title: High titer of ADAMTS13 inhibitor associated with thrombotic microangiopathy of the gut and skeletal muscle after allogeneic hematopoietic stem cell transplantation.
abstract: Transplantation-associated thrombotic microangiopathy (TMA) is one of the main complications after hematopoietic stem cell transplantation (HSCT). At the time of onset of gut TMA, a patient developed a high titer of an inhibitor of the non-immunoglobulin G type to ADAMTS13, which physiologically hydrolyzes von Willebrand factor to control spontaneous intravascular thrombus formation. The patient developed symptoms of myositis, a disorder that has occasionally been reported to manifest after HSCT and to resemble some idiopathic autoimmune diseases. However, a muscle biopsy specimen presented pathologic findings of TMA, including microvascular platelet thrombus formation, without inflammatory lymphocyte infiltration. ADAMTS13 activities returned to normal after steroid treatment, and the improvement of TMA symptoms followed. This patient appears to represent a rare case of post-HSCT TMA associated with the development of an ADAMTS13 inhibitor.
authors.affiliation: Department of Hematology, Nagoya University Graduate School of Medicine, Nagoya, Japan.
authors.forename: Tatsuya
authors.initials: T
authors.lastname: Adachi
authors.forename: Tadashi
authors.initials: T
authors.lastname: Matsushita
authors.forename: Ryoichi
authors.initials: R
authors.lastname: Ichihashi
authors.forename: Kanji
authors.initials: K
authors.lastname: Hirashima
authors.forename: Masafumi
authors.initials: M
authors.lastname: Ito
authors.forename: Akira
authors.initials: A
authors.lastname: Inukai
authors.forename: Toshiya
authors.initials: T
authors.lastname: Yokozawa
authors.forename: Tetsuya
authors.initials: T
authors.lastname: Nishida
authors.forename: Makoto
authors.initials: M
authors.lastname: Murata
authors.forename: Mutsuharu
authors.initials: M
authors.lastname: Hayashi
authors.forename: Akira
authors.initials: A
authors.lastname: Katsumi
authors.forename: Tetsuhito
authors.initials: T
authors.lastname: Kojima
authors.forename: Hidehiko
authors.initials: H
authors.lastname: Saito
authors.forename: Tomoki
authors.initials: T
authors.lastname: Naoe
mesh_terms: D051722:ADAM Proteins / Q000037:antagonists & inhibitors* / Q000097:blood / Q000276:immunology; D000071120:ADAMTS13 Protein; D000328:Adult; D000893:Anti-Inflammatory Agents / Q000008:administration & dosage; D001327:Autoimmune Diseases / Q000097:blood* / Q000188:drug therapy / Q000209:etiology / Q000276:immunology / Q000473:pathology; D019774:Blood Coagulation Factor Inhibitors / Q000097:blood* / Q000276:immunology; D018380:Hematopoietic Stem Cell Transplantation; D006801:Humans; D008228:Lymphoma, Non-Hodgkin / Q000097:blood* / Q000150:complications / Q000276:immunology / Q000473:pathology / Q000628:therapy; D008297:Male; D008775:Methylprednisolone / Q000008:administration & dosage; D013927:Thrombosis / Q000097:blood* / Q000188:drug therapy / Q000209:etiology / Q000276:immunology / Q000473:pathology
chemical_list: D000893:Anti-Inflammatory Agents; D019774:Blood Coagulation Factor Inhibitors; D051722:ADAM Proteins; D000071120:ADAMTS13 Protein; C099604:ADAMTS13 protein, human; D008775:Methylprednisolone