Clinical presentation of TTP is highly variable, ranging from minimal symptoms to critical illness, but typically includes a combination of:
- Microangiopathic hemolytic anemia (MAHA) (median hemoglobin 8-10 g/dL)
- Thrombocytopenia (median platelet count 10-30 × 109/L)
- Organ dysfunction
The historic pentad is present in only 5% of patients, and includes:1
- Thrombocytopenia
- Microangiopathic hemolytic anemia
- Neurologic symptoms
- Kidney impairment
- Fever
Symptoms may include those of:
- Anemia:
- Weakness
- Fatigue
- Jaundice
- Pallor
- Thrombocytopenia:
- Petechiae/Purpura
- Bleeding, for example:
- Epistaxis
- Gingival
- Gastrointestinal
- Hematuria
- Menorrhagia
- Hemoptysis
- Retinal hemorrhage
- Organ dysfunction, including:2
- Neurologic (about 60% of cases), including:3
- Confusion
- Headaches
- Stroke
- Coma
- Seizures
- Visual changes
- Altered speech
- Paresthesia
- Cardiovascular (heart ischemia in about 25% of cases), including:4
- Chest pain
- Arrhythmias
- Symptoms of heart failure, for example:
- Paroxysmal nocturnal dyspnea
- Orthopnea
- Dyspnea on exertion
- Fatigue and weight gain
- Cough
- Hypotension
- Myocardial infarction
- Acute cardiac arrest
- The gastrointestinal tract:
- Mesenteric ischemia (about 35% of cases), which may present with:
- Abdominal pain
- Nausea
- Vomiting
- Diarrhea
- Patients may also present with pancreatitis1
- Mesenteric ischemia (about 35% of cases), which may present with:
- The kidney:
- Hematuria and proteinuria
- Rena failure rare
- Neurologic (about 60% of cases), including:3
- Conditions associated with secondary TTP such as those related to:
- Autoimmune diseases, especially SLE
- Pregnancy
- Drugs
- HIV infection
- Pancreatitis
- Cancer
- Organ transplantation
- Consensus on the standardization of terminology in thrombotic thrombocytopenic purpura and related thrombotic microangiopathies: “Acute TTP presentations may include bleeding symptoms such as bruising or hematuria, or thrombotic symptoms associated with neurologic or cardiac involvement.”5
Lab tests
- Thrombocytopenia:6
- Caused by consumption of platelets in platelet-rich thrombi.
- Median platelet count typically 10–30 × 10 9 /L at presentation.
- MAHA:7
- Caused by mechanical fragmentation of erythrocytes during flow through partially occluded, high-shear small vessels.
- Median hemoglobin levels on admission are typically 80–100 g/L.
- Evidence of hemolysis:
- Elevated LDH, caused by:
- Hemolysis
- Tissue ischemia
- Low haptoglobin
- Increased indirect bilirubin
- Elevated LDH, caused by:
- Evidence of schistocytes on peripheral smear
- PT, aPTT typically normal
- Elevated troponin levels:
- In > 50% of cases
- Consensus on the standardization of terminology in thrombotic thrombocytopenic purpura and related thrombotic microangiopathies: “Anemia may not be immediately obvious. Thrombocytopenia is generally severe (platelet count of < 30 9 10 9 L1), but higher platelet numbers do not exclude the diagnosis”.8
Cohort studies
- Scully et al, 2019
- 145 patients with TTP
- Median platelet count (range) – 24,000 (3,000-133,000)
- Median LDH (range) – 422 (120-3343)
- Page et al, 2017
- 78 patients with TTP (defined as ADAMTS13 activity <10%)
- Fever 10%
- Neurologic changes:
- Severe 53%
- Mild 27%
- None 20%
- Thrombocytopenia 100%:
- Median platelet count 10,000
- Platelet count
- > 20,000 in 14%
- > 30,000 in 4%
- MAHA 100%
- Median Hct 21%
- Hct > 30 in 3%
- Only 14% had serum creatinine concentrations ≥2.5 mg/dL
- Blombery et al, 2016
- 57 patients with TTP (defined as ADAMTS13 activity <10%), accounting for 72 episodes
- Neurological 71% of episodes
- Hemorrhagic manifestations 46% of episodes
- GI manifestations in 39% of episodes
- Fever 28% of episodes
- Renal manifestations in 10% of episodes
- TTP pentad 7% of episodes

Clinical practice guidelines
- A British Society for Haematology Guideline: Diagnosis and management of thrombotic thrombocytopenic purpura and thrombotic microangiopathies.
