Term
|
Definition
|
|
Term
|
Definition
| rbc's with an mcv of less than 80 fL and a diameter of less than 7 u |
|
|
Term
|
Definition
| impaired hemoglobin synthesis from ineffective iron utilization, absorption or release; or decreased or defective globin synthesis. |
|
|
Term
| Microcytes are associated with what diseases? |
|
Definition
| iron deficiency anemia, thalassemia and anemia of chronic disease. |
|
|
Term
|
Definition
| rbc's with an mcv of greater than 100 fL and a diameter greater than 9 u. |
|
|
Term
| What does poikilocytosis mean? |
|
Definition
|
|
Term
| What is another name for the sea urchin type or burr cell? |
|
Definition
|
|
Term
| A rbc with 10 to 30 spicules or projections evenly distributed over the entire surface is called..? |
|
Definition
|
|
Term
| What abnormal cell shape is thought to be the result of an increase in the area of the outer leaflet of the lipid bilayer as compared to the inner layer? |
|
Definition
|
|
Term
| On a stained smear, an echinocyte could be artifactual. What would be the causes of this? |
|
Definition
| the alkaline glass effect, or ATP depletion in stored blood. |
|
|
Term
| Which abnormal red cell shape is associated with plasma chemical abnormalities such as dehydration, uremia, liver disease in newborns. |
|
Definition
|
|
Term
| An abnormal red cell that lacks the area of central pallor and has a decreased surface to volume ratio. |
|
Definition
|
|
Term
| What conditions could cause spherocytes? |
|
Definition
| Hereditary spherocytosis, hemolytic anemias |
|
|
Term
| Hereditary spherocytosis is associated with a membrane protein defect. What protein is usually defective? |
|
Definition
|
|
Term
| What causes non-hereditary spherocytes? |
|
Definition
|
|
Term
| What kind of macrocytes are formed following acute hemorrhage or hemolysis? |
|
Definition
|
|
Term
| What abnormal red cell morphology is associated with recovery from acute blood loss, hemolytic anemias, parenchymal liver disease |
|
Definition
|
|
Term
| What is a more common name for a drepanocyte? |
|
Definition
|
|
Term
| Disordered erythrocyte maturation results in what abnormal cell morphology? |
|
Definition
|
|
Term
| An oval to elongated cell with variable diameter is called... |
|
Definition
|
|
Term
| The drepanocyte or sickle cell is associated with what abnormal hemoglobin? |
|
Definition
|
|
Term
| What are two forms of Drepanocytes? |
|
Definition
| holly leaf form and sickle cell |
|
|
Term
| What shape is a dacryocyte? |
|
Definition
|
|
Term
| What abnormal red cell could result from forceful extrusion or removal of the nucleus in extramedullary hematopoiesis? |
|
Definition
|
|
Term
| Removal of rigid inclusion bodies such as hemosiderin, Howell Jolly bodies or Heinz bodies by reticuloendothelial cells can result in this cell type. |
|
Definition
|
|
Term
| What shape is a codocyte when it's in the body? |
|
Definition
|
|
Term
| What does a codocyte look like on the blood smear? |
|
Definition
|
|
Term
| Increased cholesterol and phospholipid in red cell membrane causes increased surface area resulting in what abnormal cell? |
|
Definition
|
|
Term
| What diseases are associated with codocytes? |
|
Definition
| obstructive liver disease, hemoglobinopathies (S and C), thalassemia, iron deficiency, postsplenectomy. |
|
|
Term
| What is the name for a scell that is shaped like a cup, that looks like a mouth on the blood smear? |
|
Definition
|
|
Term
| Increase in the inner lipid bilayer in relation to the outer lipid bilayer results in what abnormal cell shape? |
|
Definition
|
|
Term
| A cell of normal or slightly reduced size with 3 to 12 blunt spicules of uneven length distributed along the periphery of the cell membrane? |
|
Definition
|
|
Term
| How can you tell between an acanthocyte and echinocyte? |
|
Definition
| Acanthocyte has 3 to 12 spicules; echinocyte has 10 to 30 spicules |
|
|
Term
| This abnormal cell is caused by an increase in cholesterol, increased surface area and decreased lecithin in the cell membrane. |
|
Definition
|
|
Term
| Cells that have an essentially normal cell volume, but demonstrate one or more projections or horns. |
