Evidence of persistent neurologic injury following thrombotic thrombocytopenic purpura.

Despite improvements in our understanding of the pathophysiology of thrombotic thrombocytopenic purpura (TTP), little data exist regarding the long-term sequelae following a diagnosis of TTP. We present the results of a comprehensive evaluation of neurologic injury that included a magnetic resonance imaging (MRI), a neurocognitive testing, and an evaluation of health-related quality of life. Twenty-seven patients with a history of idiopathic TTP functioning normally in their activities of daily living were recruited from existing patient cohorts at both the Ohio State University (n 5 12) (Columbus) and the University College London Hospitals (n 5 15) (London, UK). Nine of 23 (39%) of the MRI studies were abnormal; 17/27 (63%) patients demonstrated neurocognitive impairment, particularly in tests of visual learning and memory. Health-related quality of life scores were also significantly lower than age- and gender-matched US norms for both the composite mental component score and physical component score. These data suggest that the prevalence of neurologic findings in TTP patients in remission is quite high and is largely undetected by routine clinical evaluations. Further longitudinal study will be required to define the risk for neurologic injury and the long-term prognosis in patients previously diagnosed with TTP.

Demographic and ADAMTS13 biomarker data as predictors of early recurrences of idiopathic thrombotic thrombocytopenic purpura.

OBJECTIVE: Approximately 40% of idiopathic thrombotic thrombocytopenic purpura (TTP) patients will suffer an exacerbation (recurrence of TTP within 30 d after their last plasma exchange (PE) procedure), but there are no data to predict who is at greater risk. We studied the clinical utility of demographic and ADAMTS13 biomarker data to predict the risk for exacerbation. PATIENTS: Forty-four acute episodes of idiopathic TTP from 26 patients were studied. METHODS: PE was performed plus either prednisone (1 mg/kg/d) or cyclosporin (2-3 mg/kg/d) as adjuncts. PE was continued daily until response (platelet count >150 000/microL and normalized lactate dehydrogenase) and tapered uniformly in all patients. ADAMTS13 biomarkers were studied prior to PE and after achieving a response, but within 7 d of the last PE. RESULTS: African American race (AA) was associated with an increased risk for exacerbation (P = 0.046). ADAMTS13 at presentation was also significantly lower in patients experiencing an exacerbation (P = 0.0364). After adjusting for the race effect, ADAMTS13 remained marginally significant (P = 0.0569). CONCLUSIONS: AA is significantly associated with an increased risk for exacerbations of TTP. These data also suggest that decreasing pretreatment ADAMTS13 activity was associated with an increased risk for exacerbation, even after accounting for the effect of race.

Effect of a Pharmacist-Driven Monitoring Program and Electronic Health Record on Bleeding Log Completeness and Documentation.

BACKGROUND: Pharmacists have the ability to enhance comprehensive care for bleeding disorders patients by bridging the gap between hemophilia treatment centers (HTC) and specialty pharmacies, specifically by monitoring bleeding logs. In September 2015, a pharmacist-driven monitoring program was implemented through the specialty pharmacy associated with a medical center to improve bleeding log completeness and electronic documentation for HTC patients. OBJECTIVE: To measure the effect of a pharmacist-driven bleeding disorder monitoring program on bleeding log completeness, successful bleeding log documentation in the electronic health record (EHR), and pharmacist-driven clinical interventions using an EHR tool. METHODS: A single-group pre-post intervention study was conducted of a pharmacist-driven monitoring program. Pre-implementation (January 1, 2014-December 31, 2014), all patients who received and returned a bleeding log following an appointment at the HTC were included; post-implementation (September 1, 2015-December 30, 2015) included patients seen at the HTC who chose to participate in the program for at least 3 months. Before implementation, patient-completed bleeding logs were scanned into the EHR by clinic staff. After implementation, bleeding logs were completed by a pharmacist and documented using a case management tool in the integrated EHR. Bleeding log records successfully documented in the EHR were collected. Completeness was calculated based on 10 clinical data elements for each bleeding log record. Pharmacist-driven interventions resulting from the program in the post-implementation period were recorded. RESULTS: In the pre-implementation period, 19 of 117 bleeding log records (16.2%) were documented in the EHR; all 15 (100%) records were documented post-implementation (P < 0.001). Among all clinical data elements across all records, 706 of 1,170 data elements were recorded pre-implementation (60.3%), and 120 of 150 (80.0%) were recorded post-implementation (P < 0.001). Pre-implementation, no logs were 100% complete; post-implementation, only 6.7% of logs were fully complete (P = 0.114). For the 15 bleeding log records documented in the EHR during the post-implementation period, 14 documented pharmacist-driven clinical interventions occurred. The majority of interventions fell under coordination of care (8 [57.1%]). CONCLUSIONS: Improvement in bleeding log completeness and documentation in the EHR was associated with the use of an EHR tool and pharmacist-driven monitoring program. DISCLOSURES: Not outside funding supported this study. The authors have nothing to disclose.

Cyclosporine or steroids as an adjunct to plasma exchange in the treatment of immune-mediated thrombotic thrombocytopenic purpura.

Although steroids are routinely used as an adjunct to plasma exchange (PEX) therapy in the treatment of immune-mediated thrombotic thrombocytopenic purpura (iTTP), limited data regarding their efficacy or effect on ADAMTS13 biomarkers are available. We report the results of a prospective, randomized study that compared the effectiveness of prednisone or cyclosporine (CSA) as adjuncts to PEX in the treatment of iTTP. A total of 26 of the planned 72 subjects were enrolled and treated from November 2007 until February 2014 before the study was halted after a planned interim analysis. Fourteen patients were randomly assigned to the prednisone arm, and 12 to the CSA arm of the study. One patient died in each arm of the study, and 2 patients in the prednisone arm of the study failed to achieve a response and crossed over to the CSA arm, leaving 11 patients in each arm of the study evaluable for the primary end point of exacerbation. One of the 11 prednisone-treated subjects (9%) suffered an exacerbation, whereas 3 of the 11 (27%) patients in the CSA arm suffered an exacerbation. Although there was no significant difference in the exacerbation rate, suppression of the anti-ADAMTS13 antibodies and improvement in ADAMTS13 activity in the first month after stopping PEX were significantly better in the prednisone-treated subjects. Side effects were manageable and comparable in both arms of the study. These data demonstrate the superiority of prednisone over CSA as an adjunct to PEX in the suppression of the anti-ADAMTS13 antibodies and improvement in ADAMTS13 activity. This trial was registered at www.clinicaltrials.gov as #NCT00713193.