Thrombocytopenic Purpura Patients Research Articles

Platelet/Lymphocyte Ratio (PLR) as Prognostic Factor Thrombocytopenic Purpura in Patients of Immune

Abstract Background platelet/lymphocyte ratio (PLR) is a new inflammatory marker and its level increases in many inflammatory diseases, diabetes and malignancy. In ITP patients thereis decrease in platelet count beside inflammation, so PLR is a good prognostic factor in ITP. Objective The aim of the study is to know the relation between platelet /lymphocytes ratio and the prognosis of immune thrombocytopenia Patients and Methods This was prospective study conducted on 96 patients diagnosed as immune thrombocytopenic purpura attending hematology unit of Ain Shams University divided into to3 groups (newly diagnosied, Resistant and splenectomized) and followed them after 3 months. Results Comparison between ITP sub-groups regarding CBC results at baseline revealed that Newly diagnosed ITP patients had significantly higher PLR compared to other groups while resistant/relapsed ITP patients had significantly lower PLR compared to other two groups. (table 2). Comparison between ITP sub-groups regarding CBC results after 3 months revealed that resistant/relapsed ITP patients had significantly lower PLR compared to other two groups, (table 3). on comparison PLR in newly-diagnosed ITP before and after 3 months of treatment PLR was higher Highly significant (P < 0.001) On comparison PLR in Resistant group pre and after 3 months of treatment it was lower highly significant (p < 0.001) On comparison PLR in splenectomy group pre and after 3 months of treatment PLR was higher Highly significant (p < 0.001) Conclusion PLR is a good indicator for ITP patient of different categories (newly diagnosied, Resistant and splenectomized).

Outcomes of rituximab as treatment for immune thrombocytopenic purpura in patients who have failed steroids or IVIG: A retrospective study in a community practice.

e23256 Background: Immune thrombocytopenic purpura (ITP) is a challenging disorder with multiple treatment options. The 2019 American Society of Hematology guidelines outline treatment recommendations for ITP which include intravenous immunoglobulin (IVIG) and steroids as first-line, with Thrombopoietin receptor agonists (TPO-RAs) and Rituximab as second-line agents. They favor TPO-RAs over Rituximab but suggest that the choice should be individualized. In this study, we explore the rate and duration of response to Rituximab as a second-line agent for ITP. We also analyze relapse rates and response to sequential TPO-RAs. Methods: A retrospective study of patients with ITP managed in a community practice from 2018 to 2022. The practice’s electronic medical records were searched for the keywords “ITP” and “Rituximab”. 87 patients fit the search terms. We included patients above age 18 treated with Rituximab for ITP after failing steroids or IVIG. We excluded duplicates, incomplete medical records, patients given Rituximab for reasons other than ITP, patients treated with medications other than steroids or IVIG prior to Rituximab, and patients given less than 4 doses of Rituximab. 60 out of 87 patients were eligible. The primary outcome was response to Rituximab defined as platelets above 30,000 per microliter of blood after 12 weeks of its initiation. Secondary outcomes include relapse rate, duration of response, and subsequent response to TPO-RAs. Secondary outcomes were determined by chart-reviewing physician documentation. Results: SAS 9.4 (SAS/STAT Inst., Carry NC) was used for all data. Categorical variables were quoted as absolute numbers and relative frequencies. A test result was considered statistically significant if p < 0.05. For categorical variables, likelihood ratio chi-square, and Fisher’s exact tests were conducted for 2*2 and r*c contingency tables to test associations. 96.7% of eligible patients responded to Rituximab (P < 0.0001), however 46.7% relapsed. 42.9% of relapses happened in less than 1 year, 14.3% in 1 year, 21.4% in 2 years, 7.1% in 3 years, 10.7% in 4 years, and 3.6% in 5 or more years (P = 0.0043). The response rate to TPO-RAs after Rituximab failure was 88%. No significant toxicity from Rituximab was noted beyond previously reported minor side effects, and there was no treatment-related death. Conclusions: The response rate to Rituximab was 96.7% (P < 0.0001) which was sustained in 53.3% of patients. Most relapsed patients responded well to TPO-RAs. These numbers are very comparable to previously reported response to Rituximab. Therefore, we believe that the strategy of using Rituximab as second-line and reserving TPO-RAs for non-responders is clinically-effective and certainly cost-effective given the fact that Rituximab is administered over four weeks as compared to the often lifetime treatment with TPO-RAs.

