MD Abstract Background Idiopathic thrombocytopenic purpura (ITP) is a common haematological disease during childhood, that usually has a benign course; however, literature on the recurrent form of the disease (rITP) is limited. Procedure rITP was characterized by intermittent episodes of thrombocytopenia (TP) followed by periods of recovery, unrelated to therapeutic intervention. We retrospectively reviewed features of patients with rITP, diagnosed and systematically followed up at our center, during the period 1975,2004. Results Forty-eight of 795 children with ITP (6.0 %) presented with rITP. The majority of patients (68.8%) had only one recurrence, whereas only one patient had four. A time interval between two episodes longer than 3 months (up to 96) was identified in 2/3 of episodes and <3 months in 1/3. The initial episode and the first recurrence mostly shared features of acute ITP; however, 22.9% of the episodes appeared with a chronic self-limited course. Bleeding manifestations were rare (18.6% of episodes) and mild, and they tended to occur in severely thrombocytopenic patients, mainly at the onset of the initial episode; intracranial hemorrhage (ICH) occurred in a toddler with short duration thrombocytopenia. Intravenous , globulin (IVIG) or corticosteroids were administered in 24.5% of episodes. None of the patients needed splenectomy. Conclusion: rITP is a rare, mild, self-limited type of ITP, although ICH may occur in a profoundly TP child. Recurrence may occur close or far apart to a previous isolated TP episode. The duration of episodes varies considerably from patient to patient and from episode to episode in the same patient. The pathogenesis of rITP still remains unclear. Pediatr Blood Cancer 2008;51:261,264. © 2008 Wiley-Liss, Inc. [source] Idiopathic thrombocytopenic purpura in childhood: Controversies and solutionsPEDIATRIC BLOOD & CANCER, Issue S5 2006Thomas Kühne MD Abstract Idiopathic thrombocytopenic purpura (ITP) is a bleeding disorder in patients who are otherwise healthy and present with thrombocytopenia with normal red cells and leukocytes. ITP is a diagnosis of exclusion and is in origin heterogeneous. The unknown etiology and the lack of clinical data from controlled prospective studies are reasons for controversies in diagnosis and management. Study endpoints traditionally include the velocity of platelet increase after drug intervention or observation, although a rapid elevation of the platelet count is of questionable clinical value. Evaluation of other endpoints is needed. Pediatr Blood Cancer 2006;47:650,652. © 2006 Wiley-Liss, Inc. [source] High P-glycoprotein-mediated export observed in patients with a history of idiopathic thrombocytopenic purpuraBRITISH JOURNAL OF HAEMATOLOGY, Issue 3 2002Adam S. Levy Summary., Studies have suggested that high P-glycoprotein expression in lymphocytes from patients with autoimmune disorders may affect disease outcome. Idiopathic thrombocytopenic purpura (ITP) and Evans' syndrome are widely thought to be autoimmune processes, however, the precise mechanisms remain unknown. Peripheral blood mononuclear cells from patients with refractory or recurrent ITP or Evans' syndrome were studied using the rhodamine 123 flow cytometric assay to investigate functional export levels. Lymphocytes from ITP and Evans' syndrome patients showed a significantly decreased ability to retain rhodamine, suggesting increased export protein function. Reverse transcription polymerase chain reaction distinguished P-glycoprotein as the likely export protein. [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] 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] 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] 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] 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] 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] 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] 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] The long-term efficacy of Helicobacter pylori eradication therapy in patients with idiopathic thrombocytopenic purpuraJOURNAL OF GASTROENTEROLOGY AND HEPATOLOGY, Issue 12 2007Masaaki Satake Abstract Background and Aim:, A beneficial effect of Helicobacter pylori (H. pylori) eradication in patients with H. pylori -positive idiopathic thrombocytopenic purpura (ITP) has been reported by several investigators; however, it was not clear whether the recovered platelet count after H. pylori eradication was maintained for a long