Haemolytic anemia

  • 文章类型: Case Reports
    Hereditary spherocytosis is a rare cause of chronic haemolytic anaemia. It is rarer in the black population with extremely few cases reported. Initial assessment of a patient with suspected disease should include documenting clinical features of chronic haemolysis and a family history. Its management in pregnancy is challenging and needs a multidisciplinary team. We report on a black South African woman with hereditary spherocytosis and massive splenomegaly presenting with severe haemolytic anaemia at 23rd week of pregnancy. She had a successful splenectomy.
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  • 文章类型: Journal Article
    Congenital haemolytic anaemias are inherited disorders caused by red blood cell membrane and cytoskeletal protein defects, deviant hemoglobin synthesis and metabolic enzyme deficiencies. In many cases, although the causing mutation might be known, the pathophysiology and the connection between the particular mutation and the symptoms of the disease are not completely understood. Thus effective treatment is lagging behind. As in many cases abnormal red blood cell cation content and cation leaks go along with the disease, by direct electrophysiological measurements of the general conductance of red blood cells, we aimed to assess if changes in the membrane conductance could be a possible cause. We recorded whole-cell currents from 29 patients with different types of congenital haemolytic anaemias: 14 with hereditary spherocytosis due to mutations in α-spectrin, β-spectrin, ankyrin and band 3 protein; 6 patients with hereditary xerocytosis due to mutations in Piezo1; 6 patients with enzymatic disorders (3 patients with glucose-6-phosphate dehydrogenase deficiency, 1 patient with pyruvate kinase deficiency, 1 patient with glutamate-cysteine ligase deficiency and 1 patient with glutathione reductase deficiency), 1 patient with β-thalassemia and 2 patients, carriers of several mutations and a complex genotype. While the patients with β-thalassemia and metabolic enzyme deficiencies showed no changes in their membrane conductance, the patients with hereditary spherocytosis and hereditary xerocytosis showed largely variable results depending on the underlying mutation.
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  • 文章类型: Journal Article
    Thalassemias is the consequence of a synthesis imbalance between the α and β chains of hemoglobin. It\'s a hereditary haemolytic anemias, which presents a problem of public health because of their frequency and the difficulties of their care. The objective of this work is to study the epidemiological, clinico-biological, therapeutic and evolutionary profile of thalassemic patients in Morocco.
    We\'re reporting the results of a retrospective multicenter study on all cases of thalassemia taken at the hospitals: Avicenne military hospital in Marrakech, Mohammed VI hospital in Marrakech and the regional center for blood transfusion in Agadir.
    During this period we collected 81 cases of thalassemias. The average age was 13 years (from 1 month to 60 years) with a sex ratio (M/F) of 0.8. The reason for consultation was anemia in 43% of cases. The electrophoresis of hemoglobin has shown a predominance of the β-thalassemia minor form. On the evolutionary level, iron overload and endocrine complications were the most frequent.
    The development of a prevention program based mainly on health education, the reduction of consanguineous marriages and genetic counseling, are probably the main ways of combating the emergence of new thalassemic cases.
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  • 文章类型: Case Reports
    Methylene blue is utilized as the main treatment of methemoglobinemia. Here we report two cases, in which patient suffered from aniline-induced methemoglobinemia with initial good response but later developed haemolytic anemia due to methylene blue therapy. He was treated with hydration, high flow oxygen and steroid therapy. Caution should be exercised while using methylene blue as antidote of acute methemoglobinemia as both methemoglobinemia and its antidote methylene blue can itself precipitate fatal haemolytic anemia.
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  • 文章类型: Case Reports
    Campath is being employed for the treatment of autoimmune haemolytic anemia (AIHA) whether or not associated to B cell chronic lymphoid leukaemia (CLL). CD5 negative CLL is relatively uncommon and runs an indolent course. We report a CD5 negative CLL patient who developed AIHA associated with cytomegalovirus infection reactivation whilst on treatment with Campath for progressive disease.
