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1/165. Resolution of immune haemolytic anaemia with allogeneic bone marrow transplantation after an unsuccessful autograft.

    Autologous transplantation of lymphocyte-depleted peripheral blood stem cells (PBSC) has been proposed for treatment of patients with severe autoimmune disease. However, several patients have been reported to achieve only transient remissions. We report on a child with thalassaemia intermedia and immune-mediated haemolytic anaemia, given an autologous lymphocyte-depleted PBSC transplant, who relapsed 7 weeks after transplant. A complete remission, lasting 18 months to date, was obtained with allogeneic bone marrow transplantation (BMT) from an HLA-matched unrelated donor. This experience indicates that, in selected cases, allogeneic BMT may be the treatment of choice for life-threatening autoimmune disease. A graft-versus-autoimmunity effect may favour the eradication of the recipient autoaggressive lymphocytes.
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2/165. Penicillin-induced haemolytic anaemia associated with microangiopathy.

    The development of a penicillin-induced haemolytic anaemia was studied in a patient with microangiopathy following an average daily dose of five million units of penicillin g (total dose 46 million units). It has been proposed that the combination of the benzylpenicilloyl hapten with exposed or altered erythrocyte antigens, induces an autoimmune response. In this particular case, it is suggested that erythrocyte membrane damage has predisposed to immune drug-mediated red cell damage.
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3/165. Serial changes in the galactosylation of autoantibodies and serum IgG in autoimmune haemolytic anaemia.

    A number of systemic autoimmune diseases are associated with increased levels of the agalactosyl (G0) IgG isoforms that lack a terminal galactose from the C(H)2 domain oligosaccharide. The aims were to determine whether there are also persistently high levels of G0 autoantibodies or serum IgG in autoimmune haemolytic anaemia (AIHA), and whether any changes in galactosylation over time are related to the course of disease. autoantibodies eluted from red blood cells, and serum IgG, were obtained from a patient with chronic AIHA over a 21 month period, and the degree of galactosylation measured using a lectin-binding assay. There were wide fluctuations in the galactosylation of autoantibody and serum IgG, but these changes were unrelated to the severity of the anaemia. The galactosylation of autoantibody and serum IgG varied independently, and the autoantibodies were preferentially G0 in comparison with serum IgG in only half of the serial samples. We conclude that AIHA differs from other, systemic autoimmune conditions in that high levels of G0 autoantibodies or serum IgG are not persistent, and that changes in galactosylation do not parallel the course of disease.
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4/165. Excessive apoptosis of bone marrow erythroblasts in a patient with autoimmune haemolytic anaemia with reticulocytopenia.

    We report a patient with autoimmune haemolytic anaemia (AIHA) with reticulocytopenia, who showed excessive apoptosis of erythroblasts. Ultrastructural analysis of bone marrow cells showed that 50% of erythroblasts had characteristic features of apoptosis, which was confirmed by staining with Annexin-V. In addition, in contrast to normal erythroblasts, Ig staining of the entire erythroblast population could be shown. These data show that apoptosis may contribute to the mechanism of reticulocytopenia in AIHA.
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5/165. Retractile mesenteritis presenting as fever of unknown origin and autoimmune haemolytic anaemia.

    Retractile mesenteritis is an extremely rare disease characterised by a non-specific inflammatory and fibrotic process of the mesenteric adipose tissue, which is usually accompanied by pain and a variety of other abdominal symptoms. We describe here the case of a patient with retractile mesenteritis presenting with prolonged high-grade fever and autoimmune haemolytic anaemia without abdominal symptoms. The patient's illness was complicated by chylous ascites. diagnosis was suspected by computed tomography and confirmed histologically following exploratory laparotomy. The patient was treated with prednisone and azathioprine, and he had a rapid improvement in anaemia and fever relief, but no substantial change in the mesenteric lesions. Our case adds autoimmune haemolytic anaemia to the wide spectrum of manifestations of retractile mesenteritis and implies the possible involvement of immune mechanisms in the pathogenesis of the disease.
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keywords = anaemia
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6/165. Severe haemolytic anaemia complicating infectious mononucleosis.

    The case record of a patient suffering from infectious mononucleosis, complicated by severe haemolytic anaemia, with overt intravascular haemolysis, is reported. Investigation of such cases usually shows a high titre of cold agglutinins with anti-i specificity. In the case reported, the results of serological investigations were atypical, and suggested that cold agglutinins with anti-L activity (rather than anti-i) were predominant in the aetiology of the haemolysis.
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keywords = anaemia
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7/165. Chronic lymphatic leukemia presenting as hemolytic anaemia.

    In CLL autoimmune phenomena manifest most commonly as autoimmune hemolytic anemia followed by thrombocytopenic purpura and pure red cell aplasia. Here an elderly lady presenting with AIHA as the presenting manifestation of CLL is described.
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keywords = anaemia
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8/165. pyoderma gangrenosum in a patient with autoimmune haemolytic anaemia and complement deficiency.

    About 50% of cases of pyoderma gangrenosum (PG) are associated with a variety of systemic diseases. We describe a patient with severe PG on both legs who suffered from an autoimmune haemolytic anaemia in association with a congenital deficiency of complement factors C2 and C4. To the best of our knowledge, this constellation has not been previously reported.
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keywords = anaemia
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9/165. Fatal cold anti-i autoimmune haemolytic anaemia complicating hairy cell leukaemia.

    Hairy cell leukaemia (HCL) is a rare lymphoproliferative disorder associated with pancytopenia, splenomegaly and the presence of typical hairy B lymphocytes in the bone marrow and/or peripheral blood. The most significant complication relates to opportunistic infections that arise as a consequence of neutropenia and monocytopenia. HCL is occasionally associated with systemic autoimmune disorders including polyarteritis nodosa and rheumatoid disease. Secondary autoimmune haemolytic anaemia (AIHA) appears to be rare. We report on two cases of HCL complicated by fatal cold anti-i AIHA. Fulminant haemolysis causing death is rare in cold AIHA and only a few individual cases have been reported, none having anti-i specificity.
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keywords = anaemia
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10/165. Efficacy of low dose intravenous immunoglobulins for post-splenectomy treatment of autoimmune haemolytic anaemia in a patient with hereditary spherocytosis.

    Summary Idiopathic autoimmune haemolytic anaemia developed in a patient with hereditary spherocytosis. The behaviour of some osmotic fragility tests throughout the illness and the efficacy of intravenous immunoglobulins in controlling autoimmune haemolysis which recurred post splenectomy are discussed
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