Stress-induced hyperglycemia is associated with the mortality of thrombotic thrombocytopenic purpura patients

BackgroundThrombotic thrombocytopenic purpura (TTP) is a rare thrombotic microangiopathy with a rapid progression and high mortality rate. We aimed to explore early risk factors for mortality in patients with TTP.MethodsWe conducted a retrospective analysis of 42 TTP patients that were admitted to our hospital between 2000 and 2021, with a median age of 49 (29–63) years. Risk factors for mortality were evaluated using multivariate logistic regression. Receiver operating characteristic curve analysis was used to determine the cut-off value of glucose for predicting mortality in patients, which was validated by comparison to a similar cohort in the published literature.ResultsElevated glucose level and reduced red blood cells (RBC) counts were risk factors for mortality in patients with TTP (glucose, odds ratio and 95% confidence interval: 2.476 [1.368–4.484]; RBC, odds ratio and 95% confidence interval: 0.095 [0.011–0.799]). The area under the curve of glucose was 0.827, and the cut-off value was 9.2 mmol/L, with a sensitivity of 75.0% and specificity of 95.8%. A total of 26 cases from the validation cohort had a sensitivity of 71.0% and a specificity of 84.0%. The change trends of the TTP-related laboratory indices differed during hospitalization.ConclusionHyperglycemia at admission and unstable blood glucose levels during hospitalization may be potential predictors of mortality for TTP patients. The improved prognosis was associated with the recovery of platelet counts and a significant decrease in serum lactate dehydrogenase after five days of treatment.

Nivolumab-induced Thrombotic Thrombocytopenic Purpura in Patients with Gastric Tube Cancer.

Recently, immune checkpoint inhibitors (ICIs) have been used to treat several cancer types. ICIs have been reported to cause a wide variety of immune-related adverse events, including endocrine, neurologic, gastrointestinal, and cutaneous disorders. Thrombotic thrombocytopenic purpura (TTP) is an autoimmune hematologic disorder characterized by the presence of autoantibodies against a disintegrin and metalloprotease with thrombospondin-1, member 13. Several previous cases of TTP were thought to have been caused by ICI treatment. We herein report a rare case of TTP that developed after long-term treatment with an ICI (nivolumab) for gastric tube cancer.

Does Caplacizumab for the management of thrombotic thrombocytopenic purpura increase the risk of relapse, exacerbation, and bleeding? An updated systematic review and meta-analysis based on revised criteria by the International Working Group for thrombotic thrombocytopenic purpura.

Thrombotic thrombocytopenic purpura (TTP) is a rare and life-threatening condition marked by abnormal blood clotting and organ damage. Caplacizumab is a potential treatment for the TTP management. This systematic review and meta-analysis aimed to assess Caplacizumab's effectiveness and safety in the TTP management. A comprehensive database search identified nine studies, including randomized controlled trials and observational studies. Primary outcomes included TTP exacerbation, relapse, and major bleeding. Major bleeding risk was evaluated using updated definitions recommended by the International TTP Working Group in 2021. Revised criteria proposed by the IWG for TTP recurrence were employed for a comprehensive assessment of Caplacizumab's impact on relapse and exacerbation. Analysis revealed Caplacizumab significantly reduced all-cause mortality in TTP patients. Some studies raised concerns about bleeding risk, but overall, it did not significantly differ from standard treatment. Likewise, there was no significant difference in TTP relapse rates between Caplacizumab and standard care. This study supports Caplacizumab as a potential adjunct therapy for TTP. However, careful consideration of its advantages and risks is crucial in clinical practice. Further research is needed to address concerns related to adverse effects like bleeding risk and relapse rates associated with Caplacizumab in the TTP management. The findings emphasize the importance of weighing potential benefits and risks when considering Caplacizumab as an adjunct therapy for TTP.

A-154 Cost-effectiveness of On-site Vs Send-out Measurement of ADAMTS13 Activity in the Management of Acquired Thrombotic Thrombocytopenic Purpura

