(462) Thrombotic Thrombocytopenic Purpura Versus Evans Syndrome: A Case Study

Introduction: Thrombotic Thrombocytopenic Purpura (TTP) and Evan's Syndrome are rare hematological disorders characterized by thrombocytopenia, and distinguishing between them can be challenging due to overlapping clinical features. The abstract discusses the diagnostic process, exclusion of infectious sources, and successful management of TTP through emergent plasmapheresis and blood transfusions.

Description: A 42-year-old female with a history of autoimmune disorders presented with generalized weakness, abnormal menstrual bleeding, fatigue, dizziness, and shortness of breath. Initial laboratory results revealed severe thrombocytopenia (platelet count: 5 × 10^9/L) and anemia (hemoglobin: 6.5 g/dL) with schistocytes observed on peripheral blood smear, suggestive of microangiopathic hemolytic anemia. A preliminary diagnosis of Evan's Syndrome, an autoimmune pancytopenia, was suspected. Following hospitalization, the patient developed leukocytosis, fever, tachycardia, and encephalopathy, leading to a code sepsis evaluation and ICU admission. Further investigations ruled out infectious sources, and additional symptoms, including encephalopathy and renal dysfunction, pointed towards TTP as the likely cause. Notably, the direct antiglobulin test (DAT) was negative, ruling out autoimmune hemolytic anemia as the sole cause of thrombocytopenia. Markedly reduced ADAMTS13 activity ( < 2%) supported the diagnosis of TTP. Upon confirming TTP, the patient received emergent plasmapheresis and blood transfusions, resulting in complete symptom resolution.

Discussion: Initially, the patient's presentation raised concerns about Evan's Syndrome, characterized by concurrent autoimmune hemolytic anemia and immune thrombocytopenia. However, the exclusion of infectious etiologies through specific laboratory tests aided in differentiating TTP from autoimmune causes. The prompt recognition of TTP guided the initiation of emergent plasmapheresis and blood transfusions, leading to significant symptom improvement. This case emphasizes the importance of a comprehensive diagnostic approach to differentiate TTP from other hematological disorders and underscores the significance of timely therapeutic interventions in achieving favorable outcomes.