Term
| Describe hematocrit and hemoglobin values immediately after acute blood loss |
|
Definition
| normal. The blood in the patient is the same there is just less of it. |
|
|
Term
| What causes a decrease in hematocrit following acute blood loss? |
|
Definition
| Volume replacement therapy or normal plasma volume expansion |
|
|
Term
| What happens to the wbc count following acute blood loss? |
|
Definition
|
|
Term
| What happens to the platelet count following acute blood loss or hemorrhage? |
|
Definition
|
|
Term
| What two abnormal findings would you notice on the peripheral blood smear of someone who's recently suffered acute blood loss or hemorrhage? |
|
Definition
| nucleated RBC's and immature WBC's |
|
|
Term
| How long does it take after acute blood loss for erythropoietin to cause polychromatophilic macrocytes to appear on the peripheral smear? |
|
Definition
|
|
Term
| What happens to 2,3 DPG levels following acute blood loss, and what shift is this? |
|
Definition
| Increase in intracellular 2,3 DPG; shift to the right |
|
|
Term
| After acute blood loss, when is maximum reticulocytosis reached? |
|
Definition
|
|
Term
| What are the two main classes of aplastic anemia? |
|
Definition
|
|
Term
| Aplastic anemia is mainly a disorder of what cells? |
|
Definition
|
|
Term
| What would you find in the bone marrow of a patient with aplastic anemia? |
|
Definition
| Fatty replacement of bone marrow/ hypocellular. BM is <25% cellular |
|
|
Term
| What kind of anemia is characterized by hypocellular bone marrow, pancytopenia, and a corrected retic count of less than 1%? |
|
Definition
|
|
Term
| What kind of anemia is characterized by a depletion or reduction of hematopoietic precursors in the bone marrow leading to peripheral pancytopenia? |
|
Definition
|
|
Term
| What kind of anemia is caused by damage to CFU-GEMM? |
|
Definition
|
|
Term
| What is the expected WBC count of a patient with aplastic anemia? |
|
Definition
|
|
Term
| What is the expected platelet count of a patient with aplastic anemia? |
|
Definition
|
|
Term
| 50-70% of all aplastic anemias fall into this category |
|
Definition
|
|
Term
| What does it mean if aplastic anemia is described as 'idiopathic'? |
|
Definition
| It can't be linked to any causes. |
|
|
Term
| What is one possible cause of idiopathic aplastic anemia? |
|
Definition
| possible autoimmune process |
|
|
Term
| What is responsible for 1/3 of acquired cases of aplastic anemia? |
|
Definition
| drugs- chloramphenicol, sulfa drugs and tetracyclines- antibiotics |
|
|
Term
| What kinds of chemical agents can cause aplastic anemia? |
|
Definition
| benzene, insecticides, chemotherapeutic drugs, weed killers, arsenic, CCl4 |
|
|
Term
| How do chemical agents cause aplastic anemia? |
|
Definition
| They have a toxic effect on proliferating cells. |
|
|
Term
| After exposure to ionizing radiation, how long does it take for aplastic anemia to develop? |
|
Definition
| It can develop years after exposure |
|
|
Term
| Is aplastic anemia caused by ionizing radiation reversible? |
|
Definition
| It can be, if exposure is minimal. |
|
|
Term
| Three biologic agents that could potentially cause aplastic anemia |
|
Definition
| infectious mono, hepatitis, parvovirus |
|
|
Term
|
Definition
| paroxismal nocturnal hemoglobinuria |
|
|
Term
| Describe aplastic anemia related to pregnancy |
|
Definition
| rare; remits after delivery. Thought to be hormonal |
|
|
Term
| What kind of anemia is paroxismal nocturnal hemoglobinuria associated with? |
|
Definition
|
|
Term
| An autosomal recessive disorder characterized by bone marrow hypoplasia, congenital anomalies, dwarfism and microcephaly |