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  • 文章类型: English Abstract
    OBJECTIVE: Drug-induced immune haemolytic anemia occurs in one case per million and can be fatal. Our aim was to describe the main characteristics and the type of drug involved.
    METHODS: Cases were retrospectively identified using spontaneous notifications collected by our pharmacovigilance centre and the results of immuno-hematological investigations performed by the laboratory of French blood establishment of Lyon between 2000 and 2012. Inclusion criteria were: an immune (positive direct antiglobulin test), hemolytic, anemia (haemoglobin <100 g/L), with at least a plausible causal relationship with drug exposure according to the French method of imputability or the presence of drug-dependent antibodies, and exclusion of other causes of hemolysis.
    RESULTS: Ten cases (5 men and 5 women, median age 54.4 years) were identified. Causal drugs were ambroxol, beta-interferon, cefotetan, ceftriaxone, loratadine, oxacillin, oxaliplatine, piperacillin-tazobactam, pristinamycin, and quinine. The median time to onset of anemia after starting the culprit drug was 6 days (2 hours to 16 days). The median nadir of hemoglobin was 57.9 g/L (range: 34-78). The direct antiglobulin test was positive in 8 patients: IgG only (n=4), IgG and complement (n=3), and IgA (n=1). Drug-induced immune haemolytic anemia was considered as definite in 5 cases with positive drug-induced antibodies, probable in 4 cases negative for the detection of drug-induced antibodies but with plausible or likely causal relationship with drug exposure, and probable with an autoimmune mechanism in 1 case.
    CONCLUSIONS: The diagnosis of DIIHA is often difficult because of the similarities with autoimmune haemolytic anemia and the inconstant sensitivity of immunologic tests that sometimes required repetitive assessment.
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  • 文章类型: Case Reports
    Two young women, were reffered to our hospital on two different occasions with history of breathlessness and mental confusion, following consumption of two different bio-organic plant nutrient compounds with a suicidal intent. On examination, they had cyanotic mucous membranes, and their blood samples showed the classic \'dark chocolate brown\' appearance. Work up revealed cyanosis unresponsive to oxygen supplementation and absence of cardiopulmonary abnormality. Pulse oximetry revealed saturation of 75% in case 1 and 80% in case 2, on 8 liters oxygen supplementation via face masks, although their arterial blood gas analysis was normal, suggestive of \"saturation gap\". Methemoglobinemia was suspected based on these findings and was confirmed by Carbon monoxide-oximetry (CO-oximetry). Methylene blue was administered and the patients showed dramatic improvement. Both the patients developed evidence of hemolysis approximately 72 hours following admission which improved with blood transfusion and supportive treatment. The patients were eventually discharged without any neurological sequalae.
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    文章类型: Case Reports
    A 26-year-old lady with the diagnosis of multiple sclerosis who had received interferon beta1-b for eleven months was visited in MS clinic of our hospital because of icter and fatigue. Laboratory tests showed anemia, indirect hyperbillirubinemia, increased LDH, positive direct and indirect coomb\'s tests, and increased reticulocyte count and percentage. Other causes of autoimmune hemolytic anemia (AHA) and pre-existing AHA in the patient were ruled out. After INF discontinuation, symptoms disappeared, hemoglobin increased, and indirect coomb\'s test became negative. We concluded that autoimmune hemolytic anemia should be considered in all MS patients who receive interferon beta1-b and present with symptoms and signs of anemia.
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  • 文章类型: Journal Article
    The objective of this case report is to highlight presentation, complications and treatment of metformin poisoning. Patient after ingestion of 45gms of metformin developed colicky abdominal pain, severe tachypnea and vomiting. He developed severe lactic acidosis, cardiac arrest, pancreatitis and hemolytic anemia which was treated with charcoal, sodium bicarbonate, early initiation of high volume continuous veno-venous hemofiltration and supportive therapy. Metformin poisoning is a rare presentation and we discuss course of events in the management of metformin poisoning and its associated complications.
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