Abstract Background Thrombotic thrombocytopenic purpura (TTP) is a rare thrombotic microangiopathy (TMA) caused by congenital or acquired severe deficiency of disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13 (ADAMTS-13), an enzyme cleaving von Willebrand factor (VWF). ADAMTS-13 deficiency leads to the accumulation of ultra-large VWF multimers, and subsequent platelet aggregation and microthrombi formation. Aside clinical symptoms, the diagnosis of TTP is based thrombocytopenia, hemolytic anemia with the presence of schistocytes, and severely reduced activity of ADAMTS-13 (<10%). As TTP is a life-threatening condition, it is of great importance to shorten the time to the treatment, and according to the French recommendations, the triplet therapy including plasma exchange (PEX), rituximab, caplacizumab, plus corticosteroids, must be administered to patients with suspected TTP until the results of ADAMTS-13 was obtained, and be pursued only in the case of deficiency. Therefore, a rapid ADAMTS-13 testing is critical for early diagnosis and optimal management of acute TTP. Methods We compared the cost-effectiveness of a strategy based on on-site measurement of ADAMTS-13 activity using a rapid fully automated chemiluminescent assay (HemosIL AcuStar ADAMTS-13) vs its centralized measurement in a reference laboratory, as it is currently the case at our institution. For that purpose, we created two scenarios based on either its local measurement with a maximum time-to-result of 16 h (assay performed during daytime, 7/7) or its centralized measurement with a median time-to-result of 4 days (range: 2–8, as demonstrated from the 2018–2021 estimates). The triplet therapy was hypothesized to be started on admission and stopped if ADAMTS-13 activity was >10%. In case of ADAMTS-13 activity <10%, an inhibitor screening would be performed at the reference laboratory, as this assay was not expected to be locally implemented. For the calculation, we considered the costs of reagent, shipping/handling of samples to the reference laboratory, laboratory staff workload, reimbursement rates, and treatments administered in patients with suspected TTP until availability of the ADAMTS-13 result. Results Based on 2018 to 2021 estimates, there was a mean of 60 prescriptions of ADAMTS-13 activity per year. Most were for the follow-up of TTP patients and for patients with non-TTP TMA. Only seven were for the diagnosis of acute TTP, as suggested by the French score, which was confirmed in four cases (mean values). The local measurement of ADAMTS-13 activity was found to be cost-effective with a 16%-reduction of the costs compared to its centralized measurement in those seven patients with suspected TTP during the first 4 days. Conclusion Together with its short turnaround time (33 min) and its full automation, our results suggest that the HemosIL AcuStar ADAMTS-13 activity assay could be an assay of choice to rapidly measure ADAMTS-13 activity in the plasma and thus to establish the diagnosis of acute TTP in emergency settings. Considering the local costs, using the strategy based on the local evaluation of ADAMTS-13 activity led to a 16%-reduction of the overall costs during the first 4 days after admission of the seven patients with suspected acute TTP.

Immune thrombotic thrombocytopenic purpura in patients over 60 years of age: Diagnostic pitfalls and treatment strategy.

Real-world registry studies of older patients with iTTP highlight diagnostic difficulties in comparison to patients less than 60 years of age with greater risk of renal injury, atypical neurological features and less profound cytopenia, which can result in diagnostic delays. However, there is no clear signal of significantly increased toxicity from full active treatment. Commentary on: Gómez-Seguí et al. Immune thrombotic thrombocytopenic purpura in older patients: results from the Spanish TTP Registry (REPTT). Br J Haematol 2023;203:860-871.

Prognostic factors in thrombotic thrombocytopenic purpura

Abstract Objectives Thrombotic thrombocytopenic purpura (TTP) is a rare thrombotic microangiopathy with no standardized prognostic model to predict mortality. The aim of the study is to determine parameters associated with TTP-related mortality. Methods In this cross-sectional, retrospective study, 77 TTP patients, treated with therapeutic plasma exchange between 2001 and 2019 in Ankara University Faculty of Medicine were included. Results There was no significant relationship between ADAMTS13 inhibitor levels, activity and mortality. Median number of plasmapheresis was 10 (2–32), higher in patients with complete response. Anemia, kidney injury and LDH levels were associated with survival; there was no significant relationship between platelet counts at the time of diagnosis and mortality. Mortality was lower in patients with platelet counts above 100 × 109/L and normalized LDH after treatment. Hemoglobin, albumin, LDH and creatinine levels at the time platelet counts exceeded 50 × 109/L were associated with survival. Conclusions We determined several clinical and laboratory parameters associated with mortality. Fewer numbers of plasmapheresis was associated with mortality; thus other treatments as rituximab and caplacizumab should be considered early in non-responders. Including changes in laboratory parameters may be considered in prognostic scoring systems to be developed in the future.

