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

Tatsuya Adachi; Tadashi Matsushita; Ryoichi Ichihashi; Kanji Hirashima; Masafumi Ito; Akira Inukai; Toshiya Yokozawa; Tetsuya Nishida; Makoto Murata; Mutsuharu Hayashi; Akira Katsumi; Tetsuhito Kojima; Hidehiko Saito; Tomoki Naoe

doi:10.1532/IJH97.05157

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

Int J Hematol. 2006 Jun;83(5):415-9. doi: 10.1532/IJH97.05157.

Authors

Tatsuya Adachi¹, Tadashi Matsushita, Ryoichi Ichihashi, Kanji Hirashima, Masafumi Ito, Akira Inukai, Toshiya Yokozawa, Tetsuya Nishida, Makoto Murata, Mutsuharu Hayashi, Akira Katsumi, Tetsuhito Kojima, Hidehiko Saito, Tomoki Naoe

Affiliation

¹ Department of Hematology, Nagoya University Graduate School of Medicine, Nagoya, Japan.

PMID: 16787872
DOI: 10.1532/IJH97.05157

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.

Publication types

Case Reports
Research Support, Non-U.S. Gov't

MeSH terms

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

Substances

Anti-Inflammatory Agents
Blood Coagulation Factor Inhibitors
ADAM Proteins
ADAMTS13 Protein
ADAMTS13 protein, human
Methylprednisolone