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Thrombotic Thrombocytopenic Purpura (thrombotic + thrombocytopenic_purpura)
Kinds of Thrombotic Thrombocytopenic Purpura Selected AbstractsExtensive 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] 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] 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] 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] 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] Value of ADAMTS13 activity and inhibitor in the postmortem diagnosis of thrombotic thrombocytopenic purpuraJOURNAL OF CLINICAL APHERESIS, Issue 3 2009Denis M. Dwyre Abstract Background and Objectives: Thrombotic thrombocytopenic purpura (TTP) is a clinical diagnosis that can be difficult to establish in severely ill patients. We report a case of fulminant TTP in a woman who died before receiving plasma exchange. An autopsy plasma sample was analyzed for ADAMTS13 activity and inhibitor for correlation with the diagnosis of TTP. Recognizing that hemolysis in postmortem blood samples could interfere with ADAMTS13 activity, plasma samples from four additional decedents not suspected of having TTP were analyzed and correlated with their autopsy results. The purpose of this study was to assess whether testing postmortem samples for ADAMTS13 is useful in the postmortem diagnosis of TTP. Material and Methods: Plasma samples from the index case and four non-TTP decedents were analyzed for ADAMTS13 activity, ADAMTS13 inhibitor levels, and plasma free hemoglobin (PFH). Autopsy tissues were evaluated for evidence of platelet microthrombi in all five cases. Results: The ADAMTS13 activity level in the index patient was <4%, and the inhibitor level was 1.0 inhibitor unit. Microthrombi were prominent in the heart, kidneys, pancreas, and adrenal glands, consistent with the clinical diagnosis of TTP. ADAMTS13 activity levels in the four non-TTP decedents ranged from 4 to 82% (3/4 , 26%), and inhibitor was present in two of the four samples. Postmortem PFH levels in the four non-TTP decedents ranged from 64 to 3,917 mg/dL. No microthrombi were observed. Conclusion: Low postmortem ADAMTS13 activity and evidence of inhibitor can occur in decedents without clinical or histologic evidence of TTP. Postmortem ADAMTS13 activity levels may not be valid in establishing a diagnosis of TTP, and high inhibitor levels in this setting may be related to elevated PFH. Caution must be used in the interpretation of ADAMTS13 testing in the presence of hemolysis. J. Clin. Apheresis 2009. © 2009 Wiley-Liss, Inc. [source] Atypical presentations of thrombotic thrombocytopenic purpura: A review,JOURNAL OF CLINICAL APHERESIS, Issue 1 2009Ravi Sarode Abstract Thrombotic thrombocytopenic purpura (TTP) is diagnosed by the presence of microangiopathic hemolytic anemia and thrombocytopenia in a patient who frequently presents with central nervous system involvement and, to a lesser extent, renal dysfunction. Recent understanding of the pathophysiology of TTP due to severe deficiency of von Willebrand factor cleaving protease, known as ADAMTS13, has improved diagnosis of TTP. Once the diagnosis is suspected, life-saving therapeutic plasma exchange therapy is initiated. Occasionally, an unusual clinical presentation makes TTP diagnosis difficult, thus resulting in a delay in the management of TTP. This review highlights a variety of atypical TTP presentations described in the literature. It is intended to bring unusual scenarios to the clinician's awareness, so that timely treatment can be delivered. J. Clin. Apheresis, 2009. © 2008 Wiley-Liss, Inc. [source] Thrombotic thrombocytopenic purpura: 24 years of experience at the American University of Beirut Medical CenterJOURNAL OF CLINICAL APHERESIS, Issue 3 2004Ali Shamseddine Abstract Thrombotic thrombocytopenic purpura (TTP) is a hematological syndrome defined by the presence of thrombocytopenia and microangiopathic hemolytic anemia without a clinically apparent etiology. Patients may also suffer from fever in addition to neurological and renal impairment. Treatment should be initiated as soon as possible, otherwise this rare disease can be fatal. The main treatment options include therapeutic plasma exchange, fresh frozen plasma infusion, and adjuvant agents such as steroids and antiplatelet drugs. A search of patient records was carried out at the American University of Beirut Medical Center looking for patients who developed TTP over a 24-year period extending from 1980 to 2003. Relevant information was collected and analyzed. A total of 47 records were found. All presented with anemia and thrombocytopenia, 83% had neurological symptoms, 61.7% had fever and 34% had renal impairment. All patients were treated with a multimodality regimen including therapeutic plasma exchange, FFP infusion, steroids, antiplatelet agents, vincristine and others. 