Abstract
Immune-mediated thrombotic thrombocytopenic purpura (iTTP) is a rare life-threatening thrombotic microangiopathy requiring urgent therapeutic plasma exchange (TPE). However, the exact impact of a slight delay in TPE initiation on the subsequent patients' outcome is still controversial. We aimed to study the frequency, short-term neurological consequences, and determinants of diagnostic delay in iTTP. We conducted a retrospective monocentric study including patients with a first acute episode of iTTP (2005-2020) classified into 2 groups: delayed (>24h from first hospital visit, group 1) and immediate diagnosis (≤24h, group 2). Among 42 evaluated patients, 38 were included. Eighteen cases (47%) had a delayed diagnosis (median: 5 days). The main misdiagnosis was immune thrombocytopenia (67%). The mortality rate was 5% (1 death in each group). Neurological events (stroke/TIA, seizure, altered mental status) occurred in 67% vs 30% patients in group 1 and 2, respectively (p = 0.04). Two patients in group 1 exhibited neurological sequelae. The hospital length of stay was longer in group 1 (p = 0.02). At the first hospital evaluation, potential alternative causes of thrombocytopenia were more prevalent in group 1 (33% vs 5%, p = 0.04). Anemia was less frequent in group 1 (67% vs 95%, p = 0.04). All patients had undetectable haptoglobin levels. By contrast, 26% of schistocytes counts were <1%, mostly in group 1 (62% vs 11%, p = 0.01). Diagnostic delay is highly prevalent in iTTP, with a significant impact on short-term neurological outcome. In patients with profound thrombocytopenia, the thorough search for signs of incipient organ dysfunction, systematic hemolysis workup, and proper interpretation of schistocytes count are the key elements of early diagnosis of TTP.
Highlights
At the first hospital evaluation, potential alternative causes of thrombocytopenia were more prevalent in group 1 (33% vs 5%, p = 0.04)
Diagnostic delay is highly prevalent in Immune-mediated thrombotic thrombocytopenic purpura (iTTP), with a significant impact on short-term neurological outcome
In patients with profound thrombocytopenia, the thorough search for signs of incipient organ dysfunction, systematic hemolysis workup, and proper interpretation of schistocytes count are the key elements of early diagnosis of TTP
Summary
Immune-mediated thrombotic thrombocytopenic purpura (iTTP) is a thrombotic microangiopathy (TMA), a heterogeneous group of rare acute diseases characterized by peripheral thrombocytopenia, mechanical hemolytic anemia, and ischemic organ manifestations [1]. iTTP results from severe acquired ADAMTS13 (a Disintegrin And Metalloproteinase with ThromboSpondin-1 motifs, 13th member) deficiency, which leads to the accumulation of large Von Willebrand factor multimers, microthrombi formation, ischemic organ dysfunction, and hemolysis. Immune-mediated thrombotic thrombocytopenic purpura (iTTP) is a thrombotic microangiopathy (TMA), a heterogeneous group of rare acute diseases characterized by peripheral thrombocytopenia, mechanical hemolytic anemia, and ischemic organ manifestations [1]. Only 5% of the patients exhibit all 5 signs initially [3]. Delayed TPE initiation has been associated with slower response to therapy and increased mortality in TMA as a whole [9, 10], but little data are available regarding iTTP itself. Two recent multicenter studies suggested that a slightly delayed diagnosis had no significant impact on mortality, but data regarding neurological outcomes are limited [11, 12]. Immune-mediated thrombotic thrombocytopenic purpura (iTTP) is a rare life-threatening thrombotic microangiopathy requiring urgent therapeutic plasma exchange (TPE). The exact impact of a slight delay in TPE initiation on the subsequent patients’ outcome is still controversial
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