|
Definition
|
|
Term
| Name two types of congenital aplastic anemia |
|
Definition
| Fanconi's anemia and familial aplastic anemia |
|
|
Term
| What is the difference between Fanconi's anemia and familial aplastic anemia? |
|
Definition
| familial aplastic anemia is similar to Fanconi's but without the congenital abnormalities |
|
|
Term
| What abnormality in the WBC differential is associated with aplastic anemia? |
|
Definition
| absolute granulocytopenia |
|
|
Term
| Describe the onset of aplastic anemia |
|
Definition
|
|
Term
| What percent of aplastic anemia patients die within 5 years of diagnosis? |
|
Definition
|
|
Term
| What are two treatments used for aplastic anemia? |
|
Definition
| bone marrow transplantation and immunosuppressive therapy |
|
|
Term
| Describe the prognosis of aplastic anemia caused by recent exposure to toxins? |
|
Definition
| Better prognosis than other forms of aplastic anemia |
|
|
Term
| Anemia characterized by depletion of erythroid precursors with no decrease in WBC or platelets |
|
Definition
|
|
Term
| Two main classes of pure red cell aplasia |
|
Definition
|
|
Term
| TEC or transient erythroblastemia of childhood is another name for what disorder? |
|
Definition
|
|
Term
| what are the possible causes of acquired pure red cell aplasia? |
|
Definition
| viral or bacterial infections, drug toxicity, thymoma, SLE, RA, hemolytic anemia |
|
|
Term
| What treatment is used for acquired pure red cell aplasia? |
|
Definition
| immunosuppression therapy |
|
|
Term
| Diamond-Blackfan syndrome is another name for what disorder? |
|
Definition
| congenital pure red cell aplasia |
|
|
Term
| What anemia is caused by inheritance of a defective erythroid progenitor cell- BFU-E? |
|
Definition
| Diamond-Blackfan syndrome/ congenital pure red cell aplasia |
|
|
Term
| In anemia of chronic renal failure, hemoglobin begins to decrease when blood urea nitrogen increases to what level? |
|
Definition
|
|
Term
| In anemia of chronic renal failure, what happens to erythropoietin production? |
|
Definition
| erythropoietin production by diseased kidney is decreased |
|
|
Term
| What kind of poikilocytes are seen in anemia of chronic renal failure? |
|
Definition
| burr cells and schistocytes |
|
|
Term
| Decreased erythropoietin production associated with endocrine abnormalities usually involves what glands? |
|
Definition
| pituitary, thyroid, adrenals, gonads |
|
|
Term
| Is aplastic anemia hypoproliferative or hemolytic? |
|
Definition
|
|
Term
| A group of hereditary refractory anemias characterized by ineffective erythropoiesis and secondary siderosis |
|
Definition
| congenital dyserythropoietic anemia |
|
|
Term
| How is congenital dyserythropoietic anemia inherited? |
|
Definition
|
|
Term
| What abnormality is present in the red cell precursors in the bone marrow in congenital dyserythropoietic anemia? |
|
Definition
| red cell precursors in BM exhibit multinuclearity |
|
|
Term
| Which two types of congenital dyserythropoietic anemia show megaloblastic changes in BM? |
|
Definition
|
|
Term
| Which form of congenital dyserythropoietic anemia is known as HEMPAS? |
|
Definition
|
|
Term
| anemia characterized by bone marrow replacement or infiltration by fibrotic, granulomatous or neoplastic cells? |
|
Definition
|
|
Term
| Leukoerythroblastic reaction is characteristic of what disorder? |
|
Definition
|
|
Term
| What happens to the platelet count in myelophthisic anemia? |
|
Definition
| increased, decreased or normal |
|
|
Term
| What poikilocytes are common in myelophthisic anemia? |
|
Definition
| dacryocytes and bizarre platelets |
|
|
Term
| What anemia is seen in metastatic cancer of prostate, breast and stomach? |