38 (81%) cases achieved complete remission. Out of these, 12 (31.6%) relapsed and responded to treatment. Patients who did not receive plasma exchange were more likely to relapse (P = 0.032). A second relapse was observed in 6 cases. The overall mortality rate from TTP over 24 years was 21.3%. TTP remains a fatal disease. A high index of suspicion should, therefore, always be present. Treatment options should be further developed and patients should directly be referred to tertiary care centers. J. Clin. Apheresis 19:119,124, 2004. © 2004 Wiley-Liss, Inc. [source] Role of splenectomy in patients with refractory or relapsed thrombotic thrombocytopenic purpuraJOURNAL OF CLINICAL APHERESIS, Issue 2 2003Nicole A. Aqui Abstract Thrombotic thrombocytopenic purpura (TTP) was once uniformly fatal. Therapeutic plasma exchange in combination with immunosuppressive and anti-platelet agents, however, have resulted in improved survival rates of greater than 80% for patients with TTP. In spite of aggressive plasma exchange and adjuvant therapy, a number of TTP patients are refractory to treatment. In addition, up to 40% of TTP patients who initially respond to therapy eventually relapse. Alternative therapies such as splenectomy have been used with varying degrees of success in refractory and relapsing TTP patients. The usefulness of splenectomy in preventing relapse of TTP or treating those patients who are refractory to plasma exchange remains controversial. We present a single institution's experience with 14 patients who underwent splenectomy for refractory (six patients) or relapsed (eight patients) TTP since 1984. In both patient groups, splenectomy induced stable long-term remissions. Six of six (100%) patients who were refractory to plasma exchange, survived to be discharged from the hospital, apparently free of disease. Four of eight patients (50%) who had a splenectomy for relapsing TTP went into a complete remission and had no further relapses of their disease. Moreover, in relapsing patients who failed to experience long-term remission, the relapse rate after splenectomy was 0.3 events per patient year compared to 1.0 events per patient year prior to splenectomy. We conclude that splenectomy is a reasonable treatment option for TTP patients refractory to standard plasma exchange therapy or who have experienced multiple and/or complicated relapses. We believe this is the first series that demonstrates efficacy of splenectomy in plasma exchange-refractory TTP. J. Clin. Apheresis 18:51,54, 2003. © 2003 Wiley-Liss, Inc. [source] Questionable efficacy of plasma exchange for thrombotic thrombocytopenic purpura after bone marrow transplantation,JOURNAL OF CLINICAL APHERESIS, Issue 4 2001J. Teruya Abstract Thrombotic thrombocytopenic purpura (TTP) after bone marrow transplantation (BMT) is an uncommon complication presumably associated with extensive endothelial cell damage due to Cyclosporine, total body irradiation, or other drugs. While the majority of patients with primary TTP, which is considered to be an autoimmune process, respond to plasma exchange, TTP after BMT has a very poor prognosis. A total of 7 patients out of 307 patients who underwent BMT were diagnosed with TTP during 1989,1999. The diagnosis of TTP was made based on thrombocytopenia and microhemangiopathic hemolytic anemia characterized by an elevated LDH and the presence of schistocytes on the peripheral blood smear. Five patients were treated with plasma exchange (PE) using fresh frozen plasma and/or cryoprecipitate poor plasma as replacement fluid. One patient was treated using a protein A column. One patient did not receive plasma exchange because the 125 patient was clinically stable and was discharged. It was hard to assess the efficacy of PE due to the multiplicity of the patients' clinical condition and laboratory data. At least 4 patients did not respond to PE and 2 patients were not able to be evaluated due to multi organ failure. However, all patients died. It is not clear at this moment if PE for patients with TTP after BMT is truly beneficial. J. Clin. Apheresis 16:169,174, 2001. © 2001 Wiley-Liss, Inc. [source] Patients with thrombotic thrombocytopenic purpura commonly develop metabolic alkalosis during therapeutic plasma exchangeJOURNAL OF CLINICAL APHERESIS, Issue 3 2001Marisa B. Marques Abstract Thrombotic thrombocytopenic purpura (TTP) and myasthenia gravis (MG) are category I indications for therapeutic plasma exchange (TPE). This study was based on the hypothesis that the development of metabolic alkalosis during TPE is more common in TTP than in MG, based on our previous observations. In order to test it, we compared the