1/142. 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.- - - - - - - - - - ranking = 1keywords = count (Clic here for more details about this article) |
2/142. Management of chronic myeloid leukemia during pregnancy with leukapheresis.We describe the successful treatment of a pregnant patient with chronic myelogenous leukemia in chronic phase by using only leukapheresis. Following 20 leukapheresis procedures initiated during the 13th week of gestation and performed over approximately 7 weeks, the patients white blood cell count dropped from 242,000/microl to 19,300/microl. The WBC remained stable over the ensuing 17 weeks until the time of delivery. The patient gave birth by cesarean section to a healthy 2,640 g boy at 37.5 weeks of gestation. This is the second report of the successful use of leukapheresis alone for chronic myelogenous leukemia in chronic phase during the first half of pregnancy. We conclude that where leukapheresis is available, it may provide an alternative treatment to chemotherapy or alpha-interferon, especially in light of their potential teratogenic and leukemogenic side-effects.- - - - - - - - - - ranking = 0.33333333333333keywords = count (Clic here for more details about this article) |
3/142. Spinal haematoma following epidural anaesthesia in a patient with eclampsia.A patient with a twin pregnancy required a Caesarean section for severe pre-eclampsia. Her platelet count was 71 x 10(9).l-1. Epidural anaesthesia was performed after platelet transfusion. A spinal epidural haematoma was diagnosed postoperatively. A generalised tonic-clonic seizure sparing the lower limbs enabled early diagnosis to be made. The patient recovered with no permanent neurological damage after laminectomy and clot removal. The risks and benefits of regional techniques require careful consideration, and postoperative monitoring for recovery of neural blockade is essential.- - - - - - - - - - ranking = 0.33333333333333keywords = count (Clic here for more details about this article) |
4/142. pregnancy in bone marrow failure syndromes: diamond-Blackfan anaemia and Shwachman-diamond syndrome.pregnancy in bone marrow failure syndromes has risk to mother and fetus. There are fewer than 30 reports of cases with diamond-Blackfan anaemia (DBA), and none with Shwachman-diamond syndrome (SD). We report two DBA and one SD cases. One DBA mother received transfusions intra-partum, and the other only post-partum. Both required caesarean sections (C-sections) for failure of labour to progress and severe pre-eclampsia respectively. Both subsequently resumed pre-pregnancy steroid-induced control of anaemia. approximately 40% of DBA pregnancies required maternal transfusions; 25% delivered by C-section. The SD patient also had Ehlers-Danlos (ED) syndrome and urticaria pigmentosa (UP). Her blood counts were adequate until week 38, when the platelet count dropped and a C-section was performed. pregnancy management in marrow failure disorders requires obstetricians with expertise in high-risk pregnancies, and haematologists with experience with marrow failure syndromes.- - - - - - - - - - ranking = 0.66666666666667keywords = count (Clic here for more details about this article) |
5/142. Glanzmann's thrombasthenia in pregnancy: a case and review of the literature.Glanzmann's thrombasthenia is a rare autosomal recessive bleeding disorder resulting from a deficiency of glycoprotein IIb-IIIa complex in platelets. The deficient complex normally mediates platelet aggregation by binding adhesive proteins, which form bridges between activated cells. Despite normal platelet counts, morphology, prothrombin, and activated thromboplastin times, Glanzmann's thrombasthenia is characterized by a prolonged bleeding time and a severe hemorrhagic mucocutaneous diasthesis. pregnancy and delivery are rare in these patients and have been associated with a high risk of severe hemorrhage. We present an unusual case in which a primi-gravida patient with Glanzmann's thrombasthenia underwent an uneventful pregnancy and spontaneous vaginal delivery, following intrapartum intravenous administration of single-donor platelets. Subsequent late postpartum hemorrhage required intravenous transfusion of an additional unit of single-donor platelets. In addition, we review the literature pertaining to pregnancy and Glanzmann's thrombasthenia with an emphasis on intrapartum prophylactic management.- - - - - - - - - - ranking = 0.33338047494467keywords = count, unit (Clic here for more details about this article) |
6/142. Hemorrhagic moyamoya disease during pregnancy.BACKGROUND: Intracranial hemorrhage in pregnant patients with moyamoya disease is rare. We review the case of one such patient who presented with pre-eclampsia and a catastrophic intracerebral hemorrhage in order to highlight the associated management difficulties. methods: A case of a pregnant (31 weeks) female brought to the emergency department with hypertension and a progressive decrease in her level of consciousness is presented. She rapidly developed a dilated right pupil and left extensor posturing. A CT scan of her head showed a large putamenal intracerebral hemorrhage. She was intubated, ventilated and given intravenous mannitol and magnesium sulfate. She underwent a simultaneous craniotomy and cesarean section. Post-operatively the patient's ICP and jugular venous saturation were monitored in the intensive care unit. RESULTS: The patient delivered a 1185 g infant who did well. The patient's ICP was well controlled until the tenth post-operative day when she developed malignant brain edema and died. CONCLUSION: This case highlights three important points. First, simultaneous craniotomy and cesarean section can be performed. Second, intraoperative control of bleeding Moyamoya vessels is described. Third, the difficult post-operative management of these cases is highlighted. The literature regarding moyamoya disease and pregnancy is reviewed and some recommendations for the management of this rare but potentially deadly condition are presented.- - - - - - - - - - ranking = 4.7141611338345E-5keywords = unit (Clic here for more details about this article) |
