A high LDH to AST ratio helps to differentiate pregnancy-associated thrombotic thrombocytopenic purpura (TTP) from HELLP syndrome.

OBJECTIVE: Differentiating between pre-eclampsia/HELLP syndrome and pregnancy-associated thrombotic thrombocytopenic purpura (TTP) is difficult but important in order to undertake timely and potentially life-saving plasma exchange (PEX) therapy for TTP recovery. We review our institutional experience with pregnancy-associated TTP and determine if the ratio of LDH to AST reliably distinguishes patients with TTP from those with HELLP syndrome. STUDY DESIGN: This is a retrospective case control study of all pregnant/puerperal patients with TTP from a single tertiary care center during 1986-2006. Laboratory findings in patients with TTP were compared to patients who met all criteria for class 1 or 2 HELLP syndrome within the first 24 hours of hospital admission during 2000-2007. RESULTS: Thirteen pregnant (n = 10) or puerperal (n = 3) patients with TTP were identified; 11 cases were primary, 2 were recurrent. TTP laboratory findings included LDH to AST ratios of 77 ± 42.17; Patients with HELLP syndrome (N = 83) had significantly lower LDH to AST ratios of 20.04 ± 2.13. Based on an ROC analysis, an LDH/AST ratio ≥ 22.12 discriminates well between TTP and antenatal HELLP subjects (AUC = 0.99). CONCLUSION: A high LDH to AST ratio >22.12 suggests that TTP is a more likely diagnosis than HELLP syndrome in the third trimester pregnant patient, presenting with findings that could be compatible with either diagnosis. In these circumstances, it is advisable to obtain hematology consultation and to consider PEX implementation.

Thrombotic thrombocytopenic purpura in 166 pregnancies: 1955-2006.

A review of pregnancy-associated thrombotic thrombocytopenic purpura (TTP) in 166 pregnancies was undertaken using 92 English-language publications from 1955 to 2006. Initial and recurrent TTP presents most often in the second trimester (55.5%) after 1-2 days of signs/symptoms; postpartum TTP usually occurs following term delivery. TTP with preeclampsia (n = 28) exhibits 2-4 times higher aspartate aminotransferase (AST) values and lower total lactate dehydrogenase (LDH) to AST ratios (LDH to AST ratio = 13:1), compared with TTP without preeclampsia (LDH to AST ratio = 29:1). Maternal mortality is higher with initial TTP (26% vs 10.7%), especially with concurrent preeclampsia (44.4% vs 21.8%, P < .02). Although maternal mortality with TTP has substantially declined when plasma therapy is utilized, delay of diagnosis and therapy for initial TTP confounded by preeclampsia/hemolysis, elevated liver enzymes, and low platelets (HELLP) syndrome remains a significant maternal-perinatal threat. Rapid and readily available laboratory testing to quickly diagnose TTP and HELLP syndrome/preeclampsia is desperately needed to improve care.

Pregnancy after microinsert sterilization with tubal occlusion confirmed by hysterosalpingogram.

BACKGROUND: Introduced to the U.S. market in late 2002 as a permanent method of contraception, a microinsert device is placed hysteroscopically into the fallopian tubes, not requiring incisions or general anesthesia. This report describes a case of pregnancy more than 6 months after a hysterosalpingogram (HSG) confirming bilateral occlusion after microinsert sterilization. CASE: A 30-year-old gravida 1 para 1 woman desired permanent sterilization. The patient underwent microinsert device placement and 6 months later had an HSG that confirmed bilateral tubal occlusion. More than 6 months after the confirmatory HSG, the patient became pregnant and delivered a term infant by cesarean birth. Cornual perforation was noted at surgery. CONCLUSION: This case illustrates pregnancy after microinsertion sterilization and an HSG confirming bilateral tubal occlusion, despite perforation. A microinsert device continues to be a viable option for sterilization.

Prenatal diagnoses and outcomes of congenital diaphragmatic hernia.

OBJECTIVE: To describe the prenatal diagnoses and clinical outcomes of congenital diaphragmatic hernia (CDH). METHODS: A retrospective case series was developed by reviewing 16,983 ultrasounds performed between March 2003 and January 2006 for the prenatal diagnosis of CDH. Medical records of each mother/infant pair were reviewed for demographic information, ultrasound findings, obstetric management, and outcomes. RESULTS: Nineteen fetuses were diagnosed with CDH. Only one was lost to follow-up. Median gestational age at diagnosis was 28.4W (range 17.6-36.6). Fifteen cases (79%) were left sided, 3 (16%) were right-sided, and 1 (5%) was bilateral. Seven fetuses (39%) had additional abnormalities, the most common being a single umbilical artery. Ten patients (52.6%) underwent amniocentesis for karyotype; none were aneuploid. Three patients developed hydramnios. All 18 infants were liveborn. Seven infants (39%) died shortly after birth, 6 (33%) underwent surgery with subsequent discharge, and 5 (28%) were transferred to another center. Three of these died after transfer. CONCLUSION: Prenatal diagnosis of CDH portends a poor prognosis. Thirty-nine percent of infants with this diagnosis (7/18) did not survive to undergo surgery or transfer to another facility and overall mortality was 56% (10/18). Targeted ultrasonography, extensive counseling of parents, and delivery at a tertiary care center is recommended.

Thrombotic thrombocytopenic purpura masquerading as hemolysis, elevated liver enzymes, low platelets (HELLP) syndrome in late pregnancy.

BACKGROUND: Thrombotic thrombocytopenic purpura rarely presents during late pregnancy or immediately postpartum. This report describes the clinical course of a patient considered to have hemolysis, elevated liver enzymes, low platelets (HELLP) syndrome but later determined to have thrombotic thrombocytopenic purpura. CASE: At 37 weeks of gestation, a multiparous woman was diagnosed with HELLP syndrome. She received high-dose dexamethasone, magnesium, antihypertensives, and platelets before delivery. Over the next 36 hours, renal function acutely worsened and death ensued. One week after death a plasma ADAMTS13 activity of 4% was reported. CONCLUSION: Thrombotic thrombocytopenic purpura can mimic HELLP syndrome late in gestation. Lack of response to dexamethasone within 12-24 hours and atypical relationships among laboratory values are two clues that thrombotic thrombocytopenic purpura may be the underlying pathology and that plasma exchange is emergently needed.