PB0999 - Update in The Management Tools of Thrombotic Thrombocytopenic Purpura(TTP) As One of the Rare Hematological Disorders with A Highlight On the Cost Value of Such Diseases

Background: TTP is a thrombotic microangiopathy (TMA) characterized by microangiopathic hemolytic anemia and thrombocytopenia developed due to deficiency of ADAMTS13 (a disintegrin and metalloprotease with thrombospondin type 1 repeats, member 13). T
the absence of ADAMTS13 activity causes the formation of very large (ultra-large) vWF multimers, which have an increased
tendency to attach to the endothelium.

Aims: Primary objective: To correlate the diagnosis of TTP with the validated TTP scores (PLASMIC, French and Bentley scores)
Secondary objective: To provide an estimated incidence of TTP at Our Center at King Khalid University Hospital .
To evaluate the accuracy and quality of diagnostic work up done for patients with TTP To evaluate the therapeutic options provided for patients with TTP and assess their clinical outcomes.

Methods: Retrospective observation study was conducted in our King Khalid University Hospital.
All patients who received PLEX at KKUH for definite or presumptive diagnosis of TTP between 2013-2021.Hospital’s computer with eSiHi access and Blood bank patients’ record, Target Population / Sample Size (with sample 29) patients.

Results: The clinical judgment for diagnosing Thrombotic thrombocytopenic purpura (TTP) is in concordance with the validated TTP scores at King Khalid University Hospital (KKUH). The extensive review of patients presented to KKUH with TTP diagnosis is likely to provide us with the largest local sample size of eligible patients to compare the clinical outcomes of plasma exchange (PLEX) with the other additional forms of management.

Conclusion(s): In conclusion, a major challenge in diagnosing and managing patients with TTP where a rapid
identification and management initiation has been proven to save patients’ lives. PLEX is the main
curative option for these patients but many challenges face the initiation of PLEX. Here we seek to assess
the local experience and gain more insight around the approach to similar cases in the future