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.     

Thrombotic thrombocytopenic purpura

A 34-year old primipara was admitted to hospital with dichorionic–diamniotic twins in 26+3 weeks of gestation. In suspicion of HELLP-syndrome, caesarean section was performed at 27+4 weeks of gestation, because of platelet count was reduced to 44000/µl. A re-laparotomy had to be performed because of intra-abdominal bleedings. The patient was given seven packed thrombocytes and five packed erythrocytes. The patient complained about blurred vision. The right corner of the mouth was slightly depressed in terms of a facial nerve paresis. Further platelet counts were about 50000/µl. Haemoglobin: 7.8 mg/l. D-Dimer: 1066 mg/l. LDH was elevated to 1610 U/l, reticulocytes were elevated to 13.19% and haptoglobin was reduced to <0.08 g/l. The Coombs’ test was negative. The ADAMTS-13 test showed a reduced activity. Hereby, the diagnoses of thrombotic thrombocytopenic purpura was confirmed. Plasma exchange is the most effective option; application of platelet concentrate should be avoided, because of worsening microangiopathy and subsequent neurological situation. After the patient has received plasma exchange, platelet count normalized. An ophthalmic examination showed a dysfunction in choroid perfusion as a cause for the blurred vision. After discharge regular lab tests were planned, but no further treatment was necessary at that point of time. The premature twins survived after ventricular haemorrhage, severe sepsis, bronchopulmonary dysplasia and other complications of neonates.     

Thrombotic thrombocytopenic purpura.

The patient reported here is the twentieth reported case of thrombotic thrombocytopenia purpura (TTP) occurring in pregnancy, the third survivor, and the first to be delivered by cesarean section. Diagnosis of TTP is usually made on the basis of the pentad of anemia, thrombocytopenia, renal disease, neurologic abnormalities, and fever. A peripheral smear showing microangiopathic hemolysis can be confirmatory and may even be regarded as a definitive test in questionable cases of thrombocytopenia. Although splenectomy, steroids, heparin, and early delivery have been recommended, splenectomy is the only treatment showing a statistically significant improvement in survival rate; 60% of infants born at or beyond 33 weeks' gestation have survived.     

妊娠合并血栓性血小板减少性紫癜

血栓性血小板减少性紫癜(thrombotic thrombocytopenic purpura, TTP)可发生于任何年龄,女性多见,妊娠是诱发因素之一。本文就TTP的发病机制、妊娠与TTP的相互关系、临床表现与分类、诊断与鉴别诊断、治疗以及TTP患者妊娠前咨询等方面进行讨论。     

Thrombotic thrombocytopenic purpura in pregnancy

Summary. A 20 week primigravida with intrauterine fetal death and the rare occurrence of thrombotic thrombocytopenic purpura (TTP) in pregnancy is presented. Aprotinin has been used to overcome uterine inertia non-responsive to oxytocin stimulation. TTP was successfully treated with repeated plasmaphereses. The literature is briefly reviewed and the complexity and importance of the differential diagnosis specific to the pregnant patient is discussed.     

Thrombotic thrombocytopenic purpura in early pregnancy

Thrombotic thrombocytopenic purpura during pregnancy in which both mother and fetus have survived has been an exceedingly rare occurrence, due at least in part to the difficulty in distinguishing this uncommon disease from hematologic complications of preeclampsia. In the nonpregnant patient, the use of plasma infusion or exchange plasmapheresis as therapy of choice has resulted in a dramatic increase in survival. By using more specific criteria than the classical clinical pentad, the diagnosis of thrombotic thrombocytopenic purpura during pregnancy can be made with greater accuracy. The first known instance of the successful use of exchange plasmapheresis leading to the survival of both mother and fetus in thrombotic thrombocytopenic purpura clearly not secondary to preeclampsia is reported.     

Thrombotic thrombocytopenic purpura in a newborn.

This report describes a newborn who presented with hyperbilirubinemia and thrombocytopenia. The patient recovered after treatment with antibiotics, phototherapy, and a platelet transfusion. Analysis of the plasma von Willebrand factor-cleaving metalloprotease, ADAMTS13, revealed low protease activity in the patient and her two siblings, and a mild deficiency in both parents. These results confirmed the clinical suspicion of hereditary thrombotic thrombocytopenic purpura (TTP). Although most cases of thrombocytopenia and hyperbilirubinemia in the newborn period are caused by other causes, genetic deficiency of ADAMTS13 may not be as uncommon as previously believed. Early diagnosis may have important implications for the patients.     

Thrombotic thrombocytopenic purpura first seen as massive vaginal necrosis

Thrombotic thrombocytopenic purpura is a hematologic disorder that affects the microcirculation. A 38-year-old woman was first seen with fever, thrombocytopenia, and vaginal bleeding. Pelvic examination revealed massive vaginal necrosis. Hematology consultation resulted in agreement with the diagnosis of thrombotic thrombocytopenic purpura. This is the first reported case of thrombotic thrombocytopenic purpura first seen as vaginal necrosis of which we are aware.     

Thrombotic thrombocytopenic purpura in pregnancy. A case report

Thrombotic thrombocytopenic purpura (TTP) is characterized by microangiopathic hemolytic anemia and thrombocytopenia, accompanied by microvascular thrombosis that causes variable degrees of tissue ischemia and infarction. About 10-20% of TTP cases are associated with the pregnancy. Preterm delivery and intrauterine fetal death are frequent pregnancy complications of TTP. The following paper presents the case of a 32-year-old woman with TTP relapse at 10 weeks of her second pregnancy. Despite regular fresh frozen plasma transfusions, intrauterine fetal death occurred at 21 weeks of gestation. Current views on TTP management during pregnancy have been presented in the article as well.     

Immunologic thrombocytopenic purpura and pregnancy: six new cases.

Six new cases of idiopathic thrombocytopenic purpura (ITP) associated with pregnancy are reported; one of these patients delivered triplets. In addition, 78 patients with 122 pregnancies and 124 fetuses have been reported previously. The maternal mortality rate was 4% and the fetal mortality rate was 18%. Prior recommendations for elective cesarean section in all mothers with a platelet count less than 100,000/cu mm at delivery is not supported by the data available. Treatment of pregnant women with ITP with adrenal corticosteroids has virtually eliminated maternal mortality, has replaced splenectomy as the therapy of choice during pregnancy, but has had little effect on fetal mortality.