Abstract:

Objective To improve the knowledge of the acquired thrombotic thrombocytopenic purpura (TTP) in children. Methods The clinical data of the acquired TTP case without nervous system involvement were summarized and related literatures were reviewed. Results The 12-year-old boy was admitted to our hospital because of fever and rash on the lower limbs as the first symptom. The blood test showed anemia, thrombocytopenia and blood smear showed erythroclasis, while the urinalysis showed microscopic haematuria. The blood biochemistry showed elevated bilirubin and LDH levels. However, renal function and complement level were normal. Thrombotic microangiopathy (aHUS or TTP) was considered. In further differential diagnosis, his ADAMTS13 activity showed 5% below , with the presence of ADAMTS13 antibodies. The final diagnosis was acquired TTP. Plasma exchange plus prednisolone were carried out. The patient recovered absolutely, and all the medication was stopped after 4 months. There was no relapse during the six months follow-up. After a systematic analysis of the ChianInfo, CNKI and pubmed. A total of 40 TTP patients were analyzed from 14 articals, The mean age of patients was (10.2±5.2) years old. The study included 19 men and 21 women. All patients presented with anemia and thrombocytopenia, 36 cases with fever (90%), 28 cases (70%) with nervous system involvement, 18 cases (45%) with renal involvement. During the acute phase,3 patients died and 37 patients were treated with the plasma exchange plus glucocorticoid, 31 patients (83.8%) clinical symptoms were quickly improved with plasma exchange plus glucocorticoid, while 6 had other complications were further treated with RTX. The mean follow-up time was 29 months (3-72 months) with 4 lost cases (10.8%). Within the follow-up period, 13 (39.4%) patients suffered relapses. 9 cases were given a further treatment of RTX , while 2 of them still suffered relapse during RTX treatment. Conclusion The patients presented with anemia and thrombocytopenia should be considered as TTP. Monitoring of the ADAMTS13 activity and anti-ADAMTS13 antibody plays an important role in diagnosis and differential diagnosis in TTP. Plasma exchange plus glucocorticoid or rituximab can be considered as the first line treatment.