Prem Shankar Chaurasiya1, Akash Khatri2, Shekhar Gurung3, Saurab Karki4, Sunil Shahi5, Laxmi Aryal6. 1. Kaliaya Hospital, Bara, Nepal. 2. Civil Service Hospital, Kathmandu, Nepal. 3. Shivanagar Primary Health Care Center, Chitwan, Nepal. 4. Military Hospital Itahari, Sunsari, Nepal. 5. Military Hospital Nepalgunj, Banke, Nepal. 6. Laxmi Aryal, Burtibang Primary Health Care Center, Baglung, Nepal.
Abstract
Introduction and importance: Thrombotic thrombocytopenic purpura (TTP) is a rare disease due to deficiency of ADAMTS13 which can present with anemia and thrombocytopenia. The study highlights the role of PLASMIC score in diagnosis and rituximab in the treatment of this condition. Case presentation: Herein, we report a case of 38 years old female who had presented with fever, irritability, and altered sensorium. On investigations, she had hemolytic anemia, and thrombocytopenia with peripheral blood smear showing occasional schistocytes and managed with steroids and plasma exchange. As her platelet, LDH, and a few other lab parameters failed to normalize and met the criteria of refractory TTP, hence she was started on 5 cycles of rituximab and her condition improved. Clinical discussion: Thrombotic thrombocytopenic purpura can be presumed based upon PLASMIC score where if the score is 5 or more while ADAMTS13 assay is required for confirmation. It is a life-threatening condition where treatment options include therapeutic plasma exchange (PEX), glucocorticoids, Rituximab, and caplacizumab. Rituximab is considered particularly in refractory cases. Conclusion: Thrombotic thrombocytopenic purpura can lead to complications due to low platelet counts. Hence, early diagnosis and intervention are crucial to prevent such complications.
Introduction and importance: Thrombotic thrombocytopenic purpura (TTP) is a rare disease due to deficiency of ADAMTS13 which can present with anemia and thrombocytopenia. The study highlights the role of PLASMIC score in diagnosis and rituximab in the treatment of this condition. Case presentation: Herein, we report a case of 38 years old female who had presented with fever, irritability, and altered sensorium. On investigations, she had hemolytic anemia, and thrombocytopenia with peripheral blood smear showing occasional schistocytes and managed with steroids and plasma exchange. As her platelet, LDH, and a few other lab parameters failed to normalize and met the criteria of refractory TTP, hence she was started on 5 cycles of rituximab and her condition improved. Clinical discussion: Thrombotic thrombocytopenic purpura can be presumed based upon PLASMIC score where if the score is 5 or more while ADAMTS13 assay is required for confirmation. It is a life-threatening condition where treatment options include therapeutic plasma exchange (PEX), glucocorticoids, Rituximab, and caplacizumab. Rituximab is considered particularly in refractory cases. Conclusion: Thrombotic thrombocytopenic purpura can lead to complications due to low platelet counts. Hence, early diagnosis and intervention are crucial to prevent such complications.
Authors: Antoine Froissart; Marc Buffet; Agnès Veyradier; Pascale Poullin; François Provôt; Sandrine Malot; Michael Schwarzinger; Lionel Galicier; Philippe Vanhille; Jean-Paul Vernant; Dominique Bordessoule; Bertrand Guidet; Elie Azoulay; Eric Mariotte; Eric Rondeau; Jean-Paul Mira; Alain Wynckel; Karine Clabault; Gabriel Choukroun; Claire Presne; Jacques Pourrat; Mohamed Hamidou; Paul Coppo Journal: Crit Care Med Date: 2012-01 Impact factor: 7.598
Authors: M Scully; S Cataland; P Coppo; J de la Rubia; K D Friedman; J Kremer Hovinga; B Lämmle; M Matsumoto; K Pavenski; E Sadler; R Sarode; H Wu Journal: J Thromb Haemost Date: 2017-01-30 Impact factor: 5.824