Hemostasis

Thrombotic Thrombocytopenic Purpura: A Diagnostic Case

A clinical case of thrombotic thrombocytopenic purpura: schistocytes in a blood smear, hemolysis, thrombocytopenia, and ADAMTS-13 deficiency.

⚠A case of classic thrombotic thrombocytopenic purpura (TTP). ❗A 44-year-old woman is admitted to the emergency department with confused consciousness and a hemorrhagic rash. 🩸In peripheral blood: ✅Hemoglobin 82 g/L, ✅Platelets 12 ×10^9/L. (Normal values were 3 months ago.) ✅Reticulocytes 10% (N=0.2-2), ✅Haptoglobin <10 mg/dL (N=30-200), ✅LDH 695 U/L (N=0-247), indicating hemolysis. ✅Creatinine is normal. ✅Coombs test is negative. ✅Coagulation parameters are normal. 🔬Blood smear reveals characteristic erythrocyte fragments (schistocytes), which was a decisive factor in the diagnosis of TTP and urgent initiation of therapy. ‼Analysis of metalloprotease ADAMTS-13 shows its zero activity and the presence of an inhibitor! 👉Deficiency of this enzyme leads to insufficient cleavage of von Willebrand factor macromolecules, which promotes platelet aggregation and microthrombus formation in capillaries, damage to the vascular wall, and mechanical destruction of erythrocytes in the bloodstream (intravascular hemolysis). 🙏We thank Dr. Anna Belen from Israel for providing the clinical case and presentation photos from the “five-minute meeting”.