High titer of ADAMTS13 inhibitor associated with thrombotic microangiopathy of the gut and skeletal muscle after allogeneic hematopoietic stem cell transplantation

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 hyd...

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Bibliographic Details
Published in:International journal of hematology 2006-06, Vol.83 (5), p.415-419
Main Authors: Adachi, Tatsuya, Matsushita, Tadashi, Ichihashi, Ryoichi, Hirashima, Kanji, Ito, Masafumi, Inukai, Akira, Yokozawa, Toshiya, Nishida, Tetsuya, Murata, Makoto, Hayashi, Mutsuharu, Katsumi, Akira, Kojima, Tetsuhito, Saito, Hidehiko, Naoe, Tomoki
Format: Article
Language:English
Subjects:
ADAM Proteins - antagonists & inhibitors
ADAM Proteins - blood
ADAM Proteins - immunology
ADAMTS13 Protein
Adult
Anti-Inflammatory Agents - administration & dosage
Autoimmune Diseases - blood
Autoimmune Diseases - drug therapy
Autoimmune Diseases - etiology
Autoimmune Diseases - immunology
Autoimmune Diseases - pathology
Blood Coagulation Factor Inhibitors - blood
Blood Coagulation Factor Inhibitors - immunology
Hematopoietic Stem Cell Transplantation
Humans
Lymphoma, Non-Hodgkin - blood
Lymphoma, Non-Hodgkin - complications
Lymphoma, Non-Hodgkin - immunology
Lymphoma, Non-Hodgkin - pathology
Lymphoma, Non-Hodgkin - therapy
Male
Methylprednisolone - administration & dosage
Thrombosis - blood
Thrombosis - drug therapy
Thrombosis - etiology
Thrombosis - immunology
Thrombosis - pathology
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Summary: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.
ISSN:0925-5710
1865-3774
DOI:10.1532/IJH97.05157