HEMATOLOGIC DISORDERS

• an inherited autosomal dominant disorder, affects both sexes and is most common among White people?

  Von Willebrand disease
• an inability of the platelets to aggregate and the blood vessels to constrict to aid in coagulation. Bleeding time is prolonged, with most hemorrhages occurring from mucous membrane sites?

  Von Willebrand disease
• there is a decrease in plasma levels or defect in the von willebrand factor, the ability of the blood to clot decreases leading to a heavy and continuous bleeding after an injury?

  Von Willebrand disease
• the treatment of choice for von Willebrand disease patients?

  Desmopressin
• Nursing interventions for Von Willebrand disease may include?

  monitor for bleeding

  Administer desmopressin

  educate patient about bleeding precautions
• a blood test to measure the amount of factor VIII in the blood?

  Factor VIII clotting activity
• a blood test to measure the amount of VWF in the blood?

  Von Willebrand factor antigen
• is caused by factor IX deficiency?

  Christmas disease
• treatment for christmas disease?

  concentrated factor IX
• also called factor IX?

  hemophilia B
• a genetic disorder caused by missing or defective factor IX, a clotting protein?

  hemophilia B
• People with hemophilia have low levels of?

  factor VIII

  factor IX
• treatment for hemophilia B?

  factor IX injections
• Lab test for Hemophilia?

  CBC

  factor VIII assay

  coagulation studies
• a potentially lethal immunologic response of donor T cells to the tissue of the bone marrow recipient?

  Graft versus host disease
• symptoms of this disease range from mild to severe and generally include a rash and general malaise beginning 7 to 14 days after the transplant. Severe symptoms include high fever, diarrhea, and liver and spleen enlargement?

  Graft versus host disease
• tx for chronic GVHD?

  ciclosporin
• interventions for GVHD?

  recording of I and O

  manage side effects of treatments

  monitor electrolyte imbalance
• lab tests for GVHD?

  xray

  ct scan

  ct chest
• can cause loss of function in kidney cells, which causes an accompanying decrease in erythropoietin production, resulting in a normocytic, normochromic anemia?

  anemia of renal disease
• Administration of recombinant human erythropoietin can increase RBC production and correct the anemia, but not the renal disease.
• a common complication of chronic kidney disease?

  anemia
• RF for Anemia of Renal Disease?

  alcohol abuse

  being around with toxic chemicals

  age
• treatment for anemia of renal disease?

  erythropoiesis stimulating agents

  iron supplementation
• interventions for anemia for renal disease?

  manage BP

  control glucose level

  control fluid volume
• Lab tests for Anemia with CKD?

  CBC
• autosomal recessive anemias associated with abnormalities of the beta chain of adult hemoglobin (hemoglobin A)?

  thalassemia
• Because of the imbalance in chain synthesis, an excess of freed α-globin chains accumulates within erythroid cells. Aggregation, denaturation, and degradation of these chains leads to the formation of insoluble precipitates as well as hemichromes, which damage cell membranes?

  thalassemia
• mild form of this anemia, produce a combination of both defective beta heroglobin and normal hemoglobin. The condition represents the heterozygous form of the disorder and can be compared with children having the sickle cell trait. Because there is some normal production, the RBC count is usually normal, but the hemoglobin concentration will be decreased by 2 to 3 g per 100 mL below usual levels. The blood cells are moderately hypochromic and microcytic because of the poor hemoglobin formation?

  thalassemia minor
• thalassemia minor symptoms?

  pallor
• also called Cooley anemia or Mediterranean anemia?

  Thalassemia major
• It is diagnosed at birth by a newborn metabolic blood-spot test. Because this is a beta-chain hemoglobin defect, symptoms do not become apparent until a child's fetal hemoglobin has largely been replaced by adult hemoglobin during the second half of the first year of life. Unable to produce normal beta hemoglobin, the child shows symptoms of anemia, including pallor, irritability, and anorexia?

  Thalassemia major
• are the main way to treat moderate or severe thalassemia?

  blood transfusion
• ultimate cure for thalassemia?

  Stem cell transplantation
• treatment for thalassemia?

  digitalis

  diuretics

  low sodium diet
• the most common anemia of infancy and childhood?

  iron deficiency anemia
• is a hypochromic-microcytic anemia – red blood cells (RBCs) are abnormally small with low levels of hemoglobin (hgb)?

  iron deficiency anemia
• IDA common symptoms?

  Pale conjunctiva
• IDA treatment?

  iron with vitamin C
• lab test for IDA?

  CBC

  transferrin