Thrombotic thrombocytopenic purpura is a hematologic disease with a high mortality rate that affects multiple organ systems. It is caused by a deficiency of a metalloprotease known as the ADAMTS-13 enzyme. Patients can present with fever, hemolytic anemia, thrombocytopenia, kidney injury, and neurological symptoms. Here we present a case of a female with TTP who was initially diagnosed with benign paroxysmal positional vertigo (BPPV). Her initial symptoms were vertigo, blurry vision, and gait imbalance, which were accompanied by elevated total bilirubin, acute kidney injury, thrombocytopenia, and normal hemoglobin. When the patient followed up in her primary care clinic, she was found to have worsening renal function, hemolytic anemia, and severe thrombocytopenia. She was subsequently diagnosed with TTP and referred to the emergency department (ED) where she received emergent treatment with therapeutic plasma exchange (TPE) and later rituximab. This case stresses the importance of considering TTP in patients presenting with symptoms and laboratory values suggestive of the disease, even when anemia is not present.
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http://dx.doi.org/10.7759/cureus.78305 | DOI Listing |
Zhonghua Xue Ye Xue Za Zhi
January 2025
Jiangsu Institute of Hematology, National Clinical Research Center for Hematologic Diseases, NHC Key Laboratory of Thrombosis and Hemostasis, The First Affiliated Hospital of Soochow University, Collaborative Innovation Center of Hematology, Soochow Universtiy, Suzhou 215006, China.
To evaluate the efficacy and safety of obinutuzumab combined with glucocorticoid-based therapy in patients with relapsed immune thrombotic thrombocytopenic purpura (iTTP). This study analyzed the efficacy and adverse reactions of four patients with relapsed iTTP who were treated with a combination of obinutuzumab and glucocorticoids to assess the effectiveness and safety of the treatment. All four patients had a history of multiple relapses and had previously undergone treatment with rituximab and bortezomib.
View Article and Find Full Text PDFJ Thromb Haemost
March 2025
Service d'Hématologie biologique, Hôpital Lariboisière, AP-HP.Nord, Université Paris Cité, Paris, France; INSERM UMR-S 1138, Centre de Recherche des Cordeliers, Université Paris Cité, Sorbonne Université, Paris, France.
Introduction: Thrombotic thrombocytopenic purpura (TTP) is a thrombotic microangiopathy characterized by a severe functional deficiency of ADAMTS13. Measuring ADAMTS13 activity is crucial for diagnosing TTP (<10 IU/dL), monitoring treatments, and detecting relapses (<20 IU/dL). The Technofluor® assay allows a rapid ADAMTS13 activity measurement using the CEVERON® s100 analyzer.
View Article and Find Full Text PDFStroke
March 2025
Centre National de Référence des MicroAngiopathies Thrombotiques, Paris, France (A.P., B.S.J., A.V., P.C.).
The 2024 Guidelines for the Primary Prevention of Stroke, recently updated by the American Heart Association and the American Stroke Association, serve as an essential resource for clinicians aiming to reduce the growing impact of cerebrovascular disease. These guidelines emphasize modifiable risk factors and population-specific considerations, covering a range of cardiovascular conditions, including hypertension, diabetes, atherosclerotic disease, and genetic predispositions to stroke. However, a notable omission in these guidelines is the absence of specific recommendations for patients with thrombotic thrombocytopenic purpura.
View Article and Find Full Text PDFCureus
February 2025
Medicine, Queen Elizabeth Hospital, Hong Kong, HKG.
Thrombotic microangiopathy (TMA) represents a diverse group of conditions characterized by the presence of microangiopathic hemolytic anemia (MAHA), thrombocytopenia, and ischemic end-organ injury. Thrombotic thrombocytopenic purpura (TTP) is an important cause of TMA requiring urgent identification and therapeutic plasma exchange (TPE). Cancer-related TMA is commonly misdiagnosed as TTP.
View Article and Find Full Text PDFCureus
February 2025
Nephrology, State University of New York Downstate Health Sciences University, Brooklyn, USA.
We present an unusual case of complement-mediated thrombotic microangiopathy (formerly known as atypical hemolytic uremic syndrome) associated with inflammatory disease in a young patient. A 26-year-old male patient with no significant past medical history presented to our emergency department with a four-week history of diffuse, moderate, cramping, non-radiating abdominal pain with no known aggravating or relieving factors. Abdominal pain was associated with nausea, vomiting, and bloody stools.
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