period. Method:, Thirty-eight ITP patients who were examined for H. pylori infection were assessed. H. pylori -positive patients received a standard antibiotic therapy for H. pylori eradication. We investigated the long-term effect of H. pylori eradication on platelet recovery in patients with H. pylori -positive ITP. Results:, Of the 38 ITP patients, 26 (68.4%) were positive for H. pylori. The response rate of platelet recovery was 56.5% (13/23 patients). Twelve patients showed complete response (CR) and one showed partial response (PR). The mean platelet counts 6 months after eradication significantly increased from 31 × 109/L to 129 × 109/L in 23 H. pylori -eradicated patients (P < 0.001). The median platelet counts of responders 1, 2, 3, and 4 years after eradication were 168 × 109/L (n = 10), 193 × 109/L (n = 9), 168 × 109/L (n = 7), and 243 × 109/L (n = 4) after a mean follow-up of 25.8 months. Conclusion:, Eradication therapy for H. pylori -positive patients with ITP was effective and a favorable effect was maintained for long periods. [source] Evaluation of platelet kinetics in patients with liver cirrhosis: Similarity to idiopathic thrombocytopenic purpuraJOURNAL OF GASTROENTEROLOGY AND HEPATOLOGY, Issue 1 2007Mikio Kajihara Abstract Background:, Thrombocytopenia is a common manifestation of liver cirrhosis (LC), but its underlying mechanism is not fully understood. The purpose of the present paper was to evaluate the platelet kinetics in LC patients by examining several non-invasive convenient markers. Methods:, Fifty-seven LC patients, 32 patients with idiopathic thrombocytopenic purpura (ITP), 12 with aplastic anemia (AA), and 29 healthy individuals were studied. Plasma thrombopoietin was measured by enzyme-linked immunosorbent assay. Absolute reticulated platelet (RP) count and plasma glycocalicin were used as indices for thrombopoiesis, and the indices for platelet turnover were the RP proportion and the plasma glycocalicin normalized to the individual platelet count (GCI). Results:, There was no difference in thrombopoietin levels between LC patients and healthy controls. The RP proportion and GCI were significantly higher and the absolute RP count and glycocalicin significantly lower in LC patients than in healthy controls. These markers in ITP and LC patients were comparable, but significantly different from those in AA patients. The bone marrow megakaryocyte density in LC and ITP patients was similar, and significantly higher than in AA patients. Conclusions:, Cirrhotic thrombocytopenia is a multifactorial condition involving accelerated platelet turnover and moderately impaired thrombopoiesis. Thrombopoietin deficiency is unlikely to be the primary contributor to cirrhotic thrombocytopenia. [source] Intended management of children with acute idiopathic thrombocytopenic purpura: A national surveyJOURNAL OF PAEDIATRICS AND CHILD HEALTH, Issue 1-2 2005MK Marks Objective: In Australia acute idiopathic thrombocytopenic purpura (ITP) is mainly treated by paediatricians (either general paediatricians or paediatric haematologists/oncologists). A survey was conducted to gauge the current practice of treating children with acute ITP in Australia. Methods: All practising Australian paediatricians registered by the Royal Australasian College of Physicians were surveyed regarding their intended management of children with acute ITP. The questionnaire, adapted from a study of paediatric haematologists/oncologists in North America, presented four clinical scenarios of children with acute ITP with a platelet count of 3000 × 109/L, with and without mucosal bleeding (wet and dry purpura, respectively). Questionnaires were returned by mail or filled in online at a dedicated webpage. Results: Five hundred and sixty-three of 1097 (51%) paediatricians responded to the survey. Data from 140 who had treated at least one child with ITP in the previous 12 months were analysed. Respondents indicated that children with acute ITP are usually or always hospitalised (58,92%) and that 48% would be given active treatment, even with dry purpura. Various regimens of i.v. immunoglobulin or corticosteroids are used when treatment is administered. In comparing Australian and North American management of acute ITP there were many similarities, although Australian paediatricians were less likely to arrange a bone marrow aspirate if corticosteroids were prescribed. Conclusions: There is great variation in the intended management of children with acute ITP in Australia. Previously published management recommendations regarding investigation and treatment have had little impact on intended practice. Prospective studies are required to evaluate hypotheses so as to produce evidence-based recommendations for treatment of patients with acute ITP. [source] Modulation of immune response with cytotoxic T-lymphocyte-associated antigen 4 immunoglobulin-induced anergic T cells in chronic idiopathic thrombocytopenic purpura,JOURNAL OF THROMBOSIS AND HAEMOSTASIS, Issue 1 2008X.