Comparison of absolute immature platelet count to the PLASMIC score at presentation in predicting ADAMTS13 deficiency in suspected thrombotic thrombocytopenic purpura

IntroductionThrombotic thrombocytopenic purpura (TTP) demands rapid initiation of therapeutic plasma exchange to avoid severe complications associated with it. ADAMTS13 activity <10% defines TTP, however, this is a send-out test at most institutions. We have previously reported our experience looking at absolute immature platelet counts (A-IPC) in TTP patients. Thus, we compared A-IPC on admission to the PLASMIC score to predict ADAMTS13 deficiency. Materials and methodsOf seventy-two patients identified, 52 met inclusion criteria. All patients were suspected of new-onset TTP and had A-IPC on admission. Of these patients, 25/52 were later shown to have ADAMTS13 <10%, defined as TTP group, and 27/52 had ADAMTS13 >10% and are henceforth classified as non-TTP. ResultsPatients with TTP were found to have A-IPC below reference range (<1.5 × 109/L) and responded to therapy as shown by fold-increases in A-IPC (p < 0.0001), neither seen in non-TTP patients. A-IPC had a significant correlation with ADAMTS13 deficiency (p = 0.0001) with high sensitivity, specificity, positive and negative predictive values. Comparison of A-IPC to PLASMIC score indicated that the former identified all TTP patients compared to PLASMIC score even in patients obtaining scores of 4 and 5. Finally, Receiver Operating Characteristic curves showed A-IPC had area under the curve of 0.986. ConclusionsA-IPC below reference range and A-IPC fold-increases were only observed in TTP patients. There was strong association between A-IPC and ADAMTS13 deficiency and A-IPC predicted patients with ADAMTS13 deficiency. Future larger studies are needed to determine ways to apply findings in suspected TTP patients.

Proteinuria Increases the PLASMIC and French Scores Performance to Predict Thrombotic Thrombocytopenic Purpura in Patients With Thrombotic Microangiopathy Syndrome

IntroductionPLASMIC and French scores have been developed to help clinicians in the early identification of patients with thrombotic thrombocytopenic purpura (TTP). Nevertheless, the validity of these scores in thrombotic microangiopathy (TMA) cohorts with low TTP prevalence remains uncertain. We aimed to evaluate their diagnostic value in routine clinical practice using an unselected cohort of patients with TMA. We also analyzed the value of adding proteinuria level to the scores. MethodsWe retrospectively included all patients presenting with a biological TMA syndrome between January 1, 2008, and December 31, 2019, in a tertiary hospital. TMA etiology was ascertained, and scores were evaluated. Modified scores, built by adding 1 point for low proteinuria (<1.2 g/g), were compared with original scores for TTP prediction. ResultsAmong 273 patients presenting with a full biological TMA syndrome, 238 were classified with a TMA diagnosis. Complete scores and proteinuria level were available in 134 patients with a TTP prevalence of 7.5%. Area under the receiver operating characteristic curve (AUC) of PLASMIC and French scores for TTP diagnosis was 0.65 (0.46–0.84) and 0.72 (0.51–0.93), respectively. AUC of modified PLASMIC and French scores was 0.76 (0.59–0.92) (P = 0.003 vs. standard score) and 0.81 (0.67–0.95) (P = 0.069 vs. standard score), respectively. Specificity, positive predictive value (PPV), and positive likelihood ratio of high-risk scores were significantly improved by adding proteinuria level. ConclusionPLASMIC and French scores have low predictive values when applied to an unselected TMA cohort. Including proteinuria level in the original scores improves their performance for TTP prediction.

Outcomes in 1096 patients with severe thrombotic thrombocytopenic purpura before the Caplacizumab era.

IntroductionThrombotic thrombocytopenic purpura (TTP) is a diagnostic and therapeutic emergency. Therapeutic plasma exchange (TPE) combined with immunosuppression has been the cornerstone of the initial management. To produce optimal benefits, emerging treatments must be used against a background of best standard of care. Clarifying current uncertainties is therefore crucial.MethodsThe objective of this study was to analyze a large high-quality database (Marketscan) of TTP patients managed between 2005 and 2014, in the pre-caplacizumab era, in order to assess the impact of time to first TPE and use of first-line rituximab on mortality, and whether mortality declines over time.ResultsAmong the 1096 included patients (median age 46 [IQR 35–55], 70% female), 28.8% received TPE before day 2 in the ICU. Hospital mortality was 7.6% (83 deaths). Mortality was independently associated with older age (hazard ratio [HR], 1.024/year; 95% confidence interval [95%CI], [1.009–1.040]), diagnosis of sepsis (HR, 2.360; 95%CI [1.552–3.588]), and the need for mechanical ventilation (HR, 4.103; 95%CI, [2.749–6.126]). Factors independently associated with lower mortality were TPE at ICU admission (HR, 0.284; 95%CI, [0.112–0.717]), TPE within one day after ICU admission (HR, 0.449; 95%CI, [0.275–0.907]), and early rituximab therapy (HR, 0.229; 95% CI, [0.111–0.471]). Delayed TPE was associated with significantly higher costs.ConclusionsImmediate TPE and early rituximab are associated with improved survival in TTP patients. Improved treatments have led to a decline in mortality over time, and alternate outcome variables such as the use of hospital resources or longer term outcomes therefore need to be considered.