|
Definition
|
|
Term
| What abnormal red cell morphology is associated with mechanical trauma and impact hemolysis, DIC and pulmonary emboli? |
|
Definition
|
|
Term
|
Definition
| cell fragments which are irregular in shape and size. |
|
|
Term
|
Definition
| abnormalities in hemoglobin synthesis |
|
|
Term
| if a red cell has a central pallor of greater than 3 u, what is this called? |
|
Definition
|
|
Term
| What illnesses can cause hypochromia? |
|
Definition
| iron deficiency anemia, thalassemia, sideroblastic anemia and lead poisoning |
|
|
Term
| If you see polychromasia, what are you actually seeing? |
|
Definition
|
|
Term
| What cells look gray-blue on a wright-stained smear and are also usually larger than normal? |
|
Definition
| polychromatophilic rbc's/reticulocytes |
|
|
Term
| If you saw hemoglobin C crystals in the blood smear, what would you do next? |
|
Definition
| hemoglobin electrophoresis |
|
|
Term
| Are Howell Jolly bodies visible on the wright stained smear? |
|
Definition
|
|
Term
| Is it possible for a nucleated RBC to have Howell Jolly bodies? |
|
Definition
|
|
Term
| What are Howell Jolly bodies composed of? |
|
Definition
|
|
Term
| Is it normal to see a couple of Howell Jolly bodies here and there? |
|
Definition
| No. It's always abnormal to see them. |
|
|
Term
| What is basophilic stippling made out of? |
|
Definition
|
|
Term
| What inclusions would you see in lead poisoning? |
|
Definition
|
|
Term
| what are Pappenheimer bodies made out of? |
|
Definition
|
|
Term
|
Definition
| A cell with pappenheimer bodies |
|
|
Term
| Are heinz bodies visible on the wright stained smear? |
|
Definition
|
|
Term
| What stain would you use if you wanted to check for heinz bodies? |
|
Definition
| supra-vital stain like crystal violet or brilliant cresyl blue |
|
|
Term
| what are heinz bodies made out of? |
|
Definition
| denatured or precipitated hemoglobin |
|
|
Term
| what inclusion is thought to be remnants of the mitotic spindle? |
|
Definition
|
|
Term
| The functional inability of the blood to supply the tissue with adequate oxygen for proper metabolic function |
|
Definition
|
|
Term
| What happens to hgb and hct values in anemia and why? |
|
Definition
| They are decreased because red cell mass is decreased. |
|
|
Term
| Diagnosis of anemia is based on what 3 sources of info? |
|
Definition
| patient history, physical exam, and hematologic lab findings. |
|
|
Term
| After diagnosing anemia, what is the next step? |
|
Definition
| Determining the cause or etiology. |
|
|
Term
| Why is rapid blood loss more likely to cause death than slow developing anemia, even if the amount of blood lost is the same? |
|
Definition
| In rapid blood loss, death occurs because of circulatory collapse because there is not enough liquid in the circulatory system. In anemia, the volume of red blood cells lost is replaced by plasma, so the patient still has the same volume of blood. |
|
|
Term
| In anemia, tissue acidosis occurs secondary to anaerobic glycolysis; 2,3 DPG increases within the red cell. What shift is this? |
|
Definition
| Right shift in O2 dissociation curve. |
|
|
Term
| Four factors that influence the patient's ability to adapt to anemia. |
|
Definition
| Severity of anemia, competency of cardiovascular and respiratory systems, oxygen requirement of individual, and duration of anemia. |
|
|
Term
| In order to be considered severe anemia, what should the hemoglobin value be? |
|
Definition
|
|
Term
| Would a person with a hemoglobin of 11 g/dL be considered moderately anemic? |
|
Definition
| No. Moderate anemia is hemoglobin of 7 to 10 g/dL. |
|
|
Term
| Name three morphologic categories of anemia. |
|
Definition
Microcytic, hypochromic;
macrocytic;
normocytic |
|
|
Term
| Name two physiologic categories of anemias? |
|
Definition
| Hypoproliferative and increased destruction or loss. |
|
|
Term
| What is the normal blood volume in a term infant? |
|
Definition
|
|
Term
| Why do hemoglobin and hematocrit values usually increase in the first several hours after birth? |
|
Definition
| Because of movement of plasma from intravascular to extravascular space |
