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5. All of the above
The presence of schistocytes suggests a microangiopathic process with intravascular hemolysis. In this clinical situation this suggests disseminated intravascular coagulation (DIC) or thrombotic thrombocytopenic purpura (TTP). The pathogenesis in DIC is a thrombin excess with evidence of diffuse coagulation (elevated PT, PTT, fibrin degradation products, D-dimers but decreased fibrinogen). In contrast, the pathogenesis in TTP is an endothelial defect and most of the parameters that are grossly abnormal in DIC are either normal or only slightly abnormal. Additional diagnoses that should be considered in patients presenting with an acute thrombotic microangiopathy (TMA), especially in those in whom levels of ADAMTS13 (a disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13) activity are nondeficient (>10%) including drug-induced thrombotic microangiopathy (4).
Our patient had an ADAMTS13 above 10% (40%), a normal fibrinogen level but a slightly elevated tacrolimus level of 16.2 ng/ml (usual preferred range 5-15 ng/ml).
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