1/79. 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.- - - - - - - - - - ranking = 1keywords = anaemia (Clic here for more details about this article) |
2/79. 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.- - - - - - - - - - ranking = 0.2keywords = anaemia (Clic here for more details about this article) |
3/79. Prognostic factors and management in pregnancies complicated with severe kell alloimmunization: experiences of the last 13 years.Because of the recent referral of an anti-Kell sensitized pregnant woman, whose fetus became severely anemic despite intensive antepartum surveillance, the prevalence and characteristics of fetal Kell isoimmunization were reviewed and analyzed. Cases with Kell and RhD alloimmunization requiring intrauterine intravascular transfusions (IUT) at the Mount Sinai Medical Center during the 13-year period ending March 1998 were compared. Thirty-six fetuses with RhD and 5 with Kell isoimmunization required IUTs. Lower fetal and neonatal hematocrit levels were observed in the RhD group. A significantly higher incidence of polyhydramnios was found among fetuses with Kell isoimmunization and the maternal serum titers were much lower than those in the RhD group. DeltaOD450 values did not reliably reflect the Kell sensitized fetus's condition. There were no intrauterine deaths or neonatal direct hyperbilirubinemia in the Kell group, and the hemolytic disease of the newborn was more severe in the RhD group. Although the course of the hemolytic disease in our cases of Kell isoimmunization showed a better prognosis than that in the RhD group, the importance of this condition should not be underestimated, and differences in the pathophysiology of Kell and RhD alloimmunization should be taken into consideration during the management of these cases.- - - - - - - - - - ranking = 0.97788403622169keywords = hemolytic (Clic here for more details about this article) |
4/79. Death due to thrombotic thrombocytopenic purpura in pregnancy: case report with review of thrombotic microangiopathies of pregnancy.maternal death during pregnancy, although uncommon, may result from a broad range of conditions. In this paper, a case of thrombotic thrombocytopenic purpura diagnosed by postmortem examination is presented. Thrombotic thrombocytopenic purpura is one of a subset of diseases that result in the formation of microthrombi within the vasculature, either as a primary or secondary manifestation. Other conditions included in the differential diagnosis during pregnancy are hemolytic uremic syndrome, systemic lupus erythematosus, preeclampsia-eclampsia and the hellp syndrome, acute fatty liver of pregnancy, antiphospholipid antibody syndrome, and disseminated intravascular coagulation. The histologic manifestations of these diseases can be similar and in most cases do not provide adequate information to accurately differentiate these diseases in the postmortem period. This paper addresses the need for clinical history (i.e., symptomatology, trimester of onset) and antemortem laboratory testing in addition to a thorough autopsy to accurately differentiate among the conditions named previously. In the absence of an adequate clinical history and antemortem laboratory testing, the more general diagnosis of "thrombotic microangiopathy of pregnancy" is acceptable.- - - - - - - - - - ranking = 0.48894201811085keywords = hemolytic (Clic here for more details about this article) |
5/79. 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 = 1.2keywords = anaemia (Clic here for more details about this article) |
6/79. Sickle cell disease in pregnancy.Homozygous sickle cell anaemia (Hb S) is the most common major haemoglobinopathy in the united states, occurring in approximately 1 in 626 african americans. While haemoglobinopathies involving Hb S occur commonly in blacks of African descent, they are also found in people of Middle Eastern, East Indian and Mediterranean origin. It is an uncommon disease, especially in homozygous form, in australia. We present the case of a woman in her third pregnancy, originally from ghana, with HB F, and discuss the current issues in the management of sickle cell disease in pregnancy.- - - - - - - - - - ranking = 0.2keywords = anaemia (Clic here for more details about this article) |