|
|
Term
| In the infant, what is the ratio of capillary value to venous value? |
|
Definition
|
|
Term
| What is a normal venous hemoglobin for an infant? |
|
Definition
|
|
Term
| In terms of hgb, retic count, and nRBC count, what is the difference between premature infants and term infants? |
|
Definition
| In premature infants, hgb is lower, retic count and nRBC counts are higher than term infant |
|
|
Term
| At what age does hgb concentration fall to about 11 g/dL and erythropoiesis begins to increase? |
|
Definition
|
|
Term
| What is the normal lifespan of a red blood cell in an infant? |
|
Definition
|
|
Term
| Is it normal to have megaloblastic erythropoiesis in the newborn? |
|
Definition
|
|
Term
| At what age should there no longer be nRBC's present? |
|
Definition
|
|
Term
| Is it normal to see immature white cells such as promyelocytes and blast cells in the newborn's blood smear? |
|
Definition
|
|
Term
| How does the infant's platelet count compare to adult platelet count? |
|
Definition
| Pretty much the same as adult values |
|
|
Term
| Which has a higher O2 affinity, hgb F or hgb A? |
|
Definition
|
|
Term
| At what age is the O2 dissociation curve about the same as an adult? |
|
Definition
|
|
Term
| At birth, which is more abundant, neutrophils or lymphocytes? |
|
Definition
|
|
Term
| at 7 days old, which is more abundant, neutrophils or lymphocytes? |
|
Definition
|
|
Term
| at 2 weeks old, which is more abundant, neutrophils or lymphocytes? |
|
Definition
|
|
Term
| between birth and 2 weeks old, does the wbc count increase or decrease? |
|
Definition
|
|
Term
| Are nucleoli usually seen in the mature megakaryocyte nucleus? |
|
Definition
|
|
Term
| What multipotential stem cell does the megakaryocyte originate from? |
|
Definition
|
|
Term
| What hormone stimulates the production of CFU-Meg? |
|
Definition
|
|
Term
| What is the difference between stress platelets and regular platelets? |
|
Definition
| Stress platelets are larger in size because of shorter maturation and release time; produced in response to thrombocytopenia. |
|
|
Term
|
Definition
| DNA replication without cytoplasmic divisions. Results in one multilobed nucleus. |
|
|
Term
| A giant polyploid cell is produced by what process? |
|
Definition
|
|
Term
| The ploidy of the megakaryocyte correlates with what 2 things? |
|
Definition
| Size of megakaryocyte and number of platelets produced. |
|
|
Term
| At what ploidy does a megakaryocyte become morphologically recognizable as a megakaryocyte? |
|
Definition
|
|
Term
| Stages of megakaryocyte maturation- |
|
Definition
| Megakaryoblast, Promegakaryocyte, Granular megakaryocyte, Mature megakaryocyte. |
|
|
Term
| What is the N:C ratio of the megakaryoblast? |
|
Definition
|
|
Term
| How many nucleoli are present in the nucleus of the megakaryoblast? |
|
Definition
|
|
Term
| What shape is the megakaryoblast nucleus? |
|
Definition
|
|
Term
| What megakaryocyte precursor has blunt cytoplasmic projections or blebs at periphery? |
|
Definition
|
|
Term
| What stage is the earliest morphologically recognizable megakaryocyte? |
|
Definition
|
|
Term
| What is the shape of the promegakaryocyte nucleus? |
|
Definition
|
|
Term
| How many nucleoli are usually present in the promegakaryocyte? |
|
Definition
|
|
Term
| What is the N:C ratio of the promegakaryocyte? |
|
Definition
|
|
Term
| What is the ploidy of the promegakaryocyte? |
|
Definition
|
|
Term
| What is the nucleus like in the granular megakaryocyte? |
|
Definition
| condensed multilobulated nucleus- 8N- 32N |
|
|
Term
| Can endomitosis occur in the granular megakaryocyte? |
|
Definition
|
|
Term
| What is the N:C ratio in the mature megakaryocyte? |
|
Definition
|
|
Term
| What is the ploidy of the majority of the megakaryocytes in the bone marrow? |
|
Definition
|
|
Term
| Is it possible to determine ploidy of a megakaryocyte by looking at it? |
|
Definition
|
|
Term
| The average megakaryocyte releases how many platelets? |
|
Definition
|
|
Term
| After being released from the bone marrow, where do the baby platelets go? |
|
Definition
|
|
Term
