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1/673. Two consecutive pregnancies and deliveries in a patient with von Willebrand's disease type 3.

    pregnancy and delivery are critical events in women with von Willebrand's disease type 3. Prophylactic treatment for delivery and early postpartum period is recommended. Vaginal delivery is considered safe. However, experience is based on rare case reports. We report the management of two pregnancies and successful deliveries in a woman with von Willebrand's disease type 3.
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2/673. Management of a premature infant with moderate haemophilia A using recombinant factor viii.

    The birth of a very premature infant with haemophilia A is a rare event. In this case report the problems posed in the management of a child with a factor viii level of 0.03 IU mL-1 born at 28 weeks of gestation and weighing 1590 g are considered. The value of recombinant factor viii, the pharmacokinetics of factor VIII in this situation and the importance of close cooperation between paediatricians and haematologists are discussed.
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3/673. Virtual consult--pregnant woman with sickle-cell disease.

    A young woman in her 20s with a lifelong history of sickle-cell disease presents with sickle-cell crisis while in active labor. After delivery of the neonate via cesarean section, her fever spikes to nearly 106 F (41.1 C) and stays there. Clinicians are invited to comment on this case in a discussion moderated by Joseph Pastorek, MD, FACOG.
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4/673. Thrombotic risk in thalassemic patients.

    Hemostatic parameters of 495 beta-thalassemic patients (421 with thalassemia major and 74 with thalassemia intermedia) were analyzed, to assess their association with the described thrombophilic condition and to verify the role of additional risk factors (e.g. persistent postsplenectomy thrombocytosis, insulin dependent diabetes mellitus, estrogen-progestin treatment and atrial fibrillation). The prevalence of thromboembolic accidents was 5.2% and in four patients (15.3%) inherited or acquired predisposing defects were recognized. The incidence of thromboembolic events and the associated relative risk due to hemocoagulative abnormalities in these patients are discussed.
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5/673. Factor V Leiden and antibodies against phospholipids and protein S in a young woman with recurrent thromboses and abortion.

    We describe the case of a 39-year-old woman who suffered two iliofemoral venous thromboses, a cerebral ischemic infarct and recurrent fetal loss. Initial studies showed high levels of antiphospholipid antibodies (APAs) and a moderate thrombocytopenia. After her second miscarriage, laboratory diagnosis revealed that the woman was heterozygous for the factor V Leiden mutation and had a functional protein s deficiency as well as anti-protein S and anti-beta 2-glycoprotein i antibodies. The impairment of the protein c pathway at various points could well explain the recurrent thromboses in the patient and supports the role of a disturbed protein c system in the pathophysiology of thrombosis in patients with APAs.
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keywords = deficiency, v
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6/673. A case of aplastic anaemia in pregnancy.

    Aplastic anaemia in pregnancy is an extremely rare condition with high maternal morbidity and mortality rates. Intensive haematological support remains the mainstay of therapy and a successful obstetric outcome can be best accomplished with the close clinical collaboration of the haematologist and the obstetrician as occurred with our patient reported here.
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7/673. Hereditary elliptical stomatocytosis: a case report.

    The case described demonstrates the development of elliptical stomatocytosis in a neonate and the appearance of elliptical stomatocytes in her mother whose blood film, before delivery, showed elliptocytosis. Further investigations on both individuals indicated a mild haemolytic anaemia. To our knowledge this is the second reported case of elliptical stomatocytosis.
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8/673. Low-molecular-weight heparin: more indications for use.

    The initial studies of low-molecular-weight heparin in the treatment of deep-vein thrombosis excluded pregnant women and patients with acute pulmonary embolism or a known hypercoagulable disorder. However, none of these needs to be a contraindication, and outpatient treatment is possible, provided that proper patient selection and follow-up are implemented.
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9/673. Acute pancreatitis and deep vein thrombosis associated with hellp syndrome.

    The hellp syndrome (HS) belongs to the list of obstetric complications believed to be associated with coagulation disorders. It was formerly thought that chronic intravascular clotting (DIC) in the placental vessels was the main cause. A hypercoagulable state has been reported in cases of severe HS associated with microvascular abnormalities that may involve cerebral, placental, hepatic and renal vessels. A case of acute pancreatitis and DVT of inferior cava in a pregnant woman, presenting with HS at 29 weeks, who was found to have a R506Q mutation, is reported. Preeclampsia-associated pancreatitis and DVT have rarely been reported. It is hypothesized that APC-R and Factor V Leiden mutation may prove to be new and more important markers capable of predicting a more significant maternal morbidity associated with HS. thrombosis prophylaxis may be considered during pregnancy in order to reduce hazardous multiorgan failure (MOF) in women who are heterozygous for Factor V Leiden mutation.
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10/673. Serial serum thrombopoietin levels in a pregnant woman with essential thrombocythaemia.

    thrombopoietin (TPO) is a ligand for c-mpl, which regulates the differentiation and maturation of megakaryocytes. Essential thrombocythaemia (ET) is a clonal myeloproliferative disorder. It has been reported that the platelet count declines during pregnancy in ET patients. We examined serial changes in the serum TPO level during the course of pregnancy in a patient with ET. The serum TPO level showed significant negative correlation with the platelet count. Although it mimicked the normal feed back system, the TPO levels were consistently higher than the normal upper limit. Accumulation of these data will be helpful in revealing the pathogenesis of ET and the decline in the platelet count during pregnancy.
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