Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0002874 (aplastic anemia)
 5,905 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Sera from 23 patients with idiopathic thrombocytopenic purpura (ITP), 14 patients with aplastic anemia with severe thrombocytopenia and healthy control subjects were tested for the presence of fibrinogen/fibrin degradation products (FDP), using the tanned red cell hemagglutination inhibition immunoaassay. The concentrations of circulating FDP of ITP patients (mean 12.01 mug/ml) were significantly higher than those of the patients with aplastic anemia (mean 4.01 mug/ml, p less than 0.05) or normal controls (mean 3.10 mug/ml, p less than 0.001). The patients with untreated ITP with very low platelet counts had higher levels of FDP than those of the treated group (p less than 0.01). Serum FDP and a battery of other coagulation-fibrinolysis tests were serially carried out over a period of 10 weeks in two patients with ITP. The initially high FDP promptly decreased as circulating platelets increased in response to steroid in both patients, while plasma fibrinogen, euglobulin lysis time, prothrombin time and partial thromboplastin time remained essentially normal during the course of observation. The exact source of the increased serum FDP in ITP was not established, but a few possible mechanisms responsible for this abnormality were discussed.
 ...
PMID:Fibrinogen/fibrin degradation products in serum of patients with idiopathic thrombocytopenic purpura: elevated levels during severe thrombocytopenic phase of the disease. 98 69

A 28-year-old female, weighing 46 kg, 155 cm in height, with aplastic anemia underwent implantation of iliac bone to the head of the femur. A combined technique of hemodilution and intraoperative blood salvaging was applied to supplement the blood loss during the operation. Intraoperative monitoring included continuous arterial pressure, heart rate, electrocardiograph, SVO2, bleeding time, prothrombin time (PT), activated thromboplastin time (APTT), and thromboelastography. A total of 900 ml of blood was drawn and the same volume of 5% albumin solution was infused over half an hour before the beginning of the surgery. During the operation, 2100 ml of blood was lost, and 1260 ml of autologous blood, 400 ml of homogeneous red blood cells and 5 units of fresh platelet were infused. The values of PT, APTT, bleeding time were within normal ranges after the surgery. Only 3 units of fresh platelet was infused in 2 weeks after the surgery. It was suggested that hemodilution and salvaging autotransfusion is safely performed and beneficial to minimize homogeneous blood transfusion even in a case of aplastic anemia.
 ...
PMID:[Hemodilutional and blood salvaging autotransfusions in a case of aplastic anemia]. 161 64

Various kinds of hematological abnormalities have been known to occur in liver diseases. To understand the hematological changes in acute viral hepatitis, 324 adults with acute viral hepatitis were studied. Of them, 3 were acute hepatitis A, 91 acute hepatitis B, 99 acute non-A, non-B hepatitis (NANB) and 181 acute hepatitis on chronic hepatitis B (AH on CH-B). There were 233 males and 91 females; age ranged from 16 to 74 years (mean age 39 years.) The results showed the incidences of thrombocytopenia (platelet less than 120,000/cmm), anemia (Hb less than 12 g% in male and less than 10% in female patients), leukocytosis (WBC greater than 10,000/cmm) and leukopenia (WBC less than 4,000/cmm) were 19.3%, 12.6%, 10.8% and 7.4%, respectively. Patients with AH on CH-B had significantly higher incidence of anemia and thrombocytopenia than those with acute B hepatitis; other than this, there was no significant difference. Patients with anemia, thrombocytopenia or leukocytosis had significantly higher mean levels of serum bilirubin and higher proportions of prolonged prothrombin time, suggesting that these hematological abnormalities were closely related to the severity of hepatocellular damage. In addition, there were 3 cases (0.9%) complicated with aplastic anemia. Two were NANB hepatitis and the other was AH on CH-B which was seronegative for anti-delta, possibly suggesting NANB virus superinfection. Of these 3 cases, 2 died of complications related to aplastic anemia and 1 survived with normal hematological findings 148 days later.
 ...
PMID:[Hematological abnormalities in acute viral hepatitis and acute hepatitis in HBsAg carrier]. 179 69

We report a patient with severe aplastic anaemia found to have a prolonged prothrombin time due to acquired factor VII deficiency. No evidence for a factor VII inhibitor or inactivator was demonstrable. Laboratory studies identified deficiency both of factor VII activity and factor VII antigen. The factor VII deficiency persisted from clinical presentation until approximately 50 d after allogeneic marrow transplantation when restoration of factor VII activity and antigen was noted. The patient's serum could be depleted of factor VII activity by in vitro incubation with Protein A bound to Sepharose, suggesting the presence of an IgG or IgG containing complex able to bind factor VII, but not neutralize its procoagulant activity. A dual specificity solid phase immunoassay identified a factor VII binding immunoglobulin which was detectable throughout the course of factor VII deficiency. The concordant appearance of this factor VII reactive immunoglobulin and the factor VII deficiency suggested the pathologic role of this immunoglobulin in the aetiology of the factor VII deficiency. This factor VII binding immunoglobulin may have induced rapid plasma clearance of the factor VII molecule or, alternatively, may have modified factor VII synthesis. The immunosuppressive therapy and subsequent lymphohaematopoietic engraftment following allogeneic marrow transplant was accompanied by complete resolution of the factor VII deficiency.
 ...
PMID:Acquired factor VII deficiency associated with aplastic anaemia: correction with bone marrow transplantation. 264 42