7/142. Acquired haemophilia - a study of ten cases.Clinico-haematological features in 10 patients with acquired Haemophilia are presented. Three patients had FVIII inhibitors following pregnancy while in six the cause for development of inhibitors could not be determined. One patient had acquired von Willebrand's disease. Lupus anticoagulant coexisted with factor viii inhibitors in three patients. All patients presented with sudden onset of bleeding without any past or family history of a bleeding disorder. factor viii inhibitor levels ranged from 8 to 512 Bethesda units in the nine patients. Immunosuppressive therapy was given to 8 patients, consisting of CVP regimen or corticosteroids with endoxan or cyclosporin. Seven patients had clinical and laboratory responses and one patient did not respond. One patient had severe postpartum bleeding with acute shock which was controlled with FEIBA. Diagnosis and management of idiopathic acquired Haemophilia, thus, continues to be a major challenge, and among acquired Haemophilia, postpartum Haemophilia has good prognosis.- - - - - - - - - - ranking = 4.7141611338345E-5keywords = unit (Clic here for more details about this article) |
8/142. Maternal blood coagulation factor XIII is associated with the development of cytotrophoblastic shell.We analysed the early implantation tissues of normal women and of a patient with congenital factor xiii deficiency in order to study the role of maternal subunit A of factor XIII (XIIIA) in the development of extravillous cytotrophoblast. The patient had received adequate administration of factor xiiia concentrate only up to 7 weeks of gestation (wG). Her pregnancy was maintained until the latter half of 8 wG, but was terminated by intrauterine fetal death at 9 wG. Immunohistochemical staining of cytokeratin, XIIIA and subunit S of factor XIII was performed in the early implantation tissues of normal women and of this patient. Numerous well-formed cytotrophoblastic shells and Nitabuch's layers were detected in implantation tissues at 7-8 wG in normal women, and XIIIA was present in the intercellular space in well-formed cytotrophoblastic shells, while the cytotrophoblastic shells and Nitabuch's layers in this patient's implantation tissue were poorly-formed. Furthermore, XIIIA was not detected around them. It is suggested that when the maternal plasma activity of factor XIII is low, the concentration of XIIIA at the placental bed is also low, leading to the insufficient formation of cytotrophoblastic shell and therefore an increased probability of miscarriage in patients with congenital factor xiii deficiency.- - - - - - - - - - ranking = 9.4283222676691E-5keywords = unit (Clic here for more details about this article) |
9/142. Paroxysmal nocturnal hemoglobinuria associated with in vitro inhibition of erythropoiesis by bone marrow T lymphocytes.BACKGROUND: The etiology of bone marrow failure, a prominent feature of paroxysmal nocturnal hemoglobulinuria, is presently unknown. OBJECTIVES: To evaluate the possible influence of cellular immune mechanisms in the bone marrow failure of PNH. methods: We studied marrow erythroid colony formation in a patient with paroxysmal nocturnal hemoglobinuria without hypoplastic/aplastic marrow complications. RESULTS: in vitro assays revealed a pronounced inhibition of primitive erythroid (BFU-E) progenitor cell growth by marrow T lymphocytes. Removal of T cells prior to culture resulted in a 4.5-fold enhancement of BFU-E numbers. Reevaluation of in vitro erythropoiesis during steroid administration indicated a persistent, albeit less prominent, T cell inhibitory effect. CONCLUSION: Our findings provide the first direct evidence for a cellular immune inhibitory phenomenon accompanying PNH.- - - - - - - - - - ranking = 5.1747345724697E-5keywords = assay (Clic here for more details about this article) |
10/142. A patient with cyclic neutropenia complicated by severe persistent neutropenia successfully delivered a healthy baby.We describe a 24-year-old pregnant woman complicated by cyclic neutropenia (CN), who was successfully treated with granulocyte-colony stimulating factor (G-CSF). Her white blood cell (WBC) and neutrophil count fluctuated from 2,600 to 4,600/microl, and 26 to 2,530/microl, respectively. The peak neutrophil count gradually decreased as pregnancy advanced, resulting in the disappearance of its cyclicity. At 39 weeks of pregnancy when the neutrophil count became 84/microl, the patient was started on G-CSF and her neutrophil count increased to 1,550/microl on the fourth day after delivery. She delivered a healthy baby without any complications at 39 weeks of pregnancy.- - - - - - - - - - ranking = 1.3333333333333keywords = count (Clic here for more details about this article) |
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