Acquired thrombotic thrombocytopenia purpura associated with severe ADAMTS13 deficiency in a 3-year-old boy: a case report and review of the literature
| Autor: | C-Khai Loh, Ho Wai Koo, Hamidah Alias, Farah Azima Abdul Muttlib, Woon Lee Yong, Hafiza Alauddin, Azma Rz, Sie Chong Doris Lau |
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| Rok vydání: | 2018 |
| Předmět: |
Male
Pediatrics medicine.medical_specialty Purpura Thrombotic Thrombocytopenic business.industry Public health ADAMTS13 deficiency lcsh:R lcsh:Medicine ADAMTS13 Protein General Medicine 030204 cardiovascular system & hematology ADAMTS13 03 medical and health sciences 0302 clinical medicine Surgical oncology hemic and lymphatic diseases 030220 oncology & carcinogenesis Child Preschool Medicine Humans THROMBOCYTOPENIA PURPURA Thrombotic thrombocytopenia purpura business Children |
| Zdroj: | Journal of Medical Case Reports, Vol 12, Iss 1, Pp 1-8 (2018) |
| ISSN: | 1752-1947 |
| Popis: | Background Acquired thrombotic thrombocytopenia purpura is very rarely encountered in children. It is often misdiagnosed initially when the condition is not inherited. Case presentation We describe a 3-year-old Malay boy who presented with simple febrile seizure and had no neurological deficit, however, he was found to have microangiopathic hemolytic anemia, thrombocytopenia, and elevated serum lactate dehydrogenase. An ADAMTS13 assay results showed zero activities (0%), and markedly high level of ADAMTS13 inhibitor (93.15 U/mL) confirming the diagnosis of secondary thrombotic thrombocytopenia purpura. He received fresh frozen plasma infusions for 3 days and subsequently his platelet levels normalized. Serial ADAMTS13 assay results showed improvement. He was also given a short course of prednisolone after which the ADAMTS13 activity normalized (> 114%) at the end of prednisolone course. Conclusions At presentation, acquired thrombotic thrombocytopenia purpura in a very young child is commonly misdiagnosed as other conditions like idiopathic thrombocytopenic purpura, Evans syndrome, atypical hemolytic-uremic syndrome, or malignancy. ADAMTS13 assay should be performed early when thrombotic thrombocytopenia purpura is suspected as this condition is associated with dire consequences. |
| Databáze: | OpenAIRE |
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