Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0002878 (hemolytic anemia)
 7,530 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Sera from 378 patients were assayed for antibodies to extractable nuclear antigens (ENA), ribonucleoprotein (RNP) and nonnucleoprotein (Sm). Anti-ENA antibodies were not found in control subjects, patients with rheumatic diseases and negative fluorescent antinuclear antibodies (FANA), or in patients with rheumatoid arthritis, dermatomyositis, drug-induced lupus, idiopathic thrombocytopenic purpura (ITP), or hemolytic anemia with positive FANA. Anti-Sm antibodies were found in 32 per cent of patients with systemic lupus erythematosus (SLE) and were not found in any other condition. There were no significant clinical or serological differences between patients with and without anti-Sm antibodies. Anti-RNP antibodies occurred in 15 per cent of SLE patients, 9 per cent of scleroderma patients, and in 100 per cent of patients with mixed connective tissue disease. SLE patients with anti-RNP antibodies had a significantly lower anti-DNA antibody titer and a significantly lower incidence of nephritis and impaired renal function. Anti-Sm and anti-RNP titers did not vary with changes in clinical status. Awareness of the presence of anti-Sm and anti-RNP antibodies is diagnostically useful. Anti-RNP antibodies have a prognostic value as well.
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PMID:The incidence and clinical significance of antibodies to extractable nuclear antigens. 30 May 68

A 57-year-old woman with diabetes mellitus, hypothyroidism, idiopathic thrombocytopenic purpura, myasthenia gravis, systemic lupus erythematosus, atopy, and basal cell cancer of the skin developed a severe Coombs'-positive autoimmune hemolytic anemia which was resistant to treatment with large doses of azathioprine, cytoxan, and prednisone. One year after transcervical thymectomy the hemolytic anemia disappeared and the patient has maintained a normal hemoglobin and negative Coombs' test without immunosuppressants even since. We believe this case report to be the first recorded instance of thymectomy-induced remission of autoimmune hemolytic anemia in an adult.
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PMID:Case report: Thymectomy-induced remission of acquired autoimmune hemolytic anemia in an adult with myasthenia gravis. 57 Aug 6

Sera from 530 children suffering from various diseases and from 64 controls were tested for smooth muscle autoantibodies (SMA) by indirect immunofluorescence. A high incidence of SMA (51-86%) was found in patients with viral and bacterial infections (viral hepatitis, infectious mononucleosis, measles, mumps, chickenpox, typhoid fever, and brucellosis), independently of liver invovlvement, and in patients with acute haemolytic anaemia due to G-6-PD deficiency (48%). By contrast, the incidence of SMA from patients with beta-thalassaemia major and idiopathic thrombocytopenic purpura was no higher than in the controls. The discrepancy in incidence in haemolytic anaemias due to different causes may reflect the effect of endogenous and extrinsic agents. In the viral infections, SMA were mainly of the IgM class and gave an 'SMA-V' staining pattern. In bacterial infections (typhoid fever and brucellosis), SMA were either IgG only or IgM and IgG, and the staining pattern was also mainly 'SMA-V'. In infections which affect or may affect the liver (viral hepatitis, infectious mononucleosis, typhoid fever, and brucellosis), SMA was present at high titres (1:80-1:320), whereas in infections not affecting the liver (measles, mumps, and chickenpox) the titres were lower (less than or equal to 1:80). In most patients SMA occurred transiently and without apparent pathogenetic significance. The antigen against which infection-induced SMA is directed is not actin; its nature has yet to be identified.
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PMID:Mechanisms of smooth muscle antibody production: a clinical study in children with infections, haemolytic syndromes, and idiopathic thrombocytopenic purpura. 57 62

Heme oxygenase (HO), the primary enzyme responsible for heme catabolism, was measured in spleens from 10 normal subjects, 14 patients with chronic hemolytic anemia (HA), 12 with idiopathic thrombocytopenic purpura (ITP), and 10 with various lymphoproliferative disorders (LD) to determine the splenic enzymatic capacity for heme catabolism and the response of splenic HO to hemolysis. Splenic NADPH-cytochrome c reductase activity and cytochrome P-450 levels were also measured. Splenic HO specific activity in normal spleens was 0.235 +/- 0.106 (SE) nmoles bilirubin/mg protein/min; in HA, 0.276 +/- 0.050; in ITP, 0.228 +/- 0.036; and in LD, 0.420 +/- 0.105. However, the total HO activity per spleen was significantly greater in HA (742.9 +/- 137.4 (SE) nmoles/min, p less than 0.001) and LD (681.9 +/- 180.3, p less than 0.005) than in normal spleens (137.1 +/- 55.0), but was not significantly increased in ITP (269.5 +/- 121.5). In normal spleens cytochrome P-450 was 0.052 +/- 0.006 (SE) nmoles/mg and NADPH-cytochrome c reductase activity was 8.3 +/- 2.2 (SE) nmoles/mg/min; neither of these specific activities was significantly altered in HA, ITP, or LD. Again, total activity was significantly increased in HA and LD associated with increased splenic size. Although total HO activity is greater in the larger spleens, HO activity does not increase per unit weight of tissue. In normal spleen the calculated capacity for bilirubin production by HO was 115 mg per day. This accounts for only 50% of normal daily production from erythroid sources and suggests that other sites are of major importance for hemoglobin degradation.
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PMID:Human spleen heme oxygenase in normal, hemolytic and other pathological states. 82 32

