Atypical Presentation of Thrombotic Thrombocytopenic Purpura without Hematological Features
Autor: | Varun Modi, Kok Hoe Chan, Michael Maroules, Parminder Kaur, Balraj Singh |
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Jazyk: | angličtina |
Rok vydání: | 2020 |
Předmět: |
Microangiopathic hemolytic anemia
medicine.medical_specialty Thrombotic thrombocytopenic purpura ADAMTS 13 Case Report Disease 030204 cardiovascular system & hematology lcsh:RC254-282 Gastroenterology 03 medical and health sciences 0302 clinical medicine Internal medicine hemic and lymphatic diseases medicine Transplantation business.industry Mortality rate ADAMTS Thrombotic thrombocytopenic purpura (TTP) Hematology lcsh:Neoplasms. Tumors. Oncology. Including cancer and carcinogens medicine.disease Thrombocytopenia Pathophysiology Oncology 030220 oncology & carcinogenesis Presentation (obstetrics) business Thrombotic complication |
Zdroj: | International Journal of Hematology-Oncology and Stem Cell Research Scopus-Elsevier International Journal of Hematology-Oncology and Stem Cell Research, Vol 14, Iss 3 (2020) |
ISSN: | 2008-2207 2008-3009 |
Popis: | Thrombotic thrombocytopenic purpura (TTP) is a life-threatening disease, usually diagnosed with high index of suspicion. The pathophysiology of TTP is due to severe deficiency of von Willebrand factor cleaving protease, known as ADAMTS 13. Early diagnosis is crucial as without treatment TTP is associated with high mortality rate. Plasma exchange is currently the mainstay of treatment. Nonetheless, the classical pentad of microangiopathic hemolytic anemia (MAHA), thrombocytopenia, neurological dysfunction, kidney dysfunction and fever are seen only in 40 percent of the patients. MAHA and thrombocytopenia are the common presenting features. Presentation with thrombotic complication without hematological features (MAHA and thrombocytopenia) is rare and makes the diagnosis difficult. Herein, we report an unusual presentation of a 53-year-old male, who was initially presented in 2014 with classical features of TTP, however had an atypical presentation of TTP in 2016 with only neurological features without hematological features. |
Databáze: | OpenAIRE |
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