Thrombotic thrombocytopenic purpura

Abstract

A 34-year old primipara was admitted to hospital with dichorionic–diamniotic twins in 26+3 weeks of gestation. In suspicion of HELLP-syndrome, caesarean section was performed at 27+4 weeks of gestation, because of platelet count was reduced to 44000/µl. A re-laparotomy had to be performed because of intra-abdominal bleedings. The patient was given seven packed thrombocytes and five packed erythrocytes. The patient complained about blurred vision. The right corner of the mouth was slightly depressed in terms of a facial nerve paresis. Further platelet counts were about 50000/µl. Haemoglobin: 7.8 mg/l. D-Dimer: 1066 mg/l. LDH was elevated to 1610 U/l, reticulocytes were elevated to 13.19% and haptoglobin was reduced to <0.08 g/l. The Coombs’ test was negative. The ADAMTS-13 test showed a reduced activity. Hereby, the diagnoses of thrombotic thrombocytopenic purpura was confirmed. Plasma exchange is the most effective option; application of platelet concentrate should be avoided, because of worsening microangiopathy and subsequent neurological situation. After the patient has received plasma exchange, platelet count normalized. An ophthalmic examination showed a dysfunction in choroid perfusion as a cause for the blurred vision. After discharge regular lab tests were planned, but no further treatment was necessary at that point of time. The premature twins survived after ventricular haemorrhage, severe sepsis, bronchopulmonary dysplasia and other complications of neonates.

Keywords::

• ADAMTS-13
• Coombs-test
• haemolysis
• HELLP-syndrome
• plasma exchange
• pregnancy
• thrombocytopenia

Declaration of Interest: The authors report no conflicts of interest.