-L. ZHANG Summary.,Background:,Platelet glycoprotein (GP)-reactive CD4+ T cells are essential for the stimulation and maintenance of antiplatelet autoantibody production in chronic idiopathic thrombocytopenic purpura (ITP). Blocking costimulatory signals could result in platelet-specific T-cell anergy. Methods:,GP-specific CD4+ T cells from patients with ITP were made anergic using cytotoxic T-lymphocyte-associated antigen 4 immunoglobulin (CTLA4-Ig). The CTLA4-Ig-induced GP-specific anergic T cells were investigated for their inhibitory function on GP-reactive T-cell proliferation and antibody production with in vitro culture systems. To further analyze their tolerizing mechanisms, we cocultured GP-anergic T cells with dendritic cells (DCs) from patients with ITP. Results:,Our studies demonstrated that the anergized GP-specific T cells have profound effects on both GP-specific T-cell proliferation and antibody production. These anergic T cells exerted their suppressive effects mainly in a cell contact-dependent manner, and they were not constitutively suppressive but required specific antigen stimulation to make DCs tolerogenic. The anergic T-cell-modulated DCs could induce the autoreactive T cells to be tolerant, and this effect was not restricted to T cells of the same specificity. Conclusion:,Our studies demonstrate the efficacy of CTLA4-Ig in suppressing the pathologic autoimmune responses in ITP. These findings provide new insights into the underlying mechanisms of anergy induction in chronic ITP. [source] Novel ADAMTS-13 mutations in an adult with delayed onset thrombotic thrombocytopenic purpuraJOURNAL OF THROMBOSIS AND HAEMOSTASIS, Issue 9 2006Z. TAO Summary.,Background:,Thrombotic thrombocytopenic purpura (TTP) is associated with congenital and acquired deficiency of ADAMTS-13, a metalloprotease that cleaves von Willebrand factor (VWF) and reduces its adhesive activity. Mutations throughout the ADAMTS13 gene have been identified in congenital TTP patients, most of whom have initial episodes during infancy or in early childhood. Patients and methods:,We report the case of an adult male who was diagnosed with idiopathic thrombocytopenic purpura at age 34, and with TTP 14 years later. The patient was compound heterozygous for an 18 bp in-frame deletion (C365del) in the disintegrin domain and a point mutation of R1060W in the seventh thrombospondin domain of the ADAMTS-13 gene. Conclusions:In vitro studies found that C365del and R1060W severely impair ADAMTS-13 synthesis in transfected Hela cells, whereas the deletion mutant also failed to cleave VWF under static and flow conditions. [source] Short-term and long-term failure of laparoscopic splenectomy in adult immune thrombocytopenic purpura patients: A systematic review,AMERICAN JOURNAL OF HEMATOLOGY, Issue 11 2009Joseph Mikhael Splenectomy is a common therapy for adults with chronic idiopathic thrombocytopenic purpura (ITP). Thisstudy was designed to estimate both the short-term surgical non-response rate and the long-term relapse rate after laparoscopic splenectomy. A systematic review was conducted of articles published between January 1, 1991 and January 1, 2008. Selection criteria included: chronic ITP, study enrollment in 1990 or later, ,12 months of follow-up, ,15 patients with ITP, ,75% of patients at least 14 years of age, not HIV positive, not undergoing a second splenectomy, and type of performed splenectomy clearly reported. Data were pooled across studies to estimate rates. We identified 170 articles, of which 23 met our inclusion criteria (all observational studies). These studies represent 1,223 laparoscopic splenectomies (71 or 5.6% were converted to open splenectomy during surgery). The pooled short-term surgical non-response rate among the 18 studies reporting data was 8.2% (95% CI 5.4,11.0). The pooled long-term relapse rate across all 23 studies was 43.6 per 1,000 patient years (95% CI 28.2,67.2). This translates to an approximate failure rate of 28% at 5 years for all patients undergoing splenectomy. Studies with shorter durations of follow-up had significantly higher pooled relapse rates than studies with longer follow-up (P = 0.04). Laparoscopicsplenectomy is effective for most patients. Splenectomy may have higher initial relapse rates, particularly, in the first 2 years after surgery, and the rate may decline over time. Am. J. Hematol. 