Thrombotic thrombocytopenic purpura classification

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Sogand Goudarzi, MD [2]

Overview

TTP may be classified according to ADAMTS13 gene mutations and autoantibody against ADAMTS13 into two subtypes: herditary syndromes, aquired syndromes.

Classification

TTP may be classified into several subtypes based on ADAMTS13 gene mutations(herditary syndromes) and autoantibody against ADAMTS13(aquired syndromes):^[1]^[2]

1.Hereditary:

Congenital TTP
Inherited TTP
Familial TTP
Upshaw-Schulman syndrome (USS) is an autosomal recessive disease of ADAMTS13 gene on chromosome 9q34 .

2.Acquired

Existence of an inhibitory antibody against ADAMTS13 due to the variety of conditions.

References

↑ Tersteeg C, Verhenne S, Roose E, Schelpe AS, Deckmyn H, De Meyer SF, Vanhoorelbeke K (2016). “ADAMTS13 and anti-ADAMTS13 autoantibodies in thrombotic thrombocytopenic purpura – current perspectives and new treatment strategies”. Expert Rev Hematol. 9 (2): 209–21. doi:10.1586/17474086.2016.1122515. PMID 26581428.
↑ Yoshihiro Fujimura, Masanori Matsumoto, Hideo Yagi, Akira Yoshioka, Taei Matsui & Koiti Titani (2002). “Von Willebrand factor-cleaving protease and Upshaw-Schulman syndrome”. International journal of hematology. 75 (1): 25–34. PMID 11843286. Unknown parameter |month= ignored (help)

Overview