levels of bicarbonate and potassium in both groups of patients undergoing plasmapheresis. Fifteen patients with TTP (190 procedures) and ten MG patients seen concurrently were studied. While baseline bicarbonate levels were similar among all patients, the post-procedure bicarbonate levels in TTP patients were mostly elevated with a mean ± SD of 29.4 ± 3.5 mEq/L, as opposed to decreased or unchanged in MG patients 26.3 ± 3.1 mEq/L (mean ± SD) (P = 1.4 × 10,8). Furthermore, alkalosis in the TTP group persisted throughout subsequent daily treatments. There was also a significant decrease between pre- and post-TPE potassium levels in TTP patients (P = 3 × 10,21) by paired Student's t test. Additionally, samples with levels <3.3 mEq/L were alkalotic 75% of the time. In the MG group, however, potassium was normal in 85% and 83% of the pre- and post-TPE samples, respectively. Consequently, the hypokalemia was significantly more marked in the TTP group (P = 0.0008). These data confirm that plasmapheresis commonly induces metabolic alkalosis in TTP patients, probably due to high citrate in fresh frozen plasma, the frequency of treatments, and perhaps decreased renal clearance due to disease involvement of the kidneys. J. Clin. Apheresis. 16:120,124, 2001. © 2001 Wiley-Liss, Inc. [source] Thrombotic thrombocytopenic purpura: is there more than ADAMTS-13?JOURNAL OF THROMBOSIS AND HAEMOSTASIS, Issue 3 2007J. THACHIL [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] Transient TTP in childhoodPEDIATRIC BLOOD & CANCER, Issue 3 2009Naomi P. Moskowitz MD Abstract Thrombotic thrombocytopenic purpura (TTP) is a type of microangiopathic hemolytic anemia that is uncommon in childhood. Adults with TTP have a high mortality rate unless they are treated with plasma exchange. There are few reports of children with acquired idiopathic TTP, and most of those children received some form of treatment. We describe a child with acquired idiopathic TTP who had severe thrombocytopenia and anemia that resolved over several months without the use of any medications. This case suggests that some children with acquired idiopathic TTP might be safely observed without ill effects. Pediatr Blood Cancer 2009;52:424,426. © 2008 Wiley-Liss, Inc. [source] Thrombotic thrombocytopenic purpura following transurethral resection of the prostateANAESTHESIA, Issue 9 2009S. R. Benington Summary A 65-year old man developed anaemia, profound thrombocytopenia and acute renal failure 2 days after transurethral resection of the prostate. Based on the clinical picture and blood film evidence of microangiopathic haemolysis, thrombotic thrombocytopenic purpura was diagnosed. The patient was treated with a course of plasma exchange, renal replacement therapy and methylprednisolone and made a good recovery. Thrombotic thrombocytopenic purpura is an uncommon cause of haematological and renal abnormalities in the postoperative period. It has a high mortality if untreated, and should be considered in the differential diagnosis of any postoperative patient with a low platelet count and anaemia, since prompt investigation and treatment is life-saving. [source] Refractory thrombotic thrombocytopenic purpura following influenza vaccinationANAESTHESIA, Issue 4 2009P. J. Dias Summary Thrombotic thrombocytopenic purpura (TTP) is characterised by the systemic microvascular aggregation of platelets causing ischaemia of the brain and other organs. We describe the case of a 54 year-old man who presented with neurological signs, fever, severe thrombocytopenia, microangiopathic haemolytic anaemia and renal failure 5 days after receiving an influenza vaccination. He was diagnosed with acute refractory TTP caused by autoantibody-mediated ADAMTS-13 deficiency. He required stabilisation on the critical care unit before being successfully treated with 3 l plasma exchanges for 21 days and rituximab (MabThera®) at a dose of 375 mg.m,2, given weekly for a total of 4 weeks. Vaccination is an important part of preventative medicine and reduces morbidity and mortality. Only in a few rare cases has vaccination been associated with autoimmune pathology. We could find only one similar case report of thrombotic thrombocytopenic purpura following influenza vaccination. In addition to plasma exchange, rituximab appears to be effective and well tolerated in the treatment of refractory thrombotic thrombocytopenic purpura. [source] Thrombotic thrombocytopenic purpura after stem cell transplantation presenting as blurred vision and fundal infiltratesBRITISH JOURNAL OF HAEMATOLOGY, Issue 1 2006C. Hon No abstract is available for this article. [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] 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] 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] 