PMC3719105_01
Peripheral smear showing plenty of schistocytes (indicated by arrow), polychromasia and markedly decreased platelets.
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Polymerase chain reaction. Peripheral smear showed evidence of microangiopathic hemolysis with plenty of schistocytes, polychromasia, nucleated red blood cells and severe thrombocytopenia (Figure 1).
Time course of levels of ADAM TS 13 (ng/mL) and antibody to ADAM TS 13 (BEU) in relation to treatment with weekly Rituximab.
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Assay of vWF protease activity using the Technozym ADAMTS 13 ELISA chromogenic test revealed a severe deficiency (Figure 2) with a value of less than 47 ng/mL which corresponded to less than 9% of the normal activity (normal value more than 530 ng/mL) and presence of vWF inhibitor with a value of 1.8 (normal value less than 0.4 Bethesda Equivalent Units, BEU). Platelet count increased and was 80,000/mL (Figure 2) by the 10th day of treatment.