Postpartum plasma exchange as adjunctive therapy for severe acute fatty liver of pregnancy

Abstract

Acute fatty liver of pregnancy (AFLP) is a rare disease of progressive hepatic insufficiency and secondary systemic compromise that poses significant fetal-maternal risk. Plasma exchange (PEX) is an effective bridge therapy to sustain liver function and enable hepatocellular regeneration to occur in nonpregnant patients following acute decompensation of a chronic liver disease or while awaiting liver transplantation. The application of PEX for patients with AFLP is a novel concept; since 1988 we have utilized postpartum PEX (PPEX) as adjunctive medical therapy for six patients with severe AFLP. Before PPEX initiation, four patients had signs and symptoms of encephalopathy, three required ventilatory support, five had advanced liver insufficiency, and all six were developing renal failure. PPEX was initiated 2-8 days following delivery and repeated (two to four times, mean = 3) at 24-48-h intervals thereafter. All patients responded with composite clinical (symptoms/signs) and laboratory improvement; the average length of hospitalization following final PPEX for five of six patients was 7 days. No significant PPEX-related complications occurred. PPEX utilization in patients with severe AFLP may enhance maternal recovery by preventing secondary sequelae from hepatic insufficiency until spontaneous healing can occur. Further study appears to be indicated to validate a role for PPEX as supportive therapy for puerperal patients with AFLP suffering multiorgan failure.

Effectiveness of Combining Plasma Exchange with Plasma Perfusion in Acute Fatty Liver of Pregnancy: A Retrospective Analysis

“…Large clinical trials comparing different treatment strategies and patient outcomes have not been conducted, most likely due to the rarity of AFLP. Published data on patients diagnosed with AFLP are predominantly based on small case series that have utilized conventional medical supportive care [2,14,15]. However, PE combined with PP may be an effective strategy to treat AFLP and restore hepatic function.…”

“…In view of the underlying biochemical metabolic disorders with the accumulation of large amounts of toxic substances, some blood purification technologies have been applied to AFLP [2,3]. Plasma exchange (PE) has been used with success in a few severe cases of AFLP [4,5].…”

Early identification of women with HELLP syndrome who need plasma exchange after delivery

“…In patients with HELLP syndrome, there is no consensus about when to stop the procedure. When plasma exchange is used in the treatment of other diseases (mainly thrombotic thrombocytopenic purpura and hemolytic-uremic syndrome), the main goals are removal of some plasma components such as antibodies, immune complexes, and endogenous and exogenous toxins, and replacement of some plasma proteins and coagulation factors [8,[17][18][19][20][21]. The mechanism of PPEX in HELLP patients is unknown and controversial, but it might involve removal of aggregating and procoagulant factors released from both activated platelets and endothelial cells because HELLP is associated with severe endothelial dysfunction [8,22].…”

A life-saving therapy in Class I HELLP syndrome: Therapeutic plasma exchange

“…Plasmapheresis in theory can lead to the removal of ammonia, endotoxins, bilirubin, and inflammatory cytokines from the circulation. Also, injection of large volumes of FFP (fresh frozen plasma) in this method can help to improve the DIC, and removing renin angiotensin and other vasoactive factors may improve renal function [13]. All these advantages improve hepatic, renal and neurologic function in patients with HELLP syndrome.…”