|
Definition
|
|
Term
| What anemia is seen in myelofibrosis and lipid storage diseases? |
|
Definition
|
|
Term
| What is the constant feature of hemolytic anemias? |
|
Definition
|
|
Term
| Activation of complement on red cell membrane causes what? |
|
Definition
|
|
Term
| When defective cells are removed from circulation by phagocytes in the reticuloendothelial system, what is this called? |
|
Definition
|
|
Term
| In extravascular hemolysis, is it common to have hemoglobinemia, hemoglobinuria, or hemosiderinuria? |
|
Definition
|
|
Term
| In the erythrocyte survival studies, what radioactive element is tagged onto the patient's red cells? |
|
Definition
|
|
Term
| what is the normal half life of 51Cr? |
|
Definition
|
|
Term
| Which defects are generally hereditary, intrinsic or extrinsic? |
|
Definition
|
|
Term
| Which defects are generally acquired, intrinsic or extrinsic? |
|
Definition
|
|
Term
| Defects in membrane, cell enzyme system, or hemoglobin molecule are classified as what kind of defect? |
|
Definition
|
|
Term
| Are intrinsic defects usually associated with extravascular or intravascular hemolysis? |
|
Definition
|
|
Term
| Which defects are caused by external factors in the red cell environment? |
|
Definition
|
|
Term
| Are extrinsic defects generally associated with intravascular or extravascular hemolysis? |
|
Definition
|
|
Term
| Hereditary spherocytosis is a defect in what part of the red cell? |
|
Definition
|
|
Term
| hereditary elliptocytosis is a defect in what part of the red cell? |
|
Definition
|
|
Term
| hereditary pyropoikilocytosis is a defect in what part of the cell? |
|
Definition
|
|
Term
| hereditary stomatocytosis is a defect in what part of the red cell? |
|
Definition
|
|
Term
| Paroxysmal nocturnal hemoglobinuria is a defect in what part of the red cell? |
|
Definition
|
|
Term
| How is hereditary spherocytosis inherited? |
|
Definition
| autosomal dominant- 75%; autosomal recessive- 25% |
|
|
Term
| Which disorder is caused by spectrin abnormalities which result in membrane instability and progressive membrane loss? |
|
Definition
|
|
Term
| What happens to ATP requirements in spherocytes? |
|
Definition
| increased permeability of membrane to passive influx of sodium ions leads to increased ATP requirements |
|
|
Term
| Spherocytes die after becoming trapped in what organ? |
|
Definition
|
|
Term
| which hemolytic anemia is characterized by mild to moderate anemia, jaundice, splenomegaly, chronic cholecystitis? |
|
Definition
|
|
Term
| What happens to the MCHC in hereditary spherocytosis? |
|
Definition
|
|
Term
| reticulocytes, serum bilirubin, urine and fecal urobilinogen, and osmotic fragility are all increased in what hemolytic anemia? |
|
Definition
|
|
Term
| what are the two types of crisis associated with hereditary spherocytosis? |
|
Definition
|
|
Term
| what is the normal range for osmotic fragility test? |
|
Definition
|
|
Term
| what are the osmotic fragility test results for hereditary spherocytosis? |
|
Definition
|
|
Term
| How is hereditary elliptocytosis inherited? |
|
Definition
|
|
Term
| What is the hereditary elliptocytosis gene linked to in some families? |
|
Definition
|
|
Term
| which hemolytic anemia is linked to Rh blood type in some families? |
|
Definition
| hereditary elliptocytosis |
|
|
Term
| which membrane defect results from a defect in the membrane cytoskeleton? |
|
Definition
| hereditary elliptocytosis |
|
|
Term
| What percent of patients with hereditary elliptocytosis have hemolytic variants? |
|
Definition
|
|
Term
| What percent of cells are elliptocytes in hereditary elliptocytosis? |
|
Definition
