Caramazza et al proposed an algorithm for management of patients with thrombotic thrombocytopenic purpura or hemolytic uremic syndrome (TTP-HUS). This involves use of rituximab for patients with refractory or relapsing disease. The authors are from Universitaria Policlinico "Paolo Glaccone" di Palermo in Italy.


Patient selection: high suspicion of idiopathic TTP or HUS. A patient with a diagnosis other than TTP-HUS should not receive the protocol.


Initial protocol:

(1) The patient receives a plasma infusion or plasma exchange within 24 hours of diagnosis.

(2) The patient undergoes plasma exchange for 7 to 14 days.


A patient who responds to the initial protocol:

(1) The patient undergoes consolidation with plasma exchange until a remission is achieved.

(2) Plasma exchange is discontinued and the patient is monitored for relapse.


A patient who does not respond to the plasma exchange protocol is deemed refractory:

(1) The patient is started on rituximab (375 mg per square meter BSA infused once a week for 4 weeks).

(2) If no response is achieved then an alternative therapy involving experimental drugs is started.

(3) If the patient responds to rituximab then it is stopped and the patient is monitored for relapse.


Monitoring protocol:

(1) complete blood count (platelet count, hemoglobin)

(2) serum LDH

(3) serum ADAMTS-13 activity

(4) IgG anti-ADAMTS 13 titer


The protocol is restarted if relapse occurs.


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