Sun-386 Thrombotic Thrombocytopenic Purpura in a Patient With Beta Thalassemia Trait: A Case Report

Abstract

is a life threatening condition characterized by pentad of microangiopathic hemolytic anemia, thrombocytopenia, fever, neurological manifestations and renal disease. It is related to deficiency of ADAMTS13 (A Disintegrin and Metalloproteinase with Thrombospondin type 1 motif, member 13) which is Von Willebrand factor cleaving protease. The deficiency of ADAMTS13 enzyme occurs mostly due to autoantibodies. However, it may also occur in inherited form due to mutations at gene level. There have been few reported cases of thrombotic thrombocytopenic purpura occurring in sickle cell/b +-thalassemia patients. Methods: Male patient 47 years old with no past history of any medical condition and no history of any drug intake. His condition started with attacks of headache and vomiting followed by gross hematuria then rash on both lower limbs. His laboratory investigations showed urine red blood cells over 200, microcytic hypochromic anemia with red blood cell fragments and moderate degree of polychromasia, thrombocytopenia, mild renal impairment with serum creatinine level 1.4. Transferrin Saturation was normal 48%, elevated lactate dehrogenase 1520, negative coomb's test, negative antinuclear antibodies. ADAMTS13 activity wasn't available at time of diagnosis but later it was ordered and showed decreased activity. His hemoglobin electrophoresis showed increased HBA2 level which is consistent with beta thalassemia trait. He received 3 pulse solumedrol (.5 gm) and 7 sessions' plasmapharesis. His condition improved temporarily, one week later he came with a relapse. He received 6 sessions' plasmapheresis. Results: He showed improvement and he is in remission till now. He was on solupred 60 mg and cyclosporine 200 mg daily and then tapered gradually. He is now on cyclosporine 50 mg once daily and folic acid. Conclusions: The association of anemia with thrombocytopenia in a patient with beta thalassemia trait should raise suspicion of thrombotic thrombocytopenic purpura. To our knowledge this is the first reported case of thrombotic thrombocytopenic purpura in a patient with beta thalassemia trait.