Fig. 1. Current approach for patients with suspected iTTP in Korea. Plasma exchange (PEX) should be initiated at the earliest when throm-botic thrombocytopenic purpura is suspected. Daily PEX can be discontinued once platelet count is normalized for 2 days. A suboptimal response is defined as a lack of platelet count increment after 5 days of PEX or initial im-provement followed by a decrease in platelet count while receiving PEX. Although rituximab treatment for immune-mediated thrombotic thrombocytopenic purpura (iTTP) has not been approved in Korea, the off-label use of rituximab should be considered for refractory iTTP. Currently, caplacizumab is unavailable in Korea. *ADAMTS13 gene sequencing should be considered in patients who have no detectable anti-ADAMTS13 antibodies and persistent severe ADAMTS13 deficiency during clinical remission.

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