A 9-MONTH-OLD INFANT WITH ACQUIRED IDIOPATHIC THROMBOTIC THROMBOCYTOPENIC PURPURA CAUSED BY INHIBITORY IgG-AUTOANTIBODY TO ADAMTS13
| Autor: | Atsushi Sato, Yoshiyuki Hoshi, Ryusuke Sato, Ayami Isonishi, Masue Imaizumi, Yoshihiro Fujimura, Masaei Onuma, Masanori Matsumoto, Yukiko Tsunematsu |
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| Rok vydání: | 2010 |
| Předmět: |
Male
Prednisolone Idiopathic thrombotic thrombocytopenic purpura ADAMTS13 Protein Platelet Transfusion Inhibitory postsynaptic potential Bethesda unit Methylprednisolone Autoimmune Diseases Diagnosis Differential hemic and lymphatic diseases medicine Humans Autoantibodies Purpura Thrombocytopenic Idiopathic Purpura Thrombotic Thrombocytopenic business.industry Infant Hematology Combined Modality Therapy ADAMTS13 ADAM Proteins Purpura Platelet transfusion Oncology Immunoglobulin G Pediatrics Perinatology and Child Health Immunology Etiology medicine.symptom Differential diagnosis business Immunosuppressive Agents |
| Zdroj: | Pediatric Hematology and Oncology. 27:53-58 |
| ISSN: | 1521-0669 0888-0018 |
| DOI: | 10.3109/08880010903401752 |
| Popis: | Although acquired idiopathic thrombotic thrombocytopenic purpura (ai-TTP) is rare in children, the authors present the case of a 9-month-old boy with ai-TTP showing severe deficiency of ADAMTS13 activity by its inhibitory IgG-autoantibody (4.8 Bethesda units/mL). Plasma exchange therapy was clinically effective but transient. Deficient activity of ADAMTS13 with the presence of its inhibitor persisted for 7 months after the initial diagnosis. However, other laboratory findings improved gradually with steroid (pulse) therapy. The hitherto insufficiently characterized clinical settings of ai-TTP during early childhood underscore the importance of measuring ADAMTS13 activity and its inhibitors for differential diagnosis in patients with thrombocytopenia of unknown etiology. |
| Databáze: | OpenAIRE |
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