Idiopathic Thrombocytopenic Purpura in Patients Admitted to Benghazi Children Hospital

Introduction: Childhood immune thrombocytopenia (ITP) is an acquired autoimmune disorder characterized by isolated thrombocytopenia (peripheral blood platelet count &lt;100 x 109/L). ITP is one of the most common bleeding disorders in children, with an incidence of approximately four per 100.000 per year. Aim: Aim is to review presenting features, response to therapy, and natural history of ITP treated at Benghazi medical center, department of pediatric. Methods: Cross-sectional descriptive study was carried out at the Pediatric Department of Benghazi medical center. Results: The study included 86 patients their mean age was 4.2±2.7years,with minimum age 11months and maximum age 12years.Male to female ratio was 1:1.15, female constitute to more than half (53.5%) .Mean duration of hospital stay was 5.2± 4.3 days , minimum duration was one day and maximum was 23 days. Bruise was presented by 84.9% of patients, 67.4% was presented by history of upper respiratory tract infection and 30.2% presented with bleeding from different site. Repeated admission was recorded in 93% of the patients. Family history of ITP was recorded in 4.7%. Respiratory manifestation was present in 4.8%, CNS manifestation present in 2.4%, hepatosplenomegaly in 2.4%, Lymphoadnopathy in 2.4%, renal manifestation in 1.2% and musculoskeletal manifestation in 1.2%. WBC count was &gt;4 in 1.2% , 4-11 in 73.3% and 25.5% had level &gt; 11 x103/ml, mean level was 9.7± 3.4 x103/ ml. Sever decrease of platelets was recorded in 54.7% of the patients , 36% had moderate decrease , mild recorded in 9.3% and no one had normal count .Peripheral blood smear, 26.7% of patients had normocytic normochromic. 25.5%was microcytic hypochromic 4.7% had microcytic normochromic, 1.2% had atypical lymphocyte with decrease plat, 1.2% had Normocytic hypochromic, 14% were normal and 15.5% not done. Bone marrow was normal in 68.5% of the patients, 1.2% of patients had thrombocytopenia, 1.2% of them had aplastic anemia, 1.2% of .........

Predictive Value of Risk Factors for Chronic Idiopathic Thrombocytopenic Purpura in Patients with Acute Type of Disease

BACKGROUND: Immune thrombocytopenic purpura (ITP) is an autoimmune disease in which autoantibodies react with platelet surface antigens and results in mild to severe thrombocytopenia due to decreased platelet count or inhibition of platelet production. Given the relatively high prevalence of ITP among children and the lack of standard diagnostic testing for the diagnosis of chronic disease, this study evaluated the predictive value of risk factors for chronic ITP in hospitalized patients.&#x0D; METHODS: This prospective cohort study was performed on 65 children with ITP who referred to Ali Asghar and Rasool Akram Hospitals in Tehran, Iran, during the years 2017 and 2018. Relationships between different risk factors, including age of diagnosis, gender, white cell count, primary platelet count, mean platelet volume (MPV), history and type of the previous patient infection, FCG gene mutation, and type of FCG mutation with a chronic disease incidence were investigated using multiple logistic regression model.&#x0D; RESULTS: Of 65 patients, 31 (47.69%) were male and 34 (52.31%) were female included in the study. Twenty-eight patients (43.08%) had acute ITP and 37 (56.92%) had chronic ITP. Frequency of FCG gene mutation in patients with chronic and acute type ITP was 16.36% and 7.27%, respectively (p = 0.51). No association was found between the history of the previous infection and its type with the chronic incidence of ITP. The multiple logistic regression model showed that three factors, including the absolute number of lymphocytes, age of diagnosis, and primary white blood cells (WBC) count were directly linked to chronic ITP. Furthermore, three factors of platelet, sex, and MPV were indirectly related to chronic ITP. In addition, the absolute number of lymphocytes, age of diagnosis and primary WBC count were significantly associated with chronic ITP. The receiver operating characteristic analysis showed that the cutoff rate of these factors was 0.31. Further analysis of these risk factors in comparison with the gold standard demonstrated that the diagnostic sensitivity and specificity of these risk factors for chronic ITP were 73.08% and their specificity was 88.57%, indicating the high importance and predictive power of these risk factors.&#x0D; CONCLUSIONS: According to the results of this study, for the first time in Iran, six risk factors, including the absolute number of lymphocytes, age at diagnosis, sex, MPV level, platelet level at time of diagnosis, and primary WBC count were considered as the most important risk factors affecting the incidence of chronic ITP. Of course, more comprehensive studies can definitely lead to more comprehensive models.

