A 30 years old pregnant female presented with severe preeclampsia at 35 weeks gestation. A full blood count and clinical chemistry profile were performed and the following results noted:
- Hb 123 g/L, WBC 17.7 x 109/L, platelet count 99 x 109/L and D Dimer 9.6 µg/ml. The total bilirubin was 60 µmol/L, ALT 839 U/L, AST 1739 U/L and LDH 2792 IU/L.
Four hours later the above tests were repeated with the following results:
- Hb 117 g/L, WBC 15.4 x 109/L, platelet count 55 x 109/L and D Dimer 10.4 µg/ml. The total bilirubin was 98 µmol/L, ALT 791 U/L, AST 2452 U/L and LDH 3500 IU/L.
- The initial blood film showed the presence of an occasional schistocyte or sharp fragmented red cell. The presence of so few schistocytes, together with a rapidly falling platelet count, was suggestive of a microangiopathic process. The blood film prepared 4 hours later showed an increase in the number of schistocytes. A diagnosis of HELLP was made on this patient.
The HELLP syndrome (haemolysis, elevated liver enzymes and low platelet count) is a multisystem disorder occurring in severe preeclampsia-eclampsia and is characterised by a microangiopathic haemolytic anaemia, hepatic dysfunction and thrombocytopenia. In many instances, this progresses to disseminated intravascular coagulation (DIC).
The HELLP syndrome affects an estimated 4% to 12% of patients with severe preeclampsia. Its presence is recognised by the onset of symptoms and signs of hepatic involvement such as epigastric pain, nausea and malaise, and on examination, right upper-quadrant tenderness. Because the HELLP syndrome is insidious in it’s onset, the early, milder form, may be missed unless appropriate laboratory assessment namely, FBC, LFT’s, urea and creatinine, LDH and DIC screen is performed. Thus the HELLP syndrome is often advanced before an accurate diagnosis is made.
The HELLP syndrome affects primiparous and multiparous women in the third trimester of pregnancy. Delivery of the foetus is the appropriate initial treatment. The syndrome remains active after delivery and appears to reach maximal intensity during the 24-48 hour post delivery period when thrombocytopenia, the ALT, AST and LDH all reach their peak. The pathogenesis is not clear. As a result of foetomaternal cell traffic, foetal products induce the release of agonists in the mother causing vascular endothelial damage and platelet activation. This induces DIC leading to fibrin deposition and obstruction to blood flow. Interference to flow in the liver leads to the high liver enzyme levels observed in the HELLP syndrome. Red cells are damaged as they pass through fibrin coated vessels resulting in haemolysis. Thrombocytopenia is due to both platelet consumption and destruction. There is a correlation between the platelet count and the severity of the HELLP syndrome. Platelet counts below 50 x 109/L are associated with greater maternal morbidity.
The blood picture in the HELLP syndrome is characterised by the presence of schistocytes together with thrombocytopenia. Summation at the end of the blood film report should always read ‘blood film consistent with a microangiopathic process’.
Plasmapheresis improves the HELLP parameters in non-responsive cases in the post-partum period. It removes a large variety of biologically active substances.