Presenting signs and symptoms of patients with thrombotic thrombocytopenic purpura-hemolytic uremic syndrome
| Primary diagnostic criteria: |
| Thrombocytopenia |
| Microangiopathic hemolytic anemia (defined by negative findings on direct antiglobulin test, red blood cell fragmentation, and evidence of accelerated red cell production and destruction) |
| No clinically apparent alternative explanation for thrombocytopenia and anemia |
| Other common clinical features: |
| Renal function abnormalities (proteinuria/hematuria common, acute renal failure and oliguria less common) |
| Neurologic abnormalities (mental status changes common, focal abnormalities less common) |
| Weakness |
| Abdominal symptoms (nausea, vomiting, diarrhea, pain) |
| Fever (high fever with chills is evidence against the diagnosis of TTP-HUS) |
| Primary diagnostic criteria: |
| Thrombocytopenia |
| Microangiopathic hemolytic anemia (defined by negative findings on direct antiglobulin test, red blood cell fragmentation, and evidence of accelerated red cell production and destruction) |
| No clinically apparent alternative explanation for thrombocytopenia and anemia |
| Other common clinical features: |
| Renal function abnormalities (proteinuria/hematuria common, acute renal failure and oliguria less common) |
| Neurologic abnormalities (mental status changes common, focal abnormalities less common) |
| Weakness |
| Abdominal symptoms (nausea, vomiting, diarrhea, pain) |
| Fever (high fever with chills is evidence against the diagnosis of TTP-HUS) |
The presence of the primary diagnostic criteria is sufficient to establish a presumptive diagnosis of TTP-HUS and to begin plasma exchange treatment.1,4 The additional clinical features support the diagnosis but may not be present. Profound weakness is a common symptom that appears to reflect more than just rapidly developing anemia. Abdominal symptoms are present in many more patients than those with infectious bloody diarrhea. Although fever may occur as part of the TTP-HUS syndrome,3 high fever with shaking chills more likely indicates sepsis as the primary diagnosis.