title: From humoral rejection to generalized thrombotic microangiopathy--role of acquired ADAMTS13 deficiency in a renal allograft recipient.
abstract: A 9-year-old renal transplant recipient presented with elevated serum creatinine levels 4 years post-transplant renal biopsy revealed humoral rejection including lesions suggestive for thrombotic microangiopathy (TMA). He received methylprednisolone pulses followed by a normalization of serum creatinine. Two more steroid responsive acute rejection episodes occurred. Two months later he presented rapidly progressive life threatening symptoms including bilateral pyramidal syndrome and hemoptysis. Serum haptoglobin became undetectable at this time and platelet count decreased (70000/microl), suggesting TMA. Cerebral MRI revealed generalized ischemic white matter lesions. ADAMTS13 activity decreased to < 5%. Daily plasma exchanges (PE) resulted in immediate improvement. All attempts to discontinue PE were unsuccessful. Transplantectomy resulted in normalization of generalized symptoms, hemolysis and ADAMTS13 activity (110%). Multi-organ involvement has never been reported in acquired ADAMTS13 deficiency post-transplant. Rapid resolution after transplantectomy might suggest that renal TMA was responsible for acquired ADAMTS13 deficiency and thereby triggered the generalization of TMA lesions.
authors.affiliation: Department of Pediatric Nephrology, Hôpital Trousseau; AP-HP & Université Paris VI, 75012 Paris, France. tim.ulinski@trs.aphp.fr
authors.forename: T
authors.initials: T
authors.lastname: Ulinski
authors.forename: A
authors.initials: A
authors.lastname: Charpentier
authors.forename: M
authors.initials: M
authors.lastname: Colombat
authors.forename: C
authors.initials: C
authors.lastname: Desconclois
authors.forename: B
authors.initials: B
authors.lastname: Mougenot
authors.forename: V
authors.initials: V
authors.lastname: Fremaux-Bacchi
authors.forename: C
authors.initials: C
authors.lastname: Suberbielle
authors.forename: G
authors.initials: G
authors.lastname: Deschênes
authors.forename: A
authors.initials: A
authors.lastname: Bensman
authors.forename: A
authors.initials: A
authors.lastname: Veyradier
mesh_terms: D051722:ADAM Proteins / Q000172:deficiency*; D000071120:ADAMTS13 Protein; D000917:Antibody Formation; D001921:Brain / Q000473:pathology; D002545:Brain Ischemia / Q000473:pathology; D006084:Graft Rejection / Q000276:immunology* / Q000473:pathology; D006801:Humans; D007231:Infant, Newborn; D016030:Kidney Transplantation / Q000276:immunology* / Q000473:pathology; D008279:Magnetic Resonance Imaging; D008297:Male; D014184:Transplantation, Homologous
chemical_list: D051722:ADAM Proteins; D000071120:ADAMTS13 Protein; C099604:ADAMTS13 protein, human