2009. © 2009 Wiley-Liss, Inc. [source] Recurrent idiopathic thrombocytopenic purpura in childhoodPEDIATRIC BLOOD & CANCER, Issue 2 2008Maria Vranou MD Abstract Background Idiopathic thrombocytopenic purpura (ITP) is a common haematological disease during childhood, that usually has a benign course; however, literature on the recurrent form of the disease (rITP) is limited. Procedure rITP was characterized by intermittent episodes of thrombocytopenia (TP) followed by periods of recovery, unrelated to therapeutic intervention. We retrospectively reviewed features of patients with rITP, diagnosed and systematically followed up at our center, during the period 1975,2004. Results Forty-eight of 795 children with ITP (6.0 %) presented with rITP. The majority of patients (68.8%) had only one recurrence, whereas only one patient had four. A time interval between two episodes longer than 3 months (up to 96) was identified in 2/3 of episodes and <3 months in 1/3. The initial episode and the first recurrence mostly shared features of acute ITP; however, 22.9% of the episodes appeared with a chronic self-limited course. Bleeding manifestations were rare (18.6% of episodes) and mild, and they tended to occur in severely thrombocytopenic patients, mainly at the onset of the initial episode; intracranial hemorrhage (ICH) occurred in a toddler with short duration thrombocytopenia. Intravenous , globulin (IVIG) or corticosteroids were administered in 24.5% of episodes. None of the patients needed splenectomy. Conclusion: rITP is a rare, mild, self-limited type of ITP, although ICH may occur in a profoundly TP child. Recurrence may occur close or far apart to a previous isolated TP episode. The duration of episodes varies considerably from patient to patient and from episode to episode in the same patient. The pathogenesis of rITP still remains unclear. Pediatr Blood Cancer 2008;51:261,264. © 2008 Wiley-Liss, Inc. [source] Development and research in idiopathic thrombocytopenic purpura: An inflammatory and autoimmune disorderPEDIATRIC BLOOD & CANCER, Issue S5 2006Paul Imbach MD Abstract Bleeding and isolated thrombocytopenia in idiopathic thrombocytopenic purpura (ITP) are phenomena of a transient or long-term disturbed immune response. Since 1980, ITP has become a model for targeted therapeutic immunomodulation with still unclear mechanisms of action. The systematic analysis of ITP aims to determine more clearly defined patient subgroups for targeted therapy and to explore the complex immune disturbances in this autoimmune disorder. Pediatr Blood Cancer 2006;47:685,686. © 2006 Wiley-Liss, Inc. [source] Chronic myeloid leukemia in a patient with chronic idiopathic thrombocytopenic purpura: Rapid response to imatinib mesylate (STI571)PEDIATRIC BLOOD & CANCER, Issue 2 2003Shinsaku Imashuku MD No abstract is available for this article. [source] Clinical spectrum in hospitalized children with echovirus type 13 infectionPEDIATRICS INTERNATIONAL, Issue 2 2005Ken-Ichiro Kobayashi AbstractBackground:,The aim of this study was to investigate the clinical spectrum of echovirus type 13 (E13) infection in hospitalized children. Methods:,From April to August 2002, prospective viral surveillance was performed for hospitalized patients (aged 10 days to 14 years) irrespective of their presenting symptoms and severity. Medical records of laboratory-confirmed echovirus 13 infection were reviewed. Results:,Of the 41 patients analyzed, the median age was 3.4 years and 30% of them were less than 1 year of age. The male:female ratio was 1.6:1. The main clinical features were non-specific febrile illness (nine patients), gastroenteritis (seven), bronchitis (seven), aseptic meningitis (16) and idiopathic thrombocytopenic purpura (two). Each age group had their representative symptoms: less than 1 year of age, non-specific febrile illness; from 1 to 6 years of age, enterocolitis and bronchitis; more than 6 years of age, aseptic meningitis. Conclusion:,The representative symptoms of E13 infection in hospitalized patients were variable but strongly associated with age distribution. It was of interest to note that two patients developed diopathic thrombocytopenic purpura