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Thrombotic Thrombocytopenic Purpura
[in Japanese]
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Isonishi Ayami
,
Hayakawa Masaki
,
Matsumoto Masanori
,
Yagi Hideo
,
Fujimura Yoshihiro
… Since the discovery of ADAMTS13, a von Willebrand factor-cleaving protease, it has been well established that TTP lacks the ADAMTS13 activity due to its gene mutations (termed Upshaw-Schulman syndrome, USS) or acquired auto-antibodies. … Recent studies have further indicated that approximately 20% of patients with acquired TTP have resistance to PE therapy, for whom a diagnosis of 'relapsing or intractable' TTP was made. …
Japanese journal of apheresis 31(1), 7-13, 2012-02-29
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