| How long do platelets stay in the splenic platelet pool before being distributed throughout the body? |
|
Definition
|
|
Term
| How long do platelets survive? |
|
Definition
|
|
Term
| What portion of the platelets is in the spleen at any given time? |
|
Definition
|
|
Term
| What 3 kinds of granules are in the platelet? |
|
Definition
| alpha, lysosomes, dense bodies. |
|
|
Term
| What are the most numerous granules in the platelet? |
|
Definition
|
|
Term
| Contents of dense bodies? |
|
Definition
| ADP, ATP, Ca2+, serotonin |
|
|
Term
| What is the most common nutritional deficiency in the world? |
|
Definition
|
|
Term
| unique manifestation of iron deficiency characterized by cravings for ice, clay, dirt or crisp foods |
|
Definition
|
|
Term
| What kind of place is iron deficiency most likely to occur? |
|
Definition
| in underdeveloped countries where grain is mainstay of diet and hookworm infections are common |
|
|
Term
| Pica syndrome is associated with what kind of anemia? |
|
Definition
|
|
Term
| About how much iron is lost per day? |
|
Definition
|
|
Term
| Three causes of iron deficiency? |
|
Definition
| chronic blood loss, increased demand, inadequate intake |
|
|
Term
| menstrual bleeding, gastrointestinal bleeding, hookworm infestation, regular blood donation and chronic hemolysis can all cause what? |
|
Definition
|
|
Term
| What is the earliest indicator of developing iron deficiency? |
|
Definition
| decrease in serum ferritin |
|
|
Term
| How do you calculate % transferrin saturation? |
|
Definition
|
|
Term
| Why would a person with iron deficiency be prone to toxic levels of other metals? |
|
Definition
| The absence of iron in the gut allows other metals to be absorbed if they are present. These metals include lead, cadmium and plutonium. |
|
|
Term
| Flattening or concavity of the nails is a sign of what? |
|
Definition
|
|
Term
| What is the normal value for % Fe saturation? |
|
Definition
|
|
Term
| In iron deficiency, what happens to serum iron? |
|
Definition
| Decreased, usually less than 30 ug/dL |
|
|
Term
| In iron deficiency, what happens to TIBC? |
|
Definition
|
|
Term
| In iron deficiency, what happens to % saturation? |
|
Definition
| decreased, usually less than 15% |
|
|
Term
| How is iron deficiency treated? |
|
Definition
| by giving the patient iron. |
|
|
Term
| How long of iron therapy does it take to replenish the body iron stores? |
|
Definition
|
|
Term
| Dyshemopoietic anemia in which defective hemoglobin formation is associated with excessive accumulation of iron in developing immature cells. |
|
Definition
|
|
Term
| In sideroblastic anemia, what happens to total body iron? |
|
Definition
|
|
Term
| Iron accumulates in the mitochondria in what kind of anemia? |
|
Definition
|
|
Term
| Is sideroblastic anemia hereditary or acquired? |
|
Definition
|
|
Term
| what is RARS and is it acquired or hereditary? |
|
Definition
| RARS- Refractory anemia with ringed sideroblasts. Acquired. |
|
|
Term
| Sideroblastic anemia can occur secondary to what..? (4 things) |
|
Definition
| drugs, lead, alcohol or malignancy. |
|
|
Term
| Do patients with sideroblastic anemia have too much iron or not enough? |
|
Definition
|
|
Term
| Are pappenheimer bodies present in sideroblastic anemia? |
|
Definition
|
|
Term
| what kinds of inclusions can be seen in sideroblastic anemia? |
|
Definition
| basophilic stippling and pappenheimer bodies. |
|
|
Term
| in sideroblastic anemia, what happens to serum iron and serum ferritin? |
|
Definition
|
|
Term
| in sideroblastic anemia, what happens to % saturation? |
|
Definition
|
|
Term
|
Definition
| accumulation of iron greatly surpassing normal levels |
|
|
Term
| In iron overload in sideroblastic anemia, gross hemosiderin is deposited in what? |
|
Definition
| macrophages of the spleen, liver, bone marrow and other tissues. |
|
|
Term
| a severe form of iron accumulation that has progressed over a long period of time to involve parenchymal iron deposition and organ injury. |
|
Definition
|
|
Term
| which is more severe, hemochromatosis or hemosiderosis? |
|
Definition
|
|
Term
| what is the most common genetic disorder in the US? |