|
|
Term
| How is hereditary pyropoikilocytosis inherited? |
|
Definition
|
|
Term
| In hereditary pyropoikilocytosis, what part of the membrane is defective? |
|
Definition
|
|
Term
| What temperature to the membranes fragment in hereditary pyropoikilocytosis? in normal cells? |
|
Definition
| 45-46C in hereditary pyropoikilocytosis; 49-50C in normal cells |
|
|
Term
| How is hereditary stomatocytosis inherited |
|
Definition
|
|
Term
| Which hemolytic anemia is associated with abnormal permeability of the red cell membrane to sodium and potassium ions probably due to several different membrane defects? |
|
Definition
| hereditary stomatocytosis |
|
|
Term
| in hereditary stomatocytosis, what percent of the red cells are stomatocytes? |
|
Definition
|
|
Term
| what three conditions besides hereditary stomatocytosis are associated with stomatocytes? |
|
Definition
| Rh null; acute alcoholism; liver disease |
|
|
Term
| is paroxysmal nocturnal hemoglobinuria congenital or acquired? |
|
Definition
|
|
Term
| In PNH, the cells are unusually sensitive to what? |
|
Definition
|
|
Term
| What disorder is characterized by an abnormal clone of stem cells that gives rise to cells in all three lines which are abnormally sensitive to complement mediated lysis? |
|
Definition
|
|
Term
| A chronic disease primarily of young adults characterized by chronic intravascular hemolysis with constant hemosiderinuria and intermittent hemoglobinuria? |
|
Definition
|
|
Term
| What's the only intrinsic membrane defect disorder that's not hereditary? |
|
Definition
|
|
Term
| Which hemolytic anemia could develop into acute leukemia or aplastic anemia? |
|
Definition
|
|
Term
| What disorder is associated with positive Ham;s test and positive sugar water test? |
|
Definition
|
|
Term
| What is the current test used to check for PNH? |
|
Definition
| immunophenotyping- uses monoclonal Ab's to GPI anchored molecules |
|
|
Term
| What is the most common red cell enzyme disorder? |
|
Definition
|
|
Term
| What is the normally active G6PD found in 99% of caucasians in the US? |
|
Definition
|
|
Term
| What is the form of G6PD found in about 20% of american black males? |
|
Definition
|
|
Term
| What G6PD variant is found in 10% of American blacks? |
|
Definition
|
|
Term
| How much enzyme activity does G6PD A+ have compared to G6PD B? |
|
Definition
|
|
Term
| How much enzyme activity does G6PD A- have compared to G6PD B? |
|
Definition
|
|
Term
| What is the most common abnormal G6PD variant in caucasians? |
|
Definition
|
|
Term
| How much enzyme activity does G6PD-Mediterranean and G6PD-Canton have compared to G6PD-B? |
|
Definition
|
|
Term
| Which abnormal G6PD variant is found frequently in Kurdish Jews? |
|
Definition
|
|
Term
| What is the most common enzyme deficiency in Embden-Myerhoff pathway? |
|
Definition
|
|
Term
| Deficiency of this enzyme results in the inability to generate ATP |
|
Definition
|
|
Term
| nonpolar amino acid substitution for polar one on chain surface results in what effect on the hemoglobin function? |
|
Definition
|
|
Term
| Substitutions in the heme pocket, at subunit interaction sites, at 2,3 DPG binding sites or at the C terminal end of B-chain affect what property of hemoglobin? |
|
Definition
|
|
Term
| amino acid substitutions in internal structure of hemoglobin molecule (polar for nonpolar) cause what change in hemoglobin? |
|
Definition
|
|
Term
| in hemoglibin S, what is the substitution? |
|
Definition
| valine for glutamic acid at 6th position of beta chain |
|
|
Term
| what is the amino acid substitution in hemoglobin c? |
|
Definition
| lysine for glutamic acid at 6th position of beta chain |
|
|
