Rituximab for acute plasma-refractory thrombotic thrombocytopenic purpura: A case report. (October 2022)

• Record Type: Journal Article
• Authors: Chaurasiya, Prem Shankar; Khatri, Akash; Gurung, Shekhar; Karki, Saurab; Shahi, Sunil; Aryal, Laxmi
• Is Part Of: Annals of medicine and surgery. Volume 82(2022)
• Publication Date: 2022-10
• Subjects: Anemia -- PLASMIC score -- Rituximab -- Thrombocytopenia -- Thrombotic thrombocytopenic purpura; Surgery -- Periodicals; Medicine -- Periodicals; General Surgery -- Periodicals; Education, Medical -- Periodicals; Periodicals; 617
• DOI: 10.1016/j.amsu.2022.104789
• Languages: English
• ISSNs: 2049-0801

Abstract

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. Highlights: Thrombotic thrombocytopenic purpura is a rare disease due to deficiency of ADAMTS13 where patients can present with anemia and thrombocytopenia. PLASMIC score can be used for presumptive diagnosis of this condition while ADAMTS13 assay is required for confirmation. Steroids and plasma exchange are the preferred treatment. In case of refractory cases, Rituximab can play a role to treat as well as prevent recurrence.