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Thrombotic Thrombocytopenic Purpura

In Her Blood

Step 1: View clinicals

A 64 year old lady presents 45 minutes after a generalized tonic-clonic seizure which lasted for approximately 5 minutes. She recovered consciousness immediately afterwards, but has remained drowsy and confused since then. All of this was witnessed by her daughter. She had complained 'feeling unwell' during the preceding 4 days. Her medical history is only significant for mild hypertension well controlled on Amlodipine alone. A capillary blood glucose and serum electrolytes are within normal parameters, as are serial ECGs, a noncontrast CT of the brain, and a toxicology screen. However, a complete blood count is significant for a Hb of 8.7 g/dL (normal:11-18), and platelet count of 40,000/mm3 (normal: 150,000-400,000). A follow up blood film confirms the presence of anemia and thrombocytopenia, while additionally revealing abundant reticulocytes and numerous schistocytes.

Step 2: Order all relevant investigations

Renal Functions + Liver Functions

Blood Urea: 25 mg/dL (0 - 50) Serum Creatinine: 1.7 mg/dL (0.4 - 1.5) ALT: 20 IU/L (7-47) AST: 32 IU/L (10-40) ALP: 94 IU/L (44-147) INR: 1.0 (0.9-1.1) Total Serum Bilirubin: 1.9 mg/dL (0.2-1.2) Direct Bilirubin: 0.1 mg/dL (0.1-0.4)

Coagulation Profile

INR: 1.0 (0.9-1.1) PT: 12 sec (11-13.5) APTT: 35 sec (30-50) TT: 11 sec (10-15) Fibrinogen: 300 mg/dL (150-400)

LDH + Haptoglobin

Lactate Dehydrogenase (LDH): 490 IU/L (105-333) Serum Haptoglobin: <10 mg /dL (45-165)

ADAMTS13 Activity Levels

The results of the ADAMTS13 activity assay will be available in a few hours.

Step 3: Select appropriate management

Plasma Exchange
Sodium Nitroprusside

Score: ★★☆