Case 142: update 3

ADAMTS13 activity level subsequently comes back at <5% with positive anti-ADAMTS13 IgG antibodies, consistent with a diagnosis of acquired, immune TTP. The patient continues on daily PEX and 1 mg/kg prednisolone for immunosuppression. The patient responds well with resolution of neurologic symptoms and resolution of thrombocytopenia after 13 PEX sessions. PEX is continued for a further 3 days then discontinued. Steroids are continued and she is commenced on LMWH thromboprophylaxis and aspirin.

The patient remains in hospital for the remainder of her pregnancy for strict monitoring. ADAMTS13 activity recovers to 43% and she is successfully weaned off steroids. She goes into labour at 38 weeks gestation with an uncomplicated vaginal delivery of a healthy baby boy. She enters of period of surveillance for clinical, biochemical and serological relapse but after 6 months is lost to follow up.

Two years later, she is re-referred to haematology by her GP as she has expressed a desire to become pregnant again. On review in clinic, her blood counts are completely normal and she is asymptomatic. ADAMTS13 activity level is checked, which demonstrates an activity level of 11%.

How would you counsel the patient on the potential risks of further pregnancy?

How would you manage the patient to reduce the risk of TTP relapse?

How would you monitor the patient during the pregnancy? Is there a role for prophylactic PEX during pregnancy?

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