Thrombotic thrombocytopenic purpura misdiagnosed as autoimmune cytopenia: Causes of diagnostic errors and consequence on outcome. Experience of the French thrombotic microangiopathies reference centre.
Maximilien GrallElie AzoulayLionel GalicierFrançois ProvôtAlain WynckelPascale PoullinSteven GrangeJean-Michel HalimiAlexandre LautretteYahsou DelmasClaire PresneMohamed HamidouStéphane GiraultFrédéric PènePierre PerezTarik KanouniAmélie SeguinChristiane MoussonDominique ChauveauMario Ojeda-UribeVirginie BarbayAgnès VeyradierPaul CoppoYgal BenhamouPublished in: American journal of hematology (2017)
Thrombotic thrombocytopenic purpura (TTP) has a devastating prognosis without adapted management. Sources of misdiagnosis need to be identified to avoid delayed treatment. We studied 84 patients with a final diagnosis of severe (<10%) acquired ADAMTS13 deficiency-associated TTP from our National database that included 423 patients, who had an initial misdiagnosis (20% of all TTP). Main diagnostic errors were attributed to autoimmune thrombocytopenia, associated (51%) or not (37%) with autoimmune hemolytic anemia. At admission, misdiagnosed patients were more frequently females (P = .034) with a history of autoimmune disorder (P = .017) and had organ involvement in 67% of cases; they had more frequently antinuclear antibodies (P = .035), a low/undetectable schistocyte count (P = .001), a less profound anemia (P = .008), and a positive direct antiglobulin test (DAT) (P = .008). In multivariate analysis, female gender (P = .022), hemoglobin level (P = .028), a positive DAT (P = .004), and a low schistocytes count on diagnosis (P < .001) were retained as risk factors of misdiagnosis. Platelet count recovery was significantly longer in the misdiagnosed group (P = .041) without consequence on mortality, exacerbation and relapse. However, patients in the misdiagnosed group had a less severe disease than those in the accurately diagnosed group, as evidenced by less organ involvement at TTP diagnosis (P = .006). TTP is frequently misdiagnosed with autoimmune cytopenias. A low schistocyte count and a positive DAT should not systematically rule out TTP, especially when associated with organ failure.
Keyphrases
- end stage renal disease
- chronic kidney disease
- multiple sclerosis
- risk factors
- ejection fraction
- newly diagnosed
- drug induced
- emergency department
- peripheral blood
- intensive care unit
- type diabetes
- adverse drug
- mental health
- patient safety
- patient reported outcomes
- autism spectrum disorder
- coronary artery disease
- intellectual disability
- patient reported
- electronic health record