|
Definition
| hereditary hemochromatosis |
|
|
Term
| what is the treatment for hereditary hemochromatosis? |
|
Definition
|
|
Term
| what is the most common anemia among hospitalized patients? |
|
Definition
| anemia of chronic disease |
|
|
Term
| which anemia is characterized by iron trapped in macrophages? |
|
Definition
| anemia of chronic disease |
|
|
Term
| is anemia of chronic disease hypoproliferative or related to increased destruction? |
|
Definition
|
|
Term
| is anemia of chronic disease usually severe? |
|
Definition
|
|
Term
| in anemia of chronic disease, how are the white count and platelet count usually? |
|
Definition
|
|
Term
| in anemia of chronic disease, what happens to serum iron? |
|
Definition
|
|
Term
| in anemia of chronic disease, what happens to TIBC? |
|
Definition
|
|
Term
| in anemia of chronic disease, what happens to serum ferritin? |
|
Definition
|
|
Term
| in anemia of chronic disease, what happens to % saturation? |
|
Definition
|
|
Term
| Name the two classes of macrocytic anemias |
|
Definition
| Macrocytic anemia with normoblastic maturation; macrocytic anemia with megaloblastic maturation. |
|
|
Term
| Which class of macrocytic anemia has nuclear/cytoplasmic asynchrony? |
|
Definition
| Macrocytic anemia with megaloblastic maturation |
|
|
Term
| Alcoholism, liver disease, myeloproliferative disorders, myelodisplastic syndromes, hemolysis, and acute blood loss can all cause what kind of anemia? |
|
Definition
| macrocytic anemia with normoblastic maturation. |
|
|
Term
| In liver disease or chronic hepatitis, what changes occur in the red cell membrane? |
|
Definition
| The rbc's have increased membrane surface with increased cholesterol and lecithin content. |
|
|
Term
| What is a macroovalocyte? |
|
Definition
| Large, oval red blood cell in peripheral blood; formed from megaloblastic maturation in bone marrow. |
|
|
Term
| In megaloblastic anemia, which grows faster, the cytoplasm or the nucleus? |
|
Definition
|
|
Term
| What kind of anemia is associated with impaired DNA synthesis? |
|
Definition
| Macrocytic anemia with megaloblastic maturation. |
|
|
Term
| What are the two main causes of macrocytic anemia with megaloblastic maturation? |
|
Definition
| Vitamin B12 or folate deficiency. |
|
|
Term
| How does B12 or folate deficiency cause anemia? |
|
Definition
| B12 and folate are necessary to make DNA. |
|
|
Term
| A patient with anemia who also has a sore tongue probably has what kind of anemia? |
|
Definition
| Macrocytic anemia with megaloblastic maturation, caused by B12 or folate deficiency |
|
|
Term
| What clinical symptoms differentiate B12 deficiency from folate deficiency? |
|
Definition
| Vitamin B12 deficiency causes neurological abnormalities not present with folate deficiency. |
|
|
Term
| What does pancytopenia mean? |
|
Definition
| decrease in all cellular elements- rbc's, wbc's, platelets |
|
|
Term
| In a patient with macrocytic anemia with megaloblastic maturation, how would the rbc count, wbc count, and platelet count differ from normal? |
|
Definition
| They are all decreased- pancytopenia. |
|
|
Term
| What inclusions can be seen in a patient with macrocytic anemia with megaloblastic maturation? |
|
Definition
| Howell Jolly bodies, basophilic stippling, Cabot rings. nRBC's also may be present. |
|
|
Term
| Would you expect to see pappenheimer bodies in the blood smear of a patient with macrocytic anemia? |
|
Definition
|
|
Term
| What feature on the peripheral blood smear could be the first sign of megaloblastic dyspoiesis? |
|
Definition
| hypersegmented neutrophils. |
|
|
Term
| Describe the bone marrow of a patient with macrocytic anemia with megaloblastic maturation? (cellularity and M:E ratio) |
|
Definition
| often hypercellular; M:E ratio decreased to 1:1 or lower. |
|
|
Term
| What foods is vitamin B12 found in? |
|
Definition
| meat, liver, seafood, eggs and milk. |
|
|
Term
| Where does the body store vitamin B12, and how long do the stores last? |
|
Definition
| 3-6 year store primarily in liver. |
|
|
Term
| Which is a more common cause of B12 deficiency- inadequate intake, or impaired absorption? |