Immune-mediated thrombotic thrombocytopenic purpura in patients with and without systemic lupus erythematosus: a retrospective study

BackgroundThrombotic thrombocytopenic purpura (TTP) is associated with more deleterious outcomes in patients with systemic lupus erythematosus (SLE). However, ADAMTS13 (a disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13) levels and ADAMTS13 inhibitor were not routinely assayed in most previous studies. The objective of this study is to compare the characteristics and outcomes of immune-mediated TTP (iTTP) in patients with and without SLE.MethodsThe medical data of 28 patients with iTTP from Peking Union Medical College Hospital were analysed. ADAMTS13 activity and ADAMTS13 inhibitor were measured in all patients.ResultsAll 28 patients had ADAMTS13 inhibitor and severe ADAMTS13 deficiency. iTTP was considered SLE-related (SLE-TTP) in 10 patients and primary (primary iTTP) in 18 patients. Renal involvement on presentation was more severe in patients with primary iTTP as determined by higher serum creatinine (162.7 ± 110.6 vs 73.3 ± 13.4 μmol/L, p < 0.01) and more prevalent acute kidney injury (72.2% vs 10.0%, p < 0.01) than in patients with SLE-TTP. More patients with SLE-TTP were treated with steroid pulse therapy (90.0% vs 16.7%, p < 0.01) and intravenous immunoglobulin (IVIG) (50.0% vs 5.6%, p = 0.01) compared to patients with primary iTTP. After adjustments for age and treatment, including steroid pulse therapy and IVIG treatment, the likelihood of clinical remission of SLE-TTP was significantly increased compared to that of primary iTTP (HR 7.6 [1.2, 50.1], p = 0.03). Mortality was also lower among patients with SLE-TTP than among patients with primary iTTP (0 vs 38.9%, p = 0.03).ConclusionsRenal involvement was less severe in patients with SLE-TTP than in patients with primary iTTP. The treatment responses and outcomes of SLE-TTP were no worse and perhaps even better than those of primary iTTP. When TTP is diagnosed in SLE patients, the ADAMTS13 level and ADAMTS13 inhibitor profile should be considered in addition to clinical features.

Immune Thrombocytopenic Purpura in Patients with COVID-19.

We described three COVID-19-infected patients with profound immune thrombocytopenia causing haemorrhagic mucocutaneous complications. We conclude that an immune mechanism was responsible as common causes were excluded. Since corticoids were considered harmful in the circumstances, the patients were successfully treated with intravenous immunoglobulins without later relapse.LEARNING POINTSThe severity of haemorrhagic syndrome is not correlated with the severity of COVID-19 infection.Thrombocytopenia in mild COVID-19 infection seems to have an autoimmune mechanism.Intravenous immunoglobulins (1 g/kg) should be the first line of treatment.

Time from suspected thrombotic thrombocytopenic purpura to initiation of plasma exchange and impact on survival: A 10-year provincial retrospective cohort study

BackgroundThrombotic thrombocytopenic purpura (TTP) is a thrombotic microangiopathy (TMA) with significant morbidity and mortality. Guidelines recommend initiating plasma exchange within 4–8 h of suspected diagnosis. It is unclear what are real-world practice patterns and whether delays >8 h increases mortality. ObjectivesTo determine if delayed initiation of plasma exchange is associated with increased risk of death and complications. MethodsIn this retrospective cohort study, we evaluated the time from suspected diagnosis to plasma exchange in all adults presenting with suspected TTP to apheresis centres in Alberta, Canada (2008–2018). Among patients with acquired TTP, the association between delayed plasma exchange and risk of death was evaluated using Cox regression. ResultsOverall 190 episodes of suspected TTP were included among 163 individuals. Acquired TTP was confirmed in 61 patients. Inappropriate Emergency Department triage occurred in 59%. The median time from suspected diagnosis to first plasma exchange was 10.7 h; 59% had delayed plasma exchange >8 h, among whom plasma infusion was administered in only 45%. 36% of suspected TTP and 13% of confirmed TTP patients died. Delayed plasma exchange between 8 and 24 h was not associated with a significantly higher risk of death (adjusted hazards ratio; aHR 0.63, 95% CI 0.08–4.83) in confirmed TTP. On the other hand, the risks of death (aHR 1.40, 95% CI 0.20–9.79) and major thrombotic events (aHR 2.9, 95% CI 0.6–12.8) were markedly increased with >24 h delay. ConclusionsOur study demonstrated that TTP care in a real-world setting is discordant with expert guidelines due to multiple barriers. There is a gradient of increased mortality risk and thrombotic complications with longer treatment delays, although the study is likely underpowered.