After clinical assessment, pertinent history, and family history, the clinician often has a good idea concerning the cause of a patient's bleeding. The most appropriate laboratory tests can then be ordered. Routine screening tests include a complete blood cell count, platelet count, and evaluation of a peripheral blood sample, a prothrombin time, and an activated partial thromboplastin time. Thrombocytopenia may result from idiopathic thrombocytopenic purpura, disseminated intravascular coagulation, or, less commonly, acute leukemia, aplastic anemia, thrombotic thrombocytopenic purpura, or a particular drug that a patient is taking. Again, the patient's history, physical findings, and evaluation of a well-prepared peripheral blood smear will be helpful in determining the cause of the patient's thrombocytopenia. An isolated prolongation of the activated partial thromboplastin time may result from low levels of factors VIII, IX, or XI. A slightly prolonged activated partial thromboplastin time and a moderate decrease in factor VIII may reflect von Willebrand disease or the "carrier" state for hemophilia A. In women a greatly prolonged activated partial thromboplastin time and very low levels of factor VIII (< 3%) most often result from an acquired factor VIII inhibitor (autoantibody against factor VIII) or from severe (type III) von Willebrand disease. If von Willebrand disease is suspected (because of menorrhagia with or without other mucous membrane bleeding, a positive family history, and a prolonged activated partial thromboplastin time), more specific laboratory tests for this disease should be done. These include assays of factor VIII, von Willebrand factor antigen, von Willebrand factor activity (measured by the ristocetin cofactor assay), and template bleeding time. In von Willebrand disease the defect is in von Willebrand factor. The affected individual may have subnormal levels of structurally and functionally normal von Willebrand factor (this is called "classic" or type I von Willebrand disease) or may produce von Willebrand factor that is structurally and functionally abnormal (von Willebrand disease type 2). Individuals who inherit a gene for von Willebrand disease from both parents have severe (type 3) von Willebrand disease and will have extremely low levels (< 3%) of von Willebrand factor and factor VIII and will have a very prolonged bleeding time. In most populations type I disease is the most common form, whereas type 3 is the least commonly encountered form. It should be noted that levels of von Willebrand factor can be influenced by the patient's blood type (persons who have blood type AB have 60% to 70% higher levels than do persons who have blood type O) and can be elevated during pregnancy, stress, and hyperthyroidism. The two major functions of von Willebrand factor are to serve as a "bridge" between platelets and injury sites in blood vessel walls and to protect circulating factor VIII from rapid proteolytic degradation. Thus, if a patient has either too little or functionally abnormal von Willebrand factor, the bleeding time will be prolonged and factor VIII will be decreased (because it is not being protected by von Willebrand factor). It should be determined which type of von Willebrand disease a particular patient has because treatment depends on type. Multimeric analysis of von Willebrand factor can be done with use of sodium dodecyl sulfate gels, radiolabeled antibody to von Willebrand's factor, and autoradiography. This will allow visualization of the multimeric structure of von Willebrand factor. In type I disease all bands are present, whereas in the type 2 variants 2A and 2B no high-molecular-weight multimers are seen. Desmopressin acetate (which is available in parenteral form for intravenous use and in a highly concentrated intranasal spray formulation) is the treatment of choice for classic type I disease. The drug effects a rapid release of von Willebrand factor from endothelial cell stor
 ...
PMID:Screening and diagnosis of coagulation disorders. 882 61

There is some clinical evidence that cyclosporine A (CyA) is associated with thrombotic complications of bone marrow and renal transplantation. We investigated plasma concentrations of lipoprotein(a) [Lp(a)], a potentially atherothrombotic lipoprotein, and hemostatic and vascular status in ten patients with aplastic anemia receiving CyA, eleven patients not taking it, and 38 age-matched healthy controls. Patients receiving CyA had significantly higher concentrations of plasma fibrinogen (P < 0.05), prothrombin fragment 1 + 2 (F1 + 2; P < 0.05), plasminogen activator inhibitor-1 (PAI-1; P < 0.05), and von Willebrand factor antigen (P < 0.05) than did patients not taking CyA. Plasma concentrations of Lp(a) were higher in CyA-treated patients than those not receiving it (P < 0.05) or healthy controls (P < 0.05). The difference in the Lp(a) concentration between controls and patients who did not receive CyA-treatment was not significant. Our results suggest that hypercoagulability is likely to occur during CyA therapy. Further, the presence of high concentrations of Lp(a) may accelerate the process of atherosclerosis and increase thrombotic events in patients receiving long-term CyA.
 ...
PMID:Hypercoagulability and high lipoprotein(a) levels in patients with aplastic anemia receiving cyclosporine. 889 50