|
Definition
| impaired absorption is almost always the cause of B12 deficiency. |
|
|
Term
| What problem could be caused by gastrectomy, chronic gastritis, sprue, inflammatory bowel disease or ileal resection? |
|
Definition
| Impaired absorption of vitamin B12 |
|
|
Term
| Two ways that competing intestinal flora and fauna could cause impaired absorption of vitamin B12? |
|
Definition
Blind loop syndrome- bacteria
Diphyllobothrium latum- fish tapeworm. |
|
|
Term
| Cobalamin is also known as what? |
|
Definition
|
|
Term
| what is the vitamin B12 transport protein? |
|
Definition
|
|
Term
| What autoimmune disease could cause impaired absorption of vitamin B12? |
|
Definition
|
|
Term
| What is the disease called in which intrinsic factor is not secreted by the gastric mucosa? |
|
Definition
|
|
Term
| Name 4 things that the antibodies in pernicious anemia could be directed against? |
|
Definition
| parietal cells, intrinsic factor, IF/B12 complex, complex binding sites. |
|
|
Term
| What causes megaloblastic madness? |
|
Definition
|
|
Term
| Is it possible for vitamin B12 to cause neurological abnormalities without causing anemia? |
|
Definition
|
|
Term
| Name two diseases that megaloblastic madness can resemble? |
|
Definition
| alzheimer's and schizophrenia. |
|
|
Term
| What is pteroylglutamic acid? |
|
Definition
|
|
Term
| What foods is folate found in? |
|
Definition
| leafy green vegetables, liver, meats and certain fruits. |
|
|
Term
| How is folate stored in the body, and how long does it last? |
|
Definition
| Folate is stored in the liver; usually enough supply for a few months. |
|
|
Term
| Where in the body is folate absorbed? |
|
Definition
| the duodenum and jejunum. |
|
|
Term
| What is the leading cause of folic acid deficiency? |
|
Definition
|
|
Term
| Poor diet, overcooked vegetables, poverty, alcoholism and old age can all contribute to what? |
|
Definition
|
|
Term
| Alcohol has a direct antagonistic effect on the metabolism of what nutrient? |
|
Definition
|
|
Term
| What factors could contribute to increased utilization of folate? |
|
Definition
| Chronic proliferation of cells as in hemolytic anemia, leukemia, metastatic cancer, pregnancy, sickle cell disease, infancy and young children. |
|
|
Term
| A folate antagonist used in cancer therapy. |
|
Definition
|
|
Term
| Two laboratory methods used to differentiate macrocytic anemias. |
|
Definition
| Microbiological assays, radioisotope methods. |
|
|
Term
| When would a microbiological B12 or folate assay be considered invalid? |
|
Definition
| if the patient is on antibiotics. |
|
|
Term
| To differentiate between folate vs. B12 deficiency, which is quicker, microbiological assay or radioisotope methods? |
|
Definition
|
|
Term
| Differentiation of macrocytic anemias: The serum B12 is decreased, the serum folate is increased, and the red cell folate is decreased. B12 or folate deficiency? |
|
Definition
|
|
Term
| Differentiation of macrocytic anemias: The serum B12 is normal, the serum folate is decreased, and the red cell folate is decreased. B12 or folate deficiency? |
|
Definition
|
|
Term
| Differentiation of macrocytic anemias: The serum B12 is decreased, the serum folate is decreased, and the red cell folate is decreased. B12 or folate deficiency? |
|
Definition
| Combined B12 and folate deficiency. |
|
|
Term
| What is the Schilling test used to evaluate? |
|
Definition
| The ability of the patient's intestinal tract to absorb B12. |
|
|
Term
| When would you perform a Schilling test? |
|
Definition
| After diagnosing a patient with B12 deficiency. |
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Term
| A patient has B12 deficiency. In the first stage of the Schilling test, 15% of the labeled dose is excreted in the patient's urine. What now? Do you know what's causing the deficiency? |
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Definition
| The patient probably has a dietary deficiency. |
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Term
| A patient has B12 deficiency. In the first stage of the Schilling test, 4% of the labeled dose is excreted in the patient's urine. What now? Do you know what's causing the deficiency? |
