Hemophagocytic Lymphohistiocytosis in Pregnancy: A Case Series and Review of the Current Literature.

BACKGROUND: Hemophagocytic lymphohistiocytosis (HLH) is a rare disease that can be fatal in pregnancy. We report two cases of severe HLH that highlight etoposide use in pregnancy. CASE 1: 28-year-old G2P1 with lupus presented at 18 weeks with acute hypoxic respiratory failure, hepatic dysfunction, leukopenia, thrombocytopenia, and elevated ferritin. Bone marrow biopsy confirmed HLH. Etoposide and corticosteroid treatment was initiated per HLH protocol; however clinical status declined rapidly. Fetal demise occurred at 21 weeks and she subsequently suffered a massive cerebral vascular accident. She was transitioned to comfort measures and the patient deceased. CASE 2: 37-year-old G4P3 presented at 25 weeks with fever, acute liver failure, thrombocytopenia, and elevated ferritin. HLH treatment was initiated, including etoposide, and diagnosis confirmed with liver biopsy. Fetal growth restriction was diagnosed at 27 weeks. Delivery occurred at 37 weeks. The neonate was found to be CMV positive despite negative maternal serology. CONCLUSION: The addition of etoposide to corticosteroid use is a key component in HLH treatment of nonpregnant individuals. While this is usually avoided in pregnancy, the benefit to the mother may outweigh the potential harm to the fetus in severe cases and it should be strongly considered.

Undetected Severe Fetal Myelosuppression following Administration of High-Dose Cytarabine for Acute Myeloid Leukemia: Is More Frequent Surveillance Necessary?

BACKGROUND: Cytarabine use during pregnancy carries a 5-7% risk of neonatal cytopenia. We report two cases of fetal myelosuppression following high-dose cytarabine administration for acute myeloid leukemia (AML). CASE 1: A 36-year-old G9P6 diagnosed with AML at 21 weeks was monitored for fetal anemia weekly and growth monthly. At 33 weeks (after 2 cycles), BPP was 2/10 and MCA PSV was elevated at 1.51 MoM. Urgent cesarean section was performed. The infant had an initial pH of 6.78 and pancytopenia (hematocrit 13.3%, platelets 3 K/UL, and white blood cell count 2.0 K/UL). Initially transfusion dependent, the neonate had count recovery by 3 weeks. CASE 2: A 30-year-old G4P3 with AML at 26 weeks was monitored for fetal anemia twice weekly and growth monthly. At 34 weeks (after cycle 1), she was admitted with neutropenic fever. The fetal MCA PSV was borderline at 1.48 MoM. It improved to 1.38 MoM at 35 weeks but the fetal tracing worsened. At delivery the fetus was found to have a hematocrit of 30%, but with normal platelet and WBC. The fetus did not require any transfusions. CONCLUSION: Cytarabine use during pregnancy may cause neonatal myelosuppression. We recommend monitoring for fetal anemia with MCA Dopplers twice weekly.

Perimortem Demonstration and Treatment of Recipient-to-Donor Transfusion in Monochorionic-Diamniotic Twin Gestation.

Twin-twin transfusion syndrome is a complication of monochorionic-diamniotic placentation. Should one twin die, ≈30% of co-twins will also die, and if they survive, ≈30% experience severe morbidity rates, each believed secondary to hemorrhage of the co-twin into the deceased twin. We report apparently the first ultrasound-documented case of perimortem hemorrhage in twin-twin transfusion syndrome and its treatment by emergent ultrasound-guided percutaneous cord occlusion followed by percutaneous fetal intravascular transfusion. The case illustrates three important pathophysiologic events. First, it confirms acute twin-to-twin hemorrhage occurs and reveals it can begin before the first twin dies. Thus, delivery of the survivor after its dead co-twin is discovered is unlikely to protect the survivor. Second, the elevated fetal middle cerebral artery peak systolic velocity due to acute anemia requires hours to develop. And thirdly, intracardiac epinephrine can correct the acute fetal bradycardia associated with hemorrhage that is presumably due to fetal hypotension.

The frequency of prior antenatal corticosteroid therapy in late preterm birth pregnancies.