An investigation was carried out of 821 children under the age of 16 years who were subjected to splenectomy in England and Wales during the 5 years 1960-4. A postal follow-up study provided satisfactory information concerning 96 per cent of these. Excluding early postoperative deaths, practically all the cases were followed up for 2 years or more and 70 per cent for more than 5 years. Forty-nine children had died since operation, 32 from underlying disease and 17 from infection. Fifty children (6 per cent of the survivors) had major infections considered to be due to the primary condition aggravated by splenectomy. Seven died, all from the primary disease. Sixteen children (2 per cent) developed septicaemic illnesses and 10 died. In these the effect of splenectomy was considered to be the primary factor. Fourteen of the 16 had been operated on in the first 4 years of life. Practically all the serious infections occurred within 3 years of operation and pneumococcus was the organism most frequently implicated. Ninety per cent of the splenectomies in childhood were performed for accidental injury, congenital haemolytic anaemia or idiopathic thrombocytopenic purpura. Accidental injury to the spleen rarely occurs in very young children, and in the other two conditions splenectomy can usually be safely delayed untile over the age of 3 years. If this is achieved it is estimated that the unavoidable risk of dangerous infection is less than 1 per cent. One in 10 splenectomies will be carried out for severe and potentially fatal illnesses and in this situation the risk from operation is of secondary importance. Many such conditions carry increased susceptibility to infection per se or because corticosteroids or other immune suppressants are used in their management. It is recommended that splenectomy be avoided if at all possible during the early years of life. It is further recommended that prophylactic penicillin be administered for 3 years following operation whatever its indication and whatever the age of the patient. If the underlying condition itself carries risk of infection more active and more prolonged prophylaxis may be indicated.
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PMID:Splenectomy in childhood: a review in England and Wales, 1960-4. 126 73

In haematological diseases, insufficient data has been accumulated to evaluate the efficacy of immunosuppressive drug treatment in patients with erythroid aplasia or sideroblastic anaemia. Cyclophosphamide may be efficacious in inhibiting circulating anticoagulants in patients who need continued replacement of clotting factors. Azathioprine, 6-mercaptopurine, cyclophosphamide and vincristine have been used successfully in treating patients with idiopathic thrombocytopenic purpura, and some patients with auto-immune haemolytic anaemia may benefit from the addition of purine analogues. However, the use of immunosuppressive therapy seems to accelerate the presence of haematological malignancies in patients with macroglobulinaemia. In gastro-intestinal diseases, uncontrolled studies have shown nitrogen mustard, 6-mercaptopurine and azathioprine to be of modest benefit to patients with ulcerative colitis and Crohn's disease. In a controlled trial azathioprine plus prednisone proved more effective than prednisone alone in sustaining remission in patients with Crohn's disease. In patients with either chronic active hepatitis or primary biliary cirrhosis, however, there seems to be no benefit from immunosuppressive therapy for primary treatment of these diseases. Cyclophosphamide, azathioprine and methotrexate have all been used with some success in treating patient with uveitis, and in a controlled trial cytarabine has been shown to be beneficial to patients with herpes ophthalmicus. However, no benefit has been shown to patients with the eye changes of Graves' disease with either azathioprine or methotrexate. Patients with Paget's disease appear to be helped by mithramycin. Cyclophosphamide, chlorambucil and azathioprine are ineffective in treating patients with multiple sclerosis. 6-Mercaptopurine, azathioprine, methotrexate and cyclophosphamide have all produced some benefit in patients with myasthenia gravis, and some patients with idiopathic pulmonary haemosiderosis have responded to azathioprine, 6-mercaptopurine and cyclophosphamide. Alkylating agents have proved useful in treating some patients with asthma and in treating frequent relapsers among children with the nephrotic syndrome. In adults with membrano-proliferative glomerulonephritis some patients have responded to combination therapy with cyclophosphamide, azathioprine and corticosteroids. Immunosuppressive therapy is also indicated in prolonging graft survivals in patients receiving organ transplants. Drug toxicities of immunosuppressive agents are discussed. Their long-term effects, including mutagenic potential, have as yet not been fully elucidated.
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PMID:Clinical use of immunosuppressive drugs: part II. 127 59

