title: Does corticosteroid treatment cause prolonged recovery and increased total bilirubin level in severe ADAMTS-13-deficient TTP patient?
abstract: A 41-year-old female patient complaining of fatigue, headache, mild confusion, and rush on her lower extremities was admitted to our emergency department. Laboratory tests revealed that he had anemia, thrombocytopenia, and increased levels of indirect bilirubin and lactic dehydrogenase (LDH) in blood tests. Direct and indirect Coombs tests were negative, and fragmented erythrocytes were observed in peripheral blood smears. The patient was diagnosed with thrombotic thrombocytopenic purpura (TTP). The best supportive care was provided. Therapeutic plasma exchange (TPE) and 1 mg/kg methylprednisolone treatments were administered. On the 10th day of treatment, LDH level and fragmented red blood cells in peripheral blood smear were decreased, but his direct and indirect bilirubin levels increased despite the fact that he was treated with 1 mg/kg methylprednisolone and TPE. The patient had severe ADAMTS-13 deficiency. After discontinued steroids treatment, his bilirubin level normalized within 4 days. On the 4th day after bilirubin level normalized, vincristine treatment was administered. TPE was also continued. There was no consensus about the optimal schedule for discontinuing plasmapheresis therapy, and also we observed total bilirubin level improvement with discontinued corticosteroid treatment. In this case, corticosteroid treatment was linked with the increase of total bilirubin level in severe ADAMTS-13-deficient TTP patient.
authors.affiliation: Department of Internal Medicine, Gaziantep University School of Medicine, Gaziantep, Turkey. zeynelasayiner@hotmail.com.
authors.forename: Zeynel Abidin
authors.initials: ZA
authors.lastname: Sayiner
authors.affiliation: Department of Haematology, Gaziantep University School of Medicine, Gaziantep, Turkey.
authors.forename: Didar Yanardag
authors.initials: DY
authors.lastname: Acik
authors.affiliation: Department of Haematology, Gaziantep University School of Medicine, Gaziantep, Turkey.
authors.forename: Mehmet
authors.initials: M
authors.lastname: Yilmaz
authors.affiliation: Department of Internal Medicine, Gaziantep University School of Medicine, Gaziantep, Turkey.
authors.forename: Salih
authors.initials: S
authors.lastname: Subari
authors.affiliation: Department of Infection Diseases, Gaziantep University School of Medicine, Gaziantep, Turkey.
authors.forename: Ayse Ozlem
authors.initials: AO
authors.lastname: Mete
authors.affiliation: Department of Haematology, Dicle University School of Medicine Diyarbakir, Diyarbakir, Turkey.
authors.forename: M Sinan
authors.initials: MS
authors.lastname: Dai
mesh_terms: D051722:ADAM Proteins / Q000172:deficiency*; D000071120:ADAMTS13 Protein; D000305:Adrenal Cortex Hormones / Q000008:administration & dosage* / Q000009:adverse effects*; D000328:Adult; D001663:Bilirubin / Q000097:blood*; D003131:Combined Modality Therapy / Q000009:adverse effects / Q000379:methods; D005260:Female; D006801:Humans; D010956:Plasmapheresis / Q000379:methods; D011697:Purpura, Thrombotic Thrombocytopenic / Q000139:chemically induced / Q000175:diagnosis* / Q000628:therapy*; D016896:Treatment Outcome; D015854:Up-Regulation
chemical_list: D000305:Adrenal Cortex Hormones; D051722:ADAM Proteins; D000071120:ADAMTS13 Protein; C099604:ADAMTS13 protein, human; D001663:Bilirubin
keywords: Corticosteroid treatment; Hemolysis; Severe ADAMTS-13 deficiency; TPE; TTP