Atypical Presentation of Thrombotic Thrombocytopenic Purpura without Hematological Features

• Main Authors: Balraj Singh, Kok Hoe Chan, Parminder Kaur, Varun Modi, Michael Maroules
• Format: Article
• Language: English
• Published: Tehran University of Medical Sciences 2020-07-01
• Series: International Journal of Hematology-Oncology and Stem Cell Research
• Subjects: Thrombotic thrombocytopenic purpura (TTP); ADAMTS 13; Microangiopathic hemolytic anemia; Thrombocytopenia
• Online Access: https://ijhoscr.tums.ac.ir/index.php/ijhoscr/article/view/1171

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.