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Thrombotic thrombocytopenic purpura misdiagnosed as autoimmune cytopenia: Causes of diagnostic errors and consequence on outcome. Experience of the French thrombotic microangiopathies reference centre
Author(s) -
Grall Maximilien,
Azoulay Elie,
Galicier Lionel,
Provôt François,
Wynckel Alain,
Poullin Pascale,
Grange Steven,
Halimi JeanMichel,
Lautrette Alexandre,
Delmas Yahsou,
Presne Claire,
Hamidou Mohamed,
Girault Stéphane,
Pène Frédéric,
Perez Pierre,
Kanouni Tarik,
Seguin Amélie,
Mousson Christiane,
Chauveau Dominique,
OjedaUribe Mario,
Barbay Virginie,
Veyradier Agnès,
Coppo Paul,
Benhamou Ygal
Publication year - 2017
Publication title -
american journal of hematology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 2.456
H-Index - 105
eISSN - 1096-8652
pISSN - 0361-8609
DOI - 10.1002/ajh.24665
Subject(s) - medicine , thrombotic thrombocytopenic purpura , cytopenia , schistocyte , adamts13 , autoimmune hemolytic anemia , exacerbation , gastroenterology , evans syndrome , hemolytic anemia , anemia , autoimmune thrombocytopenia , anti nuclear antibody , autoantibody , immunology , platelet , bone marrow , antibody
Abstract 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.