Hemolysis can be severe and evidenced by leukocytosis, nucleated red cells, mild to moderate thrombocytopenia, and elevated serum levels of lactic acid dehydrogenase (LDH). Because of hemoconcentration, however, the hematocrit is often within the normal range. The peripheral blood smear demonstrates echinocytosis, and hemolysis is thought to stem from efects of hypocholesterolemia on erythrocyte membranes (Cunningham, 1985).
The degree of clotting dysfunction also varies and can be serious and life threatening, especially if operative delivery is undertaken. Coagulopathy is caused by diminished hepatic procoagulant synthesis, although some evidence supports increased consumption from disseminated intravascular coagulopathy. As shown in Table 55-2, hypoibrinogenemia sometimes is profound. Of 51 women with fatty liver cared for at Parkland Hospital, almost a third had a plasma fibrinogen level nadir to < 100 mg/dL (Nelson, 2014). Modest level elevations of serum D-dimers or fibrin-split products indicate an element of consumptive coagulopathy. Although usually modest, occasionally thrombocytopenia is marked (see Table 55-2). Again, among the
TABLE 55-2. Laboratory Findings in 215 Women with Acute Fatty Liver of Pregnancy
Series No. Fibrinogen (mg/dL) Platelets (103/.LL) Creatinine (mg/dL) AST (U/L)
Pereira (1n997) 32 ND 123 (26-262) 2.7 (1n.1n-8.4) 99 (25-91n1)
Fesenmeier (2005) 16 ND 88 (22-226) 3.3 (0.5-8.6) 692 (122-3195) aFibrinogen and platelet values listed reflect the nadir for each patient, whereas creatinine and AST values reflect peak values for each patient.
AST = aspartate transaminase; NO = not done.
group from Parkland Hospital, 20 percent had platelet counts < 100,000/�L and 10 percent had platelet counts <50,000/�L (Nelson, 2014).
Various liver imaging techniques have been used to confirm the diagnosis, however, none are particularly reliable. Speciically, Castro and associates (1996) reported poor sensitivity for conirmation by sonography-three of 11 patients, computed tomography (CT)-five of 10, and magnetic resonance (MR) imaging-none of ive. Similarly, in a prospective evaluation of the Swansea criteria proposed by Ch'ng and coworkers (2002), only a quarter of women had classic sonographic findings that include maternal ascites or an �chogenic hepatic appearance (Knight, 2008). Our experiences are similar (Nelson, 2013).
The syndrome typically continues to worsen after diagnosis.
Hypoglycemia is common, and obvious hepatic encephalopathy, severe coagulopathy, and some degree of renal failure each develop in approximately half of women. Fortunately, delivery arrests liver function deterioration.
We have encountered several women with a orme fruste of this disorder. Clinical involvement is relatively minor and laboratory aberrations-usually only hemolysis and a decreased plasma fibrinogen level-herald the syndrome. hus, the spectrum of liver involvement varies from milder cases that go unnoticed or are attributed to preeclampsia, to overt hepatic failure with encephalopathy.