Case 142: update 2

Due to concerns regarding possible TTP, you make your way back to hospital immediately. You request a slew of investigations to be arranged urgently. LDH and reticulocytes are markedly elevated and DAT is negative, consistent with non-immune haemolysis.  Cardiac troponin is elevated indicating cardiac involvement. Urinalysis excludes significant proteinuria. An ADAMTS13 assay is requested and sent to the reference lab for urgent processing.

On arrival to the ward, the patient is being assessed by the obstetrics registrar. Fetal ultrasound demonstrates that the foetus is growing normally with no immediate concerns. After liaising with the renal consultant on-call, you arrange admission to the renal ward for emergency plasma exchange with S/D-plasma (1.5 plasma volumes per day). Joint care is agreed between haematology, obstetrics and renal medicine.

How would you differentiate between acquired and congenital TTP? Would it change how you manage the patient?

Given the degree of the thrombocytopenia, would you give a platelet transfusion prior to Vascath insertion?

What clinical and laboratory parameters would you use to assess response to PEX?

What role (if any) do glucocorticoids, rituximab and caplacizumab have in this patient’s management?

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