METHYLPREDNISOLONE INDUCED HYPOKALEMIA IN AN IDIOPATHIC THROMBOCYTOPENIC PURPURA (ITP) PATIENT

We reported an Idiopathic Thrombocytopenic Purpura (ITP) patient, 66-years-old (woman), with hypokalemia. She received 125 mg three times daily of methylprednisolone injection for her ITP. Corticosteroids are the initial treatment of ITP. Her potassium level decrease after she took methylprednisolone. Hypokalemia is also a common problem affecting the elderly or geriatric population. Many literatures reported side effects of corticosteroid is associated with hypokalemia. Monitoring potassium levels must be check during corticosteroid therapy. In this report, we describe the association between corticosteroid with hypokalemia effect.

Thrombotic Thrombocytopenia Purpura Case Series in Skmc 2016-2017

Background and Purpose Thrombotic thrombocytopenic purpura (TTP) is a heterogeneous disease primarily characterized by thrombocytopenia and microangiopathic hemolytic anemia. Therapeutic plasma exchange has dramatically improved mortality, allowing for emergence of refractory, relapsing, and atypical presentations. in this case series we aim to present our institutional data for Apheresis in Sheikh khalifa medical City in AbuDhabi. We will also present patient demographic and clinical presentation and treatment protocol we use Methodology -Case series with Retrospective review. -Routine laboratory tests such as peripheral blood cell counts, reticulocyte count, coagulation profile, serum lactate dehydrogenase (LDH), bilirubin, serum creatinine, cardiac enzymes, and urinalysis, were performed. -ADAMTS13 levels and inhibitor titer were determined for all patient in outside lab -Baseline demographic characteristics were calculated in frequencies and percentages. (include age ,Gender , clinical manifestations and treatment strategy) Results and Discussions thrombotic thrombocytopenic purpura (TTP) pentad consisting of fever, thrombocytopenia, microangiopathic hemolytic anemia (MAHA), neurological abnormalities, and renal failure. less than 5 % of patient reported in literature have all associated clinical features. -Total of 10 patients M:F 4:2 , Median Age 44yr 50% presented with Neurological manifestations and renal disease , 30% presented with Fever only 20% had cardiac manifestation on admission . None of the patient presented with all 5 pentad. -All patients received TPE , steroid . -90 % of the patients received Rituximab except for 1 because of Allergy. -All patients has low ADAMTS 13 , except one has normal ADAMTS13 but came with relapse and on first admission had low ADAMTS13 -All patient presented with MAHA and TCP except 2 patient whom had normal Hb but significant schistocytes on peripheral blood with TCP both patient where relapsed cases. -3 patient were relapsed 7 de novo , the 3 relapsed cases all did not receive Rituximab in first remission . One of them relapsed twice but did not received Rituximab due to allergy -Although some publication include large number of TTP patients, but only few case reports have evaluated the clinical feature, laboratory parameters and therapeutic outcome of TTP. Without treatment, TTP is almost uniformly fatal with a mortality rate approaching 90%. With the timely institution of therapeutic plasma exchange (TPE) mortality decreases to about 10%-20%. A disintegrin and metalloprotease with thrombospondin Type 1 motif, Member 13 (ADAMTS13) levels less than 5% are a hallmark of TTP. We do ADAMTS 13 Activity and inhibitor titre levels in outside facility TAWAM hospital with turn-around time of 7 days which is helpful in planning Rituximab treatment. with availability of Rituximab our relapse rates are low but not zero Conclusions -Thrombotic thrombocytopenic purpura (TTP) pentad consisting of fever, thrombocytopenia, microangiopathic hemolytic anemia (MAHA), neurological abnormalities, and renal failure. -5 % of patient reported in literature have all associated clinical features. -We found that majority of patient presented with evidence of thrombocytopenia and MAHA only. -Without treatment, TTP is almost uniformly fatal with a mortality rate approaching 90%. With the timely institution of therapeutic plasma exchange (TPE) mortality decreases to about 10%-20%. -TPE ,steroid and rituximab was very effective in achieving sustain remission in 100% of ours patients with median follow up 8 month -More awareness is needed for early diagnosis and early referral to centers with appropriate tertiary care facilities. Figure Disclosures No relevant conflicts of interest to declare.

