open access

Vol 52, No 1 (2021)
Original research article
Submitted: 2021-02-01
Accepted: 2021-02-01
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Neurological presentation predicting immune thrombotic thrombocytopenic purpura outcome

Shaimaa El-Ashwah, Esraa Jamal, Rasha Samir Shemies, Metwaly Ibrahim Mortada, Mayada A. Ghannam, Yasmine Shaaban
DOI: 10.5603/AHP.2021.0007
·
Acta Haematol Pol 2021;52(1):48-53.

open access

Vol 52, No 1 (2021)
ORIGINAL RESEARCH ARTICLE
Submitted: 2021-02-01
Accepted: 2021-02-01

Abstract

Introduction: Immune thrombotic thrombocytopenic purpura (iTTP) is a rare disorder caused by acquired autoantibodies to a disintegrin and metalloprotease with thrombospondin-1 motifs (ADAMTS-13) that normally cleaves von Willebrand factor macromolecules. It is manifested by microangiopathic hemolytic anemia, systemic microvascular thrombi formation, and subsequent end-organ ischemia (renal and neurological manifestations). Early diagnosis and management resulted in improving the survival rate. This is a retrospective study conducted to describe the clinical characteristics of patients diagnosed with iTTP, their survival, and prognostic factors affecting it.

Methods: We included adult patients who met the diagnostic criteria of iTTP between 2016 and 2019. Based on PLASMIC Score for TTP, our patients ranged from 6 to 7. ADAMTS-13 testing was not done because of financial issues.

Results: A total of 21 patients were included in this study. The median age of the studied patients was 30.45 years, and 81% of them were female. The most common clinical feature was fever (57.1%), followed by bleeding manifestations (52.4%), neurological manifestations (47.6%), renal impairment (42.9%), and cardiac manifestations (9.5%). There were a total of 4 deaths (19.04%). The overall survival was correlated significantly with neurological manifestations and PLASMIC scores (p = 0.02, 0.012, respectively).

Conclusions: Our report reinforces that iTTP is not mandatory to be presented with classic pentad. Using PLASMIC score could help in the diagnosis and prediction of survival, and we strongly suggest that the absence of neurological manifestations  results in better overall survival. Therapeutic plasma exchange should be started as soon as possible once iTTP is suspected. Rituximab has an important role in improving treatment outcomes.

Abstract

Introduction: Immune thrombotic thrombocytopenic purpura (iTTP) is a rare disorder caused by acquired autoantibodies to a disintegrin and metalloprotease with thrombospondin-1 motifs (ADAMTS-13) that normally cleaves von Willebrand factor macromolecules. It is manifested by microangiopathic hemolytic anemia, systemic microvascular thrombi formation, and subsequent end-organ ischemia (renal and neurological manifestations). Early diagnosis and management resulted in improving the survival rate. This is a retrospective study conducted to describe the clinical characteristics of patients diagnosed with iTTP, their survival, and prognostic factors affecting it.

Methods: We included adult patients who met the diagnostic criteria of iTTP between 2016 and 2019. Based on PLASMIC Score for TTP, our patients ranged from 6 to 7. ADAMTS-13 testing was not done because of financial issues.

Results: A total of 21 patients were included in this study. The median age of the studied patients was 30.45 years, and 81% of them were female. The most common clinical feature was fever (57.1%), followed by bleeding manifestations (52.4%), neurological manifestations (47.6%), renal impairment (42.9%), and cardiac manifestations (9.5%). There were a total of 4 deaths (19.04%). The overall survival was correlated significantly with neurological manifestations and PLASMIC scores (p = 0.02, 0.012, respectively).

Conclusions: Our report reinforces that iTTP is not mandatory to be presented with classic pentad. Using PLASMIC score could help in the diagnosis and prediction of survival, and we strongly suggest that the absence of neurological manifestations  results in better overall survival. Therapeutic plasma exchange should be started as soon as possible once iTTP is suspected. Rituximab has an important role in improving treatment outcomes.

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Keywords

ADAMTS13, rituximab, thrombotic microangiopathy, thrombotic thrombocytopenic purpura

About this article
Title

Neurological presentation predicting immune thrombotic thrombocytopenic purpura outcome

Journal

Acta Haematologica Polonica

Issue

Vol 52, No 1 (2021)

Article type

Original research article

Pages

48-53

DOI

10.5603/AHP.2021.0007

Bibliographic record

Acta Haematol Pol 2021;52(1):48-53.

Keywords

ADAMTS13
rituximab
thrombotic microangiopathy
thrombotic thrombocytopenic purpura

Authors

Shaimaa El-Ashwah
Esraa Jamal
Rasha Samir Shemies
Metwaly Ibrahim Mortada
Mayada A. Ghannam
Yasmine Shaaban

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