Platelet IgG has been studied primarily to develop an aid for clinical diagnosis--a search that has proven elusive (48). However, studies of the interaction of IgG and other plasma proteins with platelets have provided new insight into the intracellular pathways for the acquisition of proteins into regulated secretory granules. From a clinical perspective, it is important not to make the assumption that all platelet IgG is antiplatelet antibody. Data on platelet IgG must be distinguished between measurements of alpha-granule IgG, which comprises almost all of the total platelet IgG content, and platelet surface IgG, representing less than 1 percent of total platelet IgG. alpha-granule IgG does not appear to be antiplatelet antibody; platelet surface IgG is likely to be at least partly antiplatelet antibody. The total platelet (alpha-granule) content of IgG mirrors the concentration and composition of plasma IgG. It is increased in patients with hypergammaglobulinemia of any cause and absent in agammaglobulinemia. The platelet alpha-granule content of IgG, IgA, and albumin is proportional to their concentrations in plasma, and the platelet content of IgG, IgA and albumin also reflects their plasma concentration over a wide range of plasma abnormalities. These observations suggest that platelets acquire IgG, IgA, and albumin by fluid phase endocytosis. Total platelet IgG is consistently increased in patients with ITP and is also commonly increased in patients with thrombocytopenia due to increased platelet destruction of apparently nonimmune mechanisms. The total platelet IgG content does not appear to be increased in patients who have thrombocytopenia due to marrow failure. Therefore, the measurement of total platelet IgG may be analogous to the reticulocyte count, which is increased in patients who have accelerated erythropoiesis caused by either immune or nonimmune hemolytic anemia. The increased concentration of total platelet IgG in patients with ITP is parallel to the appearance of larger platelets produced during the stress of increased thrombopoietic stimulation. Therefore, determinations of mean platelet volume may yield clinical information equivalent to the measurement of total platelet IgG. The concentration of platelet surface IgG is greatly increased in patients with ITP, and concentrations are highest in patients with the most acute ITP. Platelet surface IgG may represent antiplatelet antibody in these patients. However, for unknown reasons, modest elevations of platelet surface IgG are found in many patients with nonimmune thrombocytopenia.(ABSTRACT TRUNCATED AT 400 WORDS)
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PMID:Platelet IgG: measurement, interpretation, and clinical significance. 200 35

Eight patients (six with idiopathic thrombocytopenic purpura, one with immune pancytopenia, and one with autoimmune haemolytic anaemia), who had previously been splenectomized, were found to have splenunculi using radioactively labelled heat damaged autologous erythrocytes. In all patients the splenunculi were found to have significant 'splenic function'. However, there was a poor correlation between the size and the function of the splenunculi. Splenunculectomies were carried out in two patients. This led to a complete haematological remission in one patient and partial remission in the other. In three patients, where splenunculectomies could not be undertaken, the patients had to remain on significant doses of immunosuppressive therapy. In one patient complete remission was achieved using a course of immunosuppressive therapy alone and in the remaining two there was insufficient information to draw valid conclusions. Blood clearance kinetic studies of heat damaged erythrocytes were found to provide an accurate functional assessment of the splenunculi in all patients. However, the presence of a functional splenunculus was found to be the cause of disease relapse in only some patients. Therefore, the management of similar patients should perhaps be along the same lines as that at their initial presentation, that is, immunosuppressive therapy should be tried if they are not on any at the time of relapse, if they are already on it, then splenunculectomy should be done.
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PMID:Detection and functional assessment of accessory splenic tissue (splenunculi) with radiolabelled heat damaged autologous erythrocytes. 208 79

We report a case of autoimmune hemolytic anemia and idiopathic thrombocytopenic purpura (Evans' syndrome) associated with gastric plasmacytoma. Recognition of this rare entity is important because surgery and chemotherapy together may achieve a cure. Possible mechanisms causing the hemolytic anemia and thrombocytopenia are discussed.
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PMID:Evans' syndrome associated with gastric plasmacytoma: case report and a review of the literature. 208 75

Two hundred patients with various haematological diseases underwent splenectomy between 1974 and 1986. The diagnoses were: Hodgkin's disease (n = 76), hairy cell leukaemia (n = 25), idiopathic thrombocytopenic purpura (n = 20), chronic lymphatic leukaemia (n = 19), haemolytic anaemia (n = 18), non-Hodgkin lymphoma (n = 16), myelofibrosis (n = 10), chronic myeloid leukaemia (n = 6), spherocytosis (n = 4), and miscellaneous (n = 6). Many of the patients were treated with corticosteroids and in poor general condition, partly as a result of chemotherapy. There were 37 postoperative complications in 29 patients (14.5%); two died, both of septicaemia. Pneumonia, bleeding, and wound infection were the most common complications, occurring in 9, 8, and 6 patients, respectively. Twelve patients required reoperation, eight for bleeding, two for intra-abdominal abscesses, and one each for pancreatitis and bowel perforation. There was no association between the diagnosis and the type of postoperative complication, but patients whose spleens weighed more than 2 kg had an increased incidence of postoperative complications (30%). We conclude that elective splenectomy is a safe treatment for haematological diseases, even in high risk patients.
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PMID:Splenectomy for haematological diseases. 232 42


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