MEDizzy - A 35-year-old woman who is 16 weeks pregnant has attended

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Combined Hematology

A 35-year-old woman who is 16 weeks pregnant has attended the acute medical unit after her first seizure. Her pregnancy has been uncomplicated thus far. Her temperature is 39.4ºC, pulse rate 86, blood pressure 125/86 mmHg. Bloods are as follows: Hb 69 g/l Platelets 43 * 109/l WBC 7.4 * 109/l Na+ 137 mmol/l K+ 4.9 mmol/l Urea 18 mmol/l Creatinine 278 µmol/l Urine dip was negative for protein and ketones. The laboratory phone you to inform you schistocytes have been seen on the blood film. Which of these best describes the pathogenesis of this condition

ExplanationB. Acquired inhibition of the protein ADAMTS13 which cleaves vWF multimers is the most common cause of TTP This woman has presented with the classical pentad of thrombotic thrombocytopenic purpura - fever, neurological dysfunction, evidence of hemolysis (blood film), renal injury and thrombocytopenia. Acquired inability to cleave vWF multimers is the most common cause of TTP. This can occasionally be prompted by pregnancy. This results in platelet deposition and widespread coagulation. ADAMTS13 is the protein responsible for this cleavage and can be inhibited by numerous causes. A congenital deficiency in this protein is a rare cause (Upshaw Schulman Syndrome). 1 - Describes disseminated intravascular coagulation. This has a similar hemolytic picture, but fever, neurological dysfunction and AKI are less common. 3 - Describes haemolytic-uraemic syndrome which is classically associated with E coli O:157; however no prodromal history of diarrhoea is mentioned. The blood results would however be rather similar. 4 - Describes pre-eclampsia. This can cause both seizures (eclampsia) and a microangiopathic hemolytic anemia. However, it would be uncommon to occur so early in pregnancy. a negative urine dip also suggests this is not the diagnosis. 5 - This is malaria. Whilst it can also cause hemolysis, it is uncommon for it to do so with this history. For malaria to be severe enough to cause seizures, one would expect the blood film to show some parasites and not just schistocytes.