Author/Authors :
Singh, Balraj Department of Hematology/Oncology - Saint Joseph University Medical Center, new Jersey, United States , Chan, Kok Hoe Department of Medical Education - Saint Michael's Medical Centre - New York Medical College, New Jersey, United States , Kaur, Parminder Department of Hematology/Oncology - Saint Joseph University Medical Center, new Jersey, United States , Modi, Varun Orange Regional Medical Centre, New Jersey, USA , Maroules, Michael Department of Hematology/Oncology - Saint Joseph University Medical Center, new Jersey, United States
Abstract :
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
Keywords :
Thrombotic thrombocytopenic purpura (TTP) , ADAMTS 13 , Microangiopathic hemolytic anemia , Thrombocytopenia
