In:
PLOS ONE, Public Library of Science (PLoS), Vol. 16, No. 11 ( 2021-11-19), p. e0260196-
Kurzfassung:
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. Aim We aimed to study the frequency, short-term neurological consequences, and determinants of diagnostic delay in iTTP. Methods 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). Results 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). Conclusion 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.
Materialart:
Online-Ressource
ISSN:
1932-6203
DOI:
10.1371/journal.pone.0260196
DOI:
10.1371/journal.pone.0260196.g001
DOI:
10.1371/journal.pone.0260196.g002
DOI:
10.1371/journal.pone.0260196.t001
DOI:
10.1371/journal.pone.0260196.t002
DOI:
10.1371/journal.pone.0260196.t003
DOI:
10.1371/journal.pone.0260196.s001
DOI:
10.1371/journal.pone.0260196.s002
DOI:
10.1371/journal.pone.0260196.s003
DOI:
10.1371/journal.pone.0260196.r001
DOI:
10.1371/journal.pone.0260196.r002
DOI:
10.1371/journal.pone.0260196.r003
DOI:
10.1371/journal.pone.0260196.r004
DOI:
10.1371/journal.pone.0260196.r005
DOI:
10.1371/journal.pone.0260196.r006
Sprache:
Englisch
Verlag:
Public Library of Science (PLoS)
Publikationsdatum:
2021
ZDB Id:
2267670-3