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] 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] 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] Value of ADAMTS13 activity and inhibitor in the postmortem diagnosis of thrombotic thrombocytopenic purpuraJOURNAL OF CLINICAL APHERESIS, Issue 3 2009Denis M. Dwyre Abstract Background and Objectives: Thrombotic thrombocytopenic purpura (TTP) is a clinical diagnosis that can be difficult to establish in severely ill patients. We report a case of fulminant TTP in a woman who died before receiving plasma exchange. An autopsy plasma sample was analyzed for ADAMTS13 activity and inhibitor for correlation with the diagnosis of TTP. Recognizing that hemolysis in postmortem blood samples could interfere with ADAMTS13 activity, plasma samples from four additional decedents not suspected of having TTP were analyzed and correlated with their autopsy results. The purpose of this study was to assess whether testing postmortem samples for ADAMTS13 is useful in the postmortem diagnosis of TTP. Material and Methods: Plasma samples from the index case and four non-TTP decedents were analyzed for ADAMTS13 activity, ADAMTS13 inhibitor levels, and plasma free hemoglobin (PFH). Autopsy tissues were evaluated for evidence of platelet microthrombi in all five cases. Results: The ADAMTS13 activity level in the index patient was <4%, and the inhibitor level was 1.0 inhibitor unit. Microthrombi were prominent in the heart, kidneys, pancreas, and adrenal glands, consistent with the clinical diagnosis of TTP. ADAMTS13 activity levels in the four non-TTP decedents ranged from 4 to 82% (3/4 , 26%), and inhibitor was present in two of the four samples. Postmortem PFH levels in the four non-TTP decedents ranged from 64 to 3,917 mg/dL. No microthrombi were observed. Conclusion: Low postmortem ADAMTS13 activity and evidence of inhibitor can occur in decedents without clinical or histologic evidence of TTP. Postmortem ADAMTS13 activity levels may not be valid in establishing a diagnosis of TTP, and high inhibitor levels in this setting may be related to elevated PFH. Caution must be used in the interpretation of ADAMTS13 testing in the presence of hemolysis. J. Clin. Apheresis 2009. © 2009 Wiley-Liss, Inc. [source] Atypical presentations of thrombotic thrombocytopenic purpura: A review,JOURNAL OF CLINICAL APHERESIS, Issue 1 2009Ravi Sarode Abstract Thrombotic thrombocytopenic purpura (TTP) is diagnosed by the presence of microangiopathic hemolytic anemia and thrombocytopenia in a patient who frequently presents with central nervous system involvement and, to a lesser extent, renal dysfunction. Recent understanding of the pathophysiology of TTP due to severe deficiency of von Willebrand factor cleaving protease, known as ADAMTS13, has improved diagnosis of TTP. Once the diagnosis is suspected, life-saving therapeutic plasma exchange therapy is initiated. Occasionally, an unusual clinical presentation makes TTP diagnosis difficult, thus resulting in a delay in the management of TTP. This review highlights a variety of atypical TTP presentations described in the literature. It is intended to bring unusual scenarios to the clinician's awareness, so that timely treatment can be delivered. J. Clin. Apheresis, 2009. © 2008 Wiley-Liss, Inc. [source] Rituximab as an adjunct to plasma exchange in TTP: A report of 12 cases and review of literature,JOURNAL OF CLINICAL APHERESIS, Issue 5 2008Sushama Jasti Abstract Idiopathic thrombotic thrombocytopenic purpura (TTP) is caused by the production of autoantibodies against the Von Willebrand factor cleaving enzyme. This provides a rationale for the use of rituximab in this disease. We report a retrospective review of 12 patients treated with rituximab for TTP refractory to plasma exchange. Eleven patients were treated during initial presentation, and one patient was treated for recurrent relapse. Ten patients responded to treatment. Median time to response after first dose of rituximab was 10 days (5,32). Of the 11 patients treated during initial presentation, nine remain free of relapse after a median follow-up of 57+ months (1+,79+). Two patients died during initial treatment. One patient was lost to follow-up 1 month after achieving complete response. The patient treated for recurrent disease during second relapse remained disease free for 2years, relapsed and was treated again with rituximab, and was in remission for 22 months. She relapsed again, was retreated, and has now been in remission for 21+ months. We conclude that rituximab is an useful addition to plasma exchange treatment in TTP, but its exact role and dosing need to be verified in prospective studies. J. Clin. Apheresis, 2008. © 2008 Wiley-Liss, Inc. [source] | ||||||||||