Abstract Number: PB1867
Meeting: ISTH 2020 Congress
Background: Thrombotic thrombocytopenic purpura(TTP) is a rare thrombotic microangiopathy characterized by severe thrombocytopenia, microangiopathic hemolytic anemia, and organ ischemia ascribable to platelet-rich microthrombi. TTP is related to a severe deficiency in ADAMTS13，the specific von Willebrand factor-cleaving metalloprotease.
Aims: To analyze the clinical characteristics, treatment and outcome of patients with TTP.
Methods: We retrospectively analyzed 83 patients with TTP treated in Chinese Academy of Medical Sciences Hematology Hospital from 1998 to 2019.
Results: Among 83 patients, there were 27 males and 56 females, with a median age of 39 (10～68) years. 41 cases showed pentad sign and 79 cases showed triple sign. 78.0% (46 / 59) of the patients had a PLASMIC score of ≥ 6. TTP gene mutation was detected in 5 of 10 patients. The activity of ADAMTS13 was detected in 10 patients before plasma exchange (PEX), 9 patients had an ADAMTS13 activity level ≤10%. 83 patients were treated with PEX/ plasma infusion+glucocorticoid, or combined with rituximab or immunosuppressants. The overall survival rate was 78.3%, the effective rate was 81.9%, the remission rate was 63.9%, and the recurrence rate was 21.7%. There was significant difference in survival rate among different treatment groups. Compare the survival group with the death group, there were significant differences in age, hemoglobin, glutamic pyruvic transaminase / glutamic oxaloacetic transaminase, lactic dehydrogenase, total bilirubin, free hemoglobin, haptoglobin and severe neuropsychiatric symptoms. Age and severe neuropsychiatric symptoms were independent factors affecting the prognosis of TTP.
Conclusions: The diagnosis of TTP needs to be obtained by combining a variety of clinical data. The detection of TTP gene and ADAMTS13 is helpful to the diagnosis of the disease. Plasma therapy combined with glucocorticoid is the first choice, which combined with rituximab and immunosuppressant therapy can reduce mortality. Age and severe neuropsychiatric symptoms contribute to the prediction of clinical outcome.
[Fig1 Treatment and outcome of 83 patients with TTP. PEX, plasma exchange; PI, plasma infusion.]
To cite this abstract in AMA style:
Xiyan W, Xiaofan L, Feng X, Wei L, Yunfei C, Yueting H, Rongfeng F, Lei Z, Renchi Y. Clinical Analysis of 83 Patients with Thrombotic Thrombocytopenic Purpura [abstract]. Res Pract Thromb Haemost. 2020; 4 (Suppl 1). https://abstracts.isth.org/abstract/clinical-analysis-of-83-patients-with-thrombotic-thrombocytopenic-purpura/. Accessed September 29, 2023.« Back to ISTH 2020 Congress
ISTH Congress Abstracts - https://abstracts.isth.org/abstract/clinical-analysis-of-83-patients-with-thrombotic-thrombocytopenic-purpura/