Which disorder typically presents with fever, neurological symptoms, hemolytic anemia, and thrombocytopenia?

Which disorder typically presents with fever, neurological symptoms, hemolytic anemia, and thrombocytopenia?

• A. Hemolytic-uremic syndrome
• B. Thrombotic thrombocytopenia purpura
• C. Disseminated intravascular coagulation
• D. Idiopathic thrombocytopenia purpura

Answer: (B)

Thrombotic thrombocytopenic purpura involves microangiopathic processes that cause a triad (along with fever) of neurologic symptoms, hemolytic anemia, and thrombocytopenia. The underlying issue is severe deficiency of the ADAMTS13 enzyme, which leads to accumulation of ultra-large von Willebrand factor multimers. These promote widespread small-vessel platelet aggregation, forming microthrombi that shear red cells as they pass through narrow vessels, producing schistocytes and hemolysis, while consuming platelets and causing thrombocytopenia. Neurologic manifestations—such as confusion, headaches, or seizures—are common due to brain microthrombi, and fever can accompany the systemic process. In contrast, hemolytic uremic syndrome tends to show more pronounced acute kidney injury with less prominent neuro symptoms and often follows diarrheal illness in children. Disseminated intravascular coagulation presents with a consumption coagulopathy evidenced by prolonged PT/aPTT and low fibrinogen, not the isolated MAHA pattern. Idiopathic thrombocytopenia purpura features isolated low platelets without hemolysis or microangiopathy. Thus, the combination of fever, neurologic symptoms, hemolytic anemia, and low platelets best fits thrombotic thrombocytopenic purpura.

Thrombotic thrombocytopenic purpura involves microangiopathic processes that cause a triad (along with fever) of neurologic symptoms, hemolytic anemia, and thrombocytopenia. The underlying issue is severe deficiency of the ADAMTS13 enzyme, which leads to accumulation of ultra-large von Willebrand factor multimers. These promote widespread small-vessel platelet aggregation, forming microthrombi that shear red cells as they pass through narrow vessels, producing schistocytes and hemolysis, while consuming platelets and causing thrombocytopenia. Neurologic manifestations—such as confusion, headaches, or seizures—are common due to brain microthrombi, and fever can accompany the systemic process. In contrast, hemolytic uremic syndrome tends to show more pronounced acute kidney injury with less prominent neuro symptoms and often follows diarrheal illness in children. Disseminated intravascular coagulation presents with a consumption coagulopathy evidenced by prolonged PT/aPTT and low fibrinogen, not the isolated MAHA pattern. Idiopathic thrombocytopenia purpura features isolated low platelets without hemolysis or microangiopathy. Thus, the combination of fever, neurologic symptoms, hemolytic anemia, and low platelets best fits thrombotic thrombocytopenic purpura.