Vol 52, No 1 (2021)
Original research article
Published online: 2021-02-26

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Neurological presentation predicting immune thrombotic thrombocytopenic purpura outcome

Shaimaa El-Ashwah1, Esraa Jamal1, Rasha Samir Shemies2, Metwaly Ibrahim Mortada3, Mayada A. Ghannam3, Yasmine Shaaban1
Acta Haematol Pol 2021;52(1):48-53.


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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