The decidual atherosis and fibrinoid necrosis seen in this case are are typical of both maternal PIH and antiphospholipid antibody syndrome. The placental intervillous fibrin deposition and focal placental infarction are also consistent findings. As a consequence, there was decreased placental function (uteroplacental insufficiency) leading to fetal intrauterine stress as evidenced by the microscopic findings of placental dysmaturity and villous fibrosis, thymic lymphocyte depletion, and extramedullary hematopoiesis of the adrenals, stomach, and lungs. Oligohydramnios may also result from uteroplacental insufficiency. Oligohydramnios in this case was suggested not only by ultrasonography, but also by the autopsy gross findings consisting of deformations from constriction in utero, including contractures of the elbow and wrist, varus deformity of the foot, displaced thumb, and upturned nose. There were no gross congenital anomalies found at autopsy.
Antiphospholipid antibody, or "lupus anticoagulant", can be a fairly frequent laboratory finding in healthy pregnant women. The antibody, either IgG or IgM, interferes with phospholipid-dependent coagulation assays, thus prolonging the partial thromboplastin time (PTT). However, due to the occasional presence of other autoantibodies that impair regulation of hemostasis, there can be an increased risk for thrombosis in some cases. This was the cause for the deep venous thrombosis in this case. A spectrum of autoantibodies that recognize different lipid-protein complexes may develop in pregnant patients and contribute to the observed clinical heterogeneity of the syndrome. Currently, no laboratory tests are available to identify the subset of patients with antiphospholipid antibodies at risk for thrombosis or abortion. Therapy is preventive and based on a cautious choice from three drugs: aspirin, heparin, and corticosteroids.
The HELLP syndrome is characterized by maternal hemolysis, elevated liver enzymes, and low platelets, all of which were present in this case. The reported incidence of HELLP has ranged from 2 to 15 percent. The HELLP syndrome is more frequently observed among preeclamptic/eclamptic multiparous and older patients. Patients present with abdominal pain, nausea and vomiting, malaise, hematuria, or even gastrointestinal bleeding. Although hypertension and proteinuria, the "classical" manifestations of preeclampsia, are usually present, the absence of signs of preeclampsia and characteristic laboratory findings, and the onset of symptoms less than one week before presentation can make diagnosis difficult or cause a delay in the diagnosis of HELLP. The HELLP syndrome can be associated with a poor maternal and perinatal outcome. Fetal growth retardation and intrauterine asphyxia are common. Life-threatening symptoms in the mother often lead to premature vaginal or cesarean delivery. Maternal mortality ranges from 0 to 24 percent and perinatal mortality from 7.7 to 60 percent.
Benirschke K and Kaufmann R. Pathology of the Human Placenta, 2nd edition. Springer-Verlag, New York, PP. 500-11, 1990.
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Schroder W, Heyl W. HELLP-syndrome. Difficulties in diagnosis and therapy of a severe form of preeclampsia. Clin Exp Obstet Gynecol. 1993;20:88-94.
Benirschke K, Kaufmann R. Pathology of the Human Placenta, 2nd edition. Springer-Verlag, New York, 1990, pp 500-510.
Reubinoff BE and Schenker JG. HELLP syndrome -- a syndrome of hemolysis, elevated liver enzymes and low platelet count -- complicating preeclampsia-eclampsia. Int J Gynecol Obstet 36:95-102, 1991.