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1/7. Major colorectal surgery in a patient with cold agglutinin disease.

    We present the case of a 62-year-old man with severe cold agglutinin disease who underwent major colorectal surgery. Cold agglutinin disease is a condition in which auto-antibodies, usually immunoglobulin m, cause red blood cell agglutination at decreased body temperature. Haemolysis may result. agglutination results in impaired perfusion, resulting in symptomatic Raynaud's phenomenon and acrocyanosis. Haemolysis can result in anaemia and thrombotic events caused by microvascular occlusion, in addition to haemoglobinuria and renal failure. Peri-operative hypothermia is common in all patients and may be associated with significant morbidity, but is potentially catastrophic in a patient suffering from cold agglutinin disease.
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2/7. triamterene-induced immune haemolytic anaemia with acute intravascular haemolysis and acute renal failure.

    Acute intravascular haemolysis and renal failure developed while a patient was taking triamterene. A direct antiglobulin test with a polyvalent reagent was positive. serum caused agglutination of normal red cells in the presence of triamterene and caused an increase of partial haemolysis of both trypsin-treated erythrocytes and red cells from a patient with paroxysmal nocturnal haemoglobinuria (PNH) in the presence of complement. From the results of antibody-neutralization test and treatment with 2-mercaptoethanol, the presence of IgM antibody with lambda light chain could be demonstrated. The triamterene seemed to bind strongly to the red cells in vitro but in vivo there was no detectable adsorption to red cells. Haptenic inhibition was not demonstrated. From these results, it was assumed that this antibody was found to cross-react with methotrexate which has a structure similar to that of triamterene.
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keywords = haemoglobinuria
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3/7. Paroxysmal cold haemoglobinuria. The most frequent acute autoimmune haemolytic anaemia in children?

    Paroxysmal cold haemoglobinuria (PCH) is a disease which today is met mainly in its acute form in children. The diagnosis is revealed by a careful serological examination. case reports of four children with PCH are given in this paper. They all showed typical clinical pictures of PCH, and displayed the expected serological findings, including a bithermic autoantibody (haemolysin), the Donath-Landsteiner antibody. Children with PCH often require immediate transfusion therapy, but certain precautions should be taken regarding transfusion policy in these patients. When the acute phase is overcome, the prognosis is excellent.
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keywords = haemoglobinuria
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4/7. sarcoidosis associated with autoimmune haemolytic anaemia and paroxysmal nocturnal haemoglobinuria red cell abnormality.

    A 29-year-old male with sarcoidosis autoimmune haemolytic anaemia and paroxysmal nocturnal haemoglobinuria is described. Throughout his illness the chest films showed fibrosis in the right hilar region and he had had several pneumonias in the right lung. He had had massive splenomegaly and a splenectomy was performed. He was treated with prednisone and cyclophosphamide. Because his blood group was initially confused, several incompatible blood transfusions were given. Two types of antibody were detected: an autoantibody with "s" specificity and an alloantibody with Rh "D" specificity. Other interesting features in this case revealed at autopsy were a viral pneumonia and toxoplasma gondii infection of the brain. As far as we know, this is the first reported patient with this unusual association.
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ranking = 5
keywords = haemoglobinuria
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5/7. Autoimmune haemolytic anaemia with monoclonal IgM (kappa) anti-P cold autohaemolysins.

    A patient is described with immunoblastic non-Hodgkin's lymphoma and autoimmune haemolytic anaemia of the cold autoantibody type. The autoantibodies were monoclonal IgM-kappa cold haemagglutinins/haemolysins with blood-group specificity, anti-P. Red-cell autoantibodies directed against blood-group-P antigen have until now only been detected, as polyclonal IgG antibodies, in paroxysmal nocturnal haemoglobinuria.
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keywords = haemoglobinuria
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6/7. Autoimmune haemolytic anaemia complicating mycoplasma pneumoniae infection.

    Although a positive direct Coombs' test is common in patients with mycoplasma pneumoniae infection, manifest haemolysis is rare. A case is presented in which M. pneumoniae infection was followed by severe autoimmune haemolytic anaemia with intravascular haemolysis and haemoglobinuria. The presence of cold haemagglutinins suggests that these autoantibodies caused the haemolysis. Corticosteroid treatment corrected the haemolysis, which was of brief duration. Published data indicate that the mycoplasma agent is capable of altering the I-receptors of normal erythrocytes by haemadsorption and production of a soluble haemolysin.
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7/7. Paroxysmal cold haemoglobinuria coexisting with cold agglutinins in a patient with syphilis resulting in peripheral gangrene: a case report.

    Cold-reactive antibodies may cause peripheral ischaemia, occasionally proceeding to gangrene on exposure to cold. A 46-year-old male with syphilis, presented with an unusual combination of both paroxysmal cold haemoglobinuria and cold agglutinin disease, resulting in peripheral gangrene and requiring bilateral below knee amputations.
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keywords = haemoglobinuria
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