Thrombotic Thrombocytopenic Purpura with Severe ADAMTS-13 Deficiency in a Patient with Antiphospholipid Antibodies and Charcot-Marie-Tooth Disease
| Autor: | Hirobumi Saitoh, Kenichi Sawada, Hideki Wakui, Mitsugu Ito, Rie Masai, Naoto Takahashi, Mayu Kosaka, Ryuta Sato |
|---|---|
| Rok vydání: | 2011 |
| Předmět: |
Adult
Hemolytic anemia medicine.medical_specialty Prednisolone Thrombotic thrombocytopenic purpura ADAMTS13 Protein Platelet Transfusion Gastroenterology Plasma Charcot-Marie-Tooth Disease hemic and lymphatic diseases Internal medicine Internal Medicine medicine Humans Purpura Thrombotic Thrombocytopenic business.industry General Medicine Antiphospholipid Syndrome medicine.disease Thrombocytopenic purpura Schistocyte ADAM Proteins Purpura Platelet transfusion Antibodies Anticardiolipin Immunology Female Fresh frozen plasma medicine.symptom business medicine.drug |
| Zdroj: | Internal Medicine. 50:487-493 |
| ISSN: | 1349-7235 0918-2918 |
| DOI: | 10.2169/internalmedicine.50.4300 |
| Popis: | A 26-year-old woman with a history of mild mental retardation, Charcot-Marie-Tooth disease (CMT) and idiopathic thrombocytopenic purpura developed severe thrombocytopenia with Coombs-negative hemolytic anemia. Magnetic resonance imaging revealed a fresh cerebral infarction in the left precentral gyrus. ADAMTS-13 deficiency caused by an inhibitor and anti-cardiolipin antibodies were detected in the blood. After treatment with prednisolone and fresh frozen plasma, ADAMTS-13 activity was normalized, the ADAMTS-13 inhibitor had disappeared and the thrombocytopenia with a bleeding tendency was improved. To our knowledge, this is the first case of thrombotic thrombocytopenic purpura caused by ADAMTS-13 deficiency associated with antiphospholipid antibodies and CMT. |
| Databáze: | OpenAIRE |
| Externí odkaz: |
|