We sought to quantify how often women with late preterm birth (LPTB) receive antenatal corticosteroid (ACS) therapy prior to 34 weeks and to determine its effects on neonatal respiratory morbidity. LPTBs (34 (0)/ (7) to 36 (6)/ (7) weeks) over a 1-year period at a single tertiary care hospital were studied. A composite neonatal respiratory outcome was defined as mechanical ventilation, continuous positive airway pressure with fraction of inspired oxygen (F IO(2)) >40% for >2 hours or F IO(2) >40% for >4 hours within the first 72 hours of life. Multivariate logistic regression analysis was used to evaluate the association between ACS therapy and neonatal respiratory morbidity. Over the study period, 503 LPTBs met the study criteria and 6.8% ( N = 34) had ACS therapy <34 weeks. Most had exposure >7 days prior to delivery (64.7%). Almost one-half of those receiving prior ACS therapy delivered between 34 and 35 weeks. There was no difference in the rate of prior ACS therapy based on LPTB indication for delivery. After adjusting for confounding factors, prior ACS therapy was not associated with lower respiratory morbidity (odds ratio [OR] 2.0, 95% confidence interval [CI] 0.2 to 16.3, P = 0.53). Advancing gestational age was the only variable associated with respiratory morbidity (OR 0.50, 95% CI 0.26 to .94, P = 0.03). In our population, prior ACS therapy was infrequent and was not associated with improvements in neonatal respiratory morbidity following LPTB.

Approximately one-third of medically indicated late preterm births are complicated by fetal growth restriction.

OBJECTIVE: The purpose of this study was to report the frequency of fetal growth restriction (FGR) based on indication for late preterm birth (LPTB). STUDY DESIGN: Singleton live born pregnancies that were delivered from 34-36 weeks 6 days of gestation over a 1-year period at a tertiary care medical center were studied. Indications for delivery were categorized as spontaneous (spontaneous preterm birth or premature rupture of membranes), medically indicated, or elective. A customized birthweight percentile was calculated for each pregnancy; the rate of FGR was compared based on indication for LPTB. RESULTS: There were 482 LPTBs that met all criteria. Customized birthweight percentiles (median; interquartile range) were different among groups (spontaneous, 45.5%; 20.8-73.5%; medically indicated, 26.9%; 4.1-63.6%; elective, 45.9%; 22.2-78.3%; P = .001). The rate of FGR was also different among groups (spontaneous, 13%; medically indicated, 32%; elective, 21%; P = .001). CONCLUSION: With the use of customized birthweight standards, we found that FGR complicated approximately one-third of all cases of medically indicated LPTB.

Late preterm birth: how often is it avoidable?

OBJECTIVE: Our objective was to describe indications for late preterm birth (LPTB) and estimate the frequency of potentially avoidable LPTB deliveries. STUDY DESIGN: Singleton pregnancies delivered between 34(0/7)-36(6/7) weeks over a 1-year period at a tertiary care medical center were studied. Indications for delivery were categorized as spontaneous (spontaneous preterm birth or premature rupture of membranes) or iatrogenic (elective or medically indicated). Potentially avoidable deliveries were defined as those with elective or medical stable, but high-risk indications. RESULTS: During the study period there were 514 LPTB (spontaneous preterm birth 36.2%, preterm premature rupture of membranes 17.7%, medically indicated 37.9%, and elective 8.2%). Potentially avoidable LPTB accounted for 17% of LPTB and were associated with later gestational age (odds ratio [OR], 4.7; 95% confidence interval [CI], 2.5-8.6), nonfaculty physician status (OR, 2.8; 95% CI, 1.5-5.1), and prior cesarean delivery (OR, 1.5; 95% CI, 1.0-2.1). CONCLUSION: At our institution, <10% of LPTB are purely elective and >80% are clearly unavoidable.

Recurrent ectopic pregnancy in a cesarean scar.

BACKGROUND: Ectopic pregnancy in a cesarean scar is a rare but well-recognized potential complication of cesarean delivery. Multiple risk factors exist, including prior uterine surgery, a history of uterine infections such as endomyometritis, and a brief interval between uterine surgery and subsequent conception. It is important to recognize such cases early, due to the risk for uterine rupture and catastrophic hemorrhage at early gestational ages. CASE: This patient presented for a dating ultrasound examination at 4 6/7 weeks of gestation. Her history was significant for an ectopic pregnancy in her cesarean scar 3 years prior that was managed by surgical resection. The initial ultrasound examination was suspicious for a recurrent ectopic pregnancy in her cesarean scar. The diagnosis was confirmed on repeat ultrasonography at 6 weeks of gestation. She was treated with methotrexate, and the pregnancy resolved without complication. CONCLUSION: Ectopic pregnancy in a cesarean scar is an important diagnosis to consider in a woman who has had a history of cesarean delivery and whose early ultrasonography shows a thin, lower uterine segment or a low implantation site. If the diagnosis is not clear on initial ultrasound examination, the patient should be followed up with serial ultrasound examinations. Once recognized, patients with this complication may be treated either surgically or medically as indicated by the clinical situation.