Immune thrombocytopenia (ITP) World Impact Survey (I-WISh): Impact of ITP on health-related quality of life.

Immune thrombocytopenia (ITP) has a substantial, multifaceted impact on patients' health-related quality of life (HRQoL). Data describing which aspects of ITP physicians and patients perceive as having the greatest impact are limited. The ITP World Impact Survey (I-WISh) was a cross-sectional survey, including 1507 patients and 472 physicians, to establish the impact of ITP on HRQoL and productivity from patient and physician perspectives. Patients reported that ITP reduced their energy levels (85% of patients), capacity to exercise (77%), and limited their ability to perform daily tasks (75%). Eighty percent of physicians reported that ITP symptoms reduced patient HRQoL, with 66% reporting ITP-related fatigue substantially reduced patient HRQoL. Patients believed ITP had a substantial impact on emotional well-being (49%) and 63% worried their condition would worsen. Because of ITP, 49% of patients had already reduced, or seriously considered reducing their working hours, and 29% had considered terminating their employment. Thirty-six percent of patients employed at the time of the survey felt ITP decreased their work productivity, while 51% of patients with high/very high symptom burden reported that ITP affected their productivity. Note, I-WISh demonstrated substantive impact of ITP on patients' HRQoL both directly for patients and from the viewpoint of their physicians. Patients reported reduced energy levels, expressed fears their condition might worsen, and those who worked experienced reduced productivity. Physicians should be aware not only of platelet counts and bleeding but also the multi-dimensional impact of ITP on patients' lives as an integral component of disease management.

Immune thrombocytopenia (ITP) World Impact Survey (iWISh): Patient and physician perceptions of diagnosis, signs and symptoms, and treatment.

Immune thrombocytopenia (ITP) is now well-known to reduce patients' health-related quality of life. However, data describing which signs and symptoms patients and physicians perceive as having the greatest impact are limited, as is understanding the full effects of ITP treatments. I-WISh (ITP World Impact Survey) was an exploratory, cross-sectional survey designed to establish the multifaceted impact of ITP, and its treatments, on patients' lives. It focused on perceptions of 1507 patients and 472 physicians from 13 countries regarding diagnostic pathway, frequency and severity of signs and symptoms, and treatment use. Twenty-two percent of patients experienced delayed diagnosis (caused by several factors), 73% of whom felt anxious as a result. Patients rated fatigue among the most frequent, severe symptom associated with ITP at diagnosis (58% most frequent; 73% most severe), although physicians assigned it lower priority (30%). Fatigue was one of the few symptoms persisting at survey completion (50% and 65%, respectively) and was the top symptom patients wanted resolved (46%). Participating physicians were experienced at treating ITP, thereby recognizing the need to limit corticosteroid use to newly-diagnosed or first-relapse patients and espoused increased use of thrombopoietin receptor agonists and anti-CD20 after relapse in patients with persistent/chronic disease. Patient and physicians were largely aligned on diagnosis, symptoms, and treatment use. I-WISh demonstrated that patients and physicians largely align on overall ITP symptom burden, with certain differences, for example, fatigue. Understanding the emotional and clinical toll of ITP on the patient will facilitate shared decision-management, setting and establishment of treatment goals and disease stage-appropriate treatment selection.

Cognitive symptoms are common in immune thrombocytopenia and associate with autonomic symptom burden.

OBJECTIVES: People with adult immune thrombocytopenia (ITP) are commonly thought to have an isolated blood disorder, but many also describe memory and concentration problems. Cognitive impairment commonly associates with autonomic dysfunction. Here, we quantified cognitive symptoms in a large cohort of patients with ITP compared with controls and explored the relationship with autonomic symptoms. METHODS: Patients with ITP were approached in the UK via the national ITP Support Association and invited to complete Composite Autonomic Symptom Scale (COMPASS; measure of autonomic symptoms) and Cognitive Failures Questionnaire (CFQ) together with one from a friend of similar age and sex without ITP. RESULTS: Three hundred and ninety-eight patients with ITP completed measures with paired data from a representative group of 189 patients and controls (47%). Both autonomic and cognitive symptom burden were higher in ITP compared with controls (COMPASS score (48 ± 14 vs. 38 ± 12; P > 0.0001); CFQ (43 ± 17 vs. 36 ± 13; P < 0.0001). A positive relationship was seen between increasing cognitive symptoms and higher COMPASS score (P < 0.0001; r(2) = 0.1). Increasing cognitive symptoms did not associate with platelet count (P = 0.08, r(2) = 0.008). Multivariate analysis confirmed age and COMPASS independently associated with higher CFQ but not platelet count. CONCLUSION: Immune thrombocytopenia is more than a bleeding disorder; cognitive symptoms are common and independently associate with autonomic symptoms but not disease severity.

Fatigue in adult patients with primary immune thrombocytopenia.

BACKGROUND: Patients with primary immune thrombocytopenia (ITP) commonly describe symptoms of fatigue. However, hematologists rarely consider fatigue a manifestation of ITP. OBJECTIVES: To document the prevalence of fatigue among patients with ITP and to determine the patient characteristics that are associated with fatigue. Using a cross-sectional design, we surveyed 1871 members of the UK ITP Support Association [585 (31%) responded], and 93 patients enrolled in the Oklahoma (US) ITP Registry [68 (73%) responded] with questions about their ITP and with validated symptom assessment scales for fatigue, daytime sleepiness, and orthostatic symptoms. RESULTS: The prevalence of fatigue among both UK (39%) and US (22%) patients was significantly greater than expected compared with normal subjects (P<0.0001 and P<0.0001 respectively). In univariate analysis of the combined cohorts, fatigue was associated with a platelet count <100000/µL, treatment with steroids, bleeding symptoms, presence of other medical conditions, daytime sleepiness, and orthostatic symptoms. Fatigue was not associated with age, gender, duration of ITP, or splenectomy status. Multivariate analysis of the combined cohorts was stratified for the presence or absence of bleeding symptoms. Among 107 patients with bleeding symptoms, fatigue was independently associated with a platelet count <100000/µL and female gender. Among 491 patients without bleeding symptoms, fatigue was independently associated with a platelet count <30000/µL, presence of other medical conditions, daytime sleepiness, and orthostatic symptoms. CONCLUSIONS: Fatigue is a common symptom among patients with ITP. These data provide the basis for future studies to define the clinical importance of fatigue in ITP.

International consensus report on the investigation and management of primary immune thrombocytopenia.

Previously published guidelines for the diagnosis and management of primary immune thrombocytopenia (ITP) require updating largely due to the introduction of new classes of therapeutic agents, and a greater understanding of the disease pathophysiology. However, treatment-related decisions still remain principally dependent on clinical expertise or patient preference rather than high-quality clinical trial evidence. This consensus document aims to report on new data and provide consensus-based recommendations relating to diagnosis and treatment of ITP in adults, in children, and during pregnancy. The inclusion of summary tables within this document, supported by information tables in the online appendices, is intended to aid in clinical decision making.