Predictive Value of Risk Factors for Chronic Idiopathic Thrombocytopenic Purpura in Patients with Acute Type of Disease

Background: Immune thrombocytopenic purpura (ITP) is an autoimmune disease in which autoantibodies react with platelet surface antigens and results in mild to severe thrombocytopenia due to decreased platelet count or inhibition of platelet production. Given the relatively high prevalence of ITP among children and the lack of standard diagnostic testing for the diagnosis of chronic disease, this study evaluated the predictive value of risk factors for chronic ITP in hospitalized patients. Methods: This prospective cohort study was performed on 65 children with ITP who referred to Ali Asghar and Rasool Akram hospitals in Tehran, Iran during the years 2017 and 2018. Relationships between different risk factors including age of diagnosis, gender, white cell count, primary platelet count, Mean platelet volume (MPV) , history and type of previous patient infection, FCG gene mutation, and type of FCG mutation with chronic disease incidence were investigated using multiple logistic regression model. Results: Of 65 patients, 31 (47.69%) were male and 34 (52.31%) were included in the study. 28 patients (43.08%) had acute ITP and 37 (56.92%) had chronic ITP. Frequency of FCG gene mutation in patients with chronic and acute type ITP was 16.36% and 7.27%, respectively (p = 0.51). No association was found between the history of previous infection and its type with the chronic incidence of ITP. Multiple Logistic regression model showed that 3 factors including absolute number of lymphocytes, age of diagnosis and primary WBC count were directly linked to chronic ITP. Furthermore, 3 factors of platelet, sex and MPV were indirectly related to chronic ITP. In addition, absolute number of lymphocytes, age of diagnosis and primary WBC count were significantly associated with chronic ITP. The ROC analysis showed that the cut off rate of these factors was 0.31. Further analysis of these risk factors in comparison with the gold standard demonstrated that the diagnostic sensitivity and specificity of these risk factors for chronic ITP were 73.08% and their specificity was 88.57%, indicating the high importance and predictive power of these risk factors. Conclusion: According to the results of the current study, evaluation of 6 major risk factors including absolute lymphocyte count, age at diagnosis, sex, MPV level, platelet level at diagnosis and primary WBC count for the identification of chronic ITP is recommended before IVIG treatment. Of course, more comprehensive studies can definitely lead to more comprehensive models. Funding Statement: The authors stated: Not applicable. Declaration of Interests: The authors declare that they have no competing interests. Ethics Approval Statement: The children participated in the study with the full parental consent, ensuring that all children's information was kept confidential. The project was initially approved by the Ethics Committee of Iran University of Medical Sciences with code IR.IUMS.FDM.REC. 1396.29265

Thrombotic thrombocytopenic purpura patients' attitudes toward depression management: A qualitative study.

Background and aimsThrombotic thrombocytopenic purpura (TTP) is a rare disorder characterized by acute episodes of systemic microvascular thrombosis; TTP is more common in adults, women, and African‐Americans (Blacks). Our Oklahoma TTP Registry documented that survivors have an increased prevalence of depression compared with the general population; however, many patients' depression remains untreated. Moreover, studies identifying attitudes toward depression management are lacking. The objective of this study was to identify TTP patients' attitudes towards pharmacotherapy. As a secondary question, we explored attitudes towards counseling.MethodsWe interviewed TTP patients with major depression who had experience with different management strategies (previous/current pharmacotherapy treatment versus no pharmacotherapy treatment). Eligibility criteria included (a) age > 18 years, (b) ADAMTS13‐deficient TTP, (c) enrolled in the Oklahoma Registry, and (d) moderate/major depression on either the Beck Depression Inventory II or Patient Health Questionnaire from 2004 to 2012. Qualitative purposive sampling was used to interview patients with a range of experiences with TTP and depression symptom management. Our study was based on the theoretical framework of the Theory of Reasoned Action. Patients were asked about their views on depression (attitudes), their family and friends' views (social norms), and ways they cope with depression.ResultsSemi‐structured interviews were conducted between June and October 2013. Data saturation was achieved after interviewing 16 patients (nine, pharmacotherapy and seven, no pharmacotherapy). The majority (88%) were women; 56% were Black, and the median age was 49 years. Patients in both groups believed TTP was life altering and traumatic and that counseling improved depressive symptoms. However, the pharmacologic group believed medication improved one's quality of life, whereas the no pharmacotherapy group was not sure pharmacotherapy was effective and expressed fears related to potential addiction and side effects. When asked about cultural views to depression management, many Black patients stated that in the Black community, a person is taught to deal with his/her emotional issues instead of asking strangers for help.ConclusionEnsuring effective depression management is a critical part of TTP care. Understanding attitudes toward management will assist in tailoring patient discussions.