PHYSICIANS’ PERCEPTIONS ON CAUSES OF PRIMARY AND SECONDARY ITP AND LEADING CAUSES OF MISDIAGNOSIS: RESULTS FROM THE ITP WORLD IMPACT SURVEY (I-WISH)
Author(s): ,
James B Bussel
Affiliations:
Division of Hematology/Oncology,Weill Cornell Medical College,New York,United States
,
Yoshiaki Tomiyama
Affiliations:
Osaka University Hospital,Osaka,Japan
,
Marc Michel
Affiliations:
Henri Mondor University Hospital,Créteil,France
,
Drew Provan
Affiliations:
Barts and The London School of Medicine and Dentistry,London,United Kingdom
,
Ming Hou
Affiliations:
Department of Hematology,Shandong University,Jinan,China
,
Cristina Santoro
Affiliations:
Hematology, Department of Translational and Precision Medicine,Sapienza University,Rome,Italy
,
Alexandra Kruse
Affiliations:
Platelet Disorder Support Association,Cleveland,United States
,
Caroline Kruse
Affiliations:
Platelet Disorder Support Association,Cleveland,United States
,
Mervyn Morgan
Affiliations:
ITP Support Association,Bolnhurst,United Kingdom
,
Barbara Lovrencic
Affiliations:
Italian Association Immune Thrombocytopenic Purpura,Caprino Veronese,Italy
,
Tom Bailey
Affiliations:
Bespoke Team,Adelphi Real World,Macclesfield,United Kingdom
,
Miona Stankovic
Affiliations:
Novartis,Basel,Switzerland
Waleed Ghanima
Affiliations:
Department of Medicine,Ostfold Hospital Trust,Kalnes,Norway
EHA Library. Bussel J. Jun 14, 2019; 266511; PF712
Dr. J Bussel
Dr. J Bussel
Contributions
Abstract

Abstract: PF712

Type: Poster Presentation

Presentation during EHA24: On Friday, June 14, 2019 from 17:30 - 19:00

Location: Poster area

Background
Immune thrombocytopenia (ITP) is a heterogeneous syndrome with variable clinical manifestations across patients (pts), which makes diagnosis challenging and requires tailored investigations. There is a need for improved accuracy and novel testing in diagnosing ITP and better use of existing tests, e.g. reticulated platelets, platelet antibodies.

Aims
I-WISh studied the burden of ITP and its impact on quality of life using a global pt and physicians sampling frame. This analysis reports physicians’ perspectives on the difficulties inherent in ITP diagnosis.

Methods
I-WISh is a cross-sectional survey of 1507 ITP pts and 472 physicians across 13 countries. Participants (pts and physicians) completed a 30-minute online survey that included demographics, signs and symptoms, impact of symptoms, and pt–physician relationships. A steering committee of expert physicians and pt advocacy ITP specialists designed and endorsed the survey materials. All physician-reported data were collected from a single physician survey, which asked physicians about their experience and perceptions of managing ITP patients. Physicians assigned a rank of 1, 2, or 3 from a list of 15 conditions that could be misdiagnosed as ITP.

Results
472 physicians with a mean number of ITP pts (SD) of 34 (50) and mean (SD) of 18 (36) newly diagnosed pts in the past year completed the survey. According to physicians’ recollections, 72% of ITP pts have primary ITP and the remaining 28% have secondary ITP. The survey revealed that exclusion of other disorders was perceived as the leading cause of delays in making an accurate diagnosis of ITP (68%). 53% of physicians indicated misdiagnosis as a contributing factor in delayed diagnosis, with 71% reporting that pts were misdiagnosed 1–25% of the time. Other contributors to delayed diagnosis were access to diagnostic examination (55%) and time to refer pts to a specialist (58%). Out of 441 responses, causes of secondary ITP featured in the top 3 were systemic lupus erythematosus (46%), drug-induced thrombocytopenia (DIT; 46%), chronic lymphocytic leukemia (CLL; 45%), antiphospholipid syndrome (30%), hepatitis C virus (26%), autoimmune lymphoproliferative syndrome (post-treatment) (ALPS; 23%), Helicobacter pylori infection (20%), Evans syndrome (17%), and HIV (13%) (Fig). DIT (67%) was the most frequent condition misdiagnosed as ITP, followed by aplastic anemia/myelodysplastic syndromes (MDS; 49%), liver disease (46%), hypersplenism (38%), and leukemia (29%) (Fig). 18% of pts reported being first diagnosed with another condition.

Conclusion
Accurate diagnosis of ITP is difficult. No formula exists allowing accurate diagnosis in all locations and no testing is universally considered to be specific or sensitive. Secondary ITP diagnosis in as many as 28% of ITP pts adds to the difficulties of diagnosing primary ITP; this number is ~10% higher than previous estimates (Cines et al. Hematol Oncol Clin North Am 2009;129:818). Given that the only positive diagnosis of ITP is response to ITP-specific treatment, this would nonetheless not exclude secondary ITP. Surprisingly, the leading misdiagnosis being DIT highlights the need for better testing of DIT (currently unavailable anywhere) and the need to replace all medications the patient is taking at diagnosis. Liver disease needs to be suspected early, and testing for this could include ultrasonography (Rajan et al. Brit J Haematol 2005;23:1155). The high rate of secondary ITP and absence of specific testing for ITP contribute to the difficulties with diagnosis.

Session topic: 32. Platelets disorders

Keyword(s): Diagnosis, Immune thrombocytopenia (ITP)

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