along with the infection. [source] Interferon-alpha therapy in idiopathic thrombocytopenic purpuraPEDIATRICS INTERNATIONAL, Issue 6 2001Bunyamin Dikici AbstractBackground: Acute idiopathic thrombocytopenic purpura (ITP) represents the most frequent hemorrhagic diathesis in childhood. Up to 30% of patients with ITP are regarded as refractory to standard therapy. The rare mortality from acute ITP in childhood is almost exclusively due to intracranial hemorrhage. This complication occurs in less than 1% of ITP patients. This study was designed to evaluate the effect of ,-interferon (IFN-,) in eight patients whom did not respond to conventional therapy. Method: In spite of conventional therapies, the patient whose platelet count could not be increased to 50`109/L were accepted as refractory ITP. Eight of these patients whose platelet count lower than 20`109/L were included in the prospective cohort study. Interferon alpha 2b 5 MU/m2 was administered subcutaneously three times a week, totalling 12 times in a month. According to the platelet count on the 28th day of therapy, we grouped the patients into three categories. After 60 days, the survey was re-evaluated according to the platelet count. Results: The mean age of children was 3.5±2.5 (ranged between 3.5 and 9) years. Six of them were boys and two were girls. There was no response in one patient, partial response in one, and good response in six patients on the 28th day of therapy. The maximum rise in platelet count was observed from 7 to 14 days after the initiation of interferon. The median platelet count which was 15±5`109/L before therapy, raised to 60±12`109/L after therapy. However, on the 60th day of therapy, there were only two patients who had a platelet count over 100`109/L. Conclusion: In our study, we did not observe the long-term benefit of IFN-, therapy in refractor ITP in childhood. However, in good responding patients, platelet levels were increased in a short time. Alpha-interferon may be alternative therapy for patients whom had a platelet count below 20`109/L and not responding to standard therapy, or for patients whom immunosuppressive therapy is contraindicated. [source] Refractory idiopathic thrombocytopenic purpura treated with the soluble tumor necrosis factor receptor etanerceptAMERICAN JOURNAL OF HEMATOLOGY, Issue 4 2008Gregory Litton MD No abstract is available for this article. [source] Successful control of severe postoperative bleeding with recombinant factor VIIa in a case of refractory idiopathic thrombocytopenic purpuraAMERICAN JOURNAL OF HEMATOLOGY, Issue 3 2007Carlos Aguilar No abstract is available for this article. [source] Analysis of outcome of laparoscopic splenectomy for idiopathic thrombocytopenic purpura by platelet countAMERICAN JOURNAL OF HEMATOLOGY, Issue 2 2005A. Keidar Abstract Laparoscopic splenectomy (LS) is now performed routinely in patients with idiopathic thrombocytopenic purpura (ITP) refractory to the medical treatment. Low preoperative platelet count was deemed to be a contraindication for a laparoscopic approach; however, there is no data reporting the outcome in those patients. We aimed to evaluate the influence of the preoperative platelet count on the operative and postoperative course and complication rate. Retrospective cohort study that was conducted in tertiary care university-affiliated medical center and included 110 consecutive patients who underwent LS. All patients were divided into three groups by their preoperative platelet counts: ,20 × 109/L (n = 12), (20,50) × 109/L (n = 18), and >50 × 109/L (n = 80). The outcome and the influence of preoperative factors predictive of complications, blood transfusion, and length of stay were compared between the groups. Patients with a platelet count of ,20 × 109/L had a much longer hospital stay, received more blood transfusions, and suffered more complications than patients with platelet counts of (20,50) × 109/L or higher (P < 0.05). Transfused patients had a longer hospital stay than non-transfused patients (2.08 vs. 6.4 days, P = 0.029). The strongest predictor for transfusion was the platelet count (odds ratio = 23, P = 0.008). LS in patients with very low platelet counts is feasible and reasonably safe, but the platelet count is a major determinant of morbidity. Every effort should be made to elevate platelet levels to >20 × 109/L before surgery. Patients with counts >20 × 109/L can safely undergo LS. Am. J. Hematol. 80:95,100, 2005. © 2005 Wiley-Liss, Inc. [source] | |||||||||||||||||||||||||||||||