7/79. Aplastic anaemia during pregnancy: variable clinical course and outcome.Aplastic anaemia occurring during pregnancy is a rare event and is associated with high maternal and fetal morbidity and mortality. prognosis is poorer when aplastic anaemia develops during pregnancy and many such patients have an unsuccessful pregnancy outcome. We report two cases of aplastic anaemia associated with pregnancy. Both the patients had very different antenatal course and outcome. Their etiopathogenesis and management is discussed.- - - - - - - - - - ranking = 1.4keywords = anaemia (Clic here for more details about this article) |
8/79. Labor analgesia in a patient with paroxysmal nocturnal hemoglobinuria with thrombocytopenia.BACKGROUND AND OBJECTIVES: Paroxysmal nocturnal hemoglobinuria (PNH) is a form of acquired hemolytic anemia in which a defect of glycophosphatidylinositol anchor proteins in the cell membrane of bone marrow stem cells leads to activation of the complement system and consequent destruction of defective cells. The characteristics of this disease are an increased frequency of thrombotic events, anemia, and thrombocytopenia. methods: We report a case of a pregnant patient with PNH with thrombocytopenia who delivered vaginally after receiving epidural labor analgesia. Prophylaxis of thromboembolism was performed with heparin 1 hour after the removal of the epidural catheter, and repeated at 12-hour intervals. Sensory changes or motor changes and pain were monitored every 10 minutes for 8 hours after delivery. RESULTS: During analgesia, the patient reported complete pain relief. Delivery and the immediate postpartum period were without any untoward events. CONCLUSIONS: Four major factors influenced the anesthetic conduct used for the present patient: (1) the risk of an acute hemolytic crisis, (2) the need to perform prophylaxis for thromboembolism, (3) the need to reduce labor stress, and (4) minimizing the risk of missing an epidural hematoma. We also present a survey of the literature about PNH and discuss the anesthetic conduct in this patient.- - - - - - - - - - ranking = 0.97788403622169keywords = hemolytic (Clic here for more details about this article) |
9/79. Hyperhemolytic transfusion reaction in sickle cell disease.BACKGROUND: An atypical form of life-threatening hemolytic transfusion reaction (HTR) in patients with sickle cell disease (SCD) has been well described in the literature. Continuation of blood transfusion may be lethal, as it can further exacerbate hemolysis. The pathophysiologic mechanism of HTR is not well understood. case reports: Two cases of severe HTR in SCD after the transfusion of compatible RBC units are reported. hemolysis of both autologous and transfused cells was documented in Case 1 by urine Hb high-performance liquid chromotography. Multispecific HLA antibodies were identified in Case 1. Reticulocytopenia was noted in both cases during the acute hemolytic process. This was followed by a rise in reticulocyte count during receipt of IVIG and steroid therapy. bone marrow examination during reticulocytopenia in Case 2 showed erythroid hyperplasia. CONCLUSION: In SCD, both mature sickle cells and sickle reticulocytes adhere more readily to macrophages. In view of the bone marrow aspiration results, it appears that the recipients' HbS cells are destroyed by hyperactive macrophages and that the reticulocytopenia observed during HTR is likely to be due to peripheral consumption (i.e., destruction by macrophages), rather than suppression of erythropoiesis. Cessation of hemolysis during IVIG and steroid treatment may be due to IVIG's blocking of the adhesion of sickle cells and reticulocytes to macrophages, together with steroid suppression of macrophage activity.- - - - - - - - - - ranking = 2.9336521086651keywords = hemolytic (Clic here for more details about this article) |
10/79. Hb Mont Saint Aignan [beta128(H6)Ala-->Pro]: a new unstable variant leading to chronic microcytic anemia.Hb Mont Saint-Aignan [beta128(H6)Ala-->Pro] is a mildly unstable variant, associated with hemolytic anemia, marked microcytosis and increased alpha/beta biosynthetic ratio (1.55 versus 1.1 /- 0.1 in the control). The abnormal chain was isolated by selective precipitation with isopropanol and the structural modification determined by protein chemistry methods (reversed phase high performance liquid chromatography and mass spectrometry). Possible mechanisms underlying the beta( )-thalassemia-like expression of this variant are discussed.- - - - - - - - - - ranking = 0.48894201811085keywords = hemolytic (Clic here for more details about this article) |
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