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Definition
| Now the second stage of the Schilling test has to be done to differentiate between pernicious anemia and malabsorption problem. |
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Term
| A patient has B12 deficiency. In the first stage of the Schilling test, 5% of the labeled dose is excreted in the patient's urine. In the second stage, 5% is excreted. What is indicated by this? |
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Definition
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Term
| A patient has B12 deficiency. In the first stage of the Schilling test, 1% of the labeled dose is excreted in the patient's urine. In the second stage, 50% is excreted. What does this indicate? |
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Definition
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Term
| What is given in the second stage of the Schilling test that is not given in the first? |
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Definition
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Term
| What two factors might invalidate the Schilling test? |
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Definition
| Incomplete urine collection; impaired renal function. |
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Term
| What is achlorohydria and what does it indicate? |
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Definition
| Achlorohydria is the absence of free HCL in the gastric juices after histamine stimulation. Achlorohydria is indirect evidence of pernicious anemia. |
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Term
| Name two substances secreted by the parietal cells? |
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Definition
| intrinsic factor (IF) and hydrochloric acid (HCL) |
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Term
| Increased excretion of MMA in urine is indirect evidence for what? |
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Definition
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Term
| What does MMA stand for, and what is the significance of it? |
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Definition
| Urine methylmalonic acid; increased urine MMA levels could indicate B12 deficiency. |
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Term
| What does homocysteine indicate, and what body fluid is it found in? |
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Definition
| It's measured in plasma and it's increased in patients with B12 or folate deficiency. |
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Term
| What substance in serum has a predictive value for increased risk of MI and venous thrombosis, and also is an early detector of B12 deficiency? |
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Definition
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Term
| A patient has increased MCV, decreased Hgb and Hct, and increased retic count. What is the likely diagnosis? |
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Definition
| Anemia secondary to bleeding, hemolysis or response to therapy. |
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Term
| A patient has increased MCV, decreased Hgb and Hct, and round macrocytes present in the smear. No other morphological oddities are noted. What is the probable diagnosis? |
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Definition
| Normoblastic macrocytic anemia secondary to liver disease, alcoholism, MDS, MD. |
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Term
| A patient has increased MCV, and decreased Hgb and Hct. The peripheral smear shows macroovalocytes, dacryocytes, Howell Jolly Bodies and the occasional hypersegmented neutrophil. What is the probable diagnosis? |
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Definition
| Macrocytic anemia with megaloblastic maturation secondary to B12 or folate deficiency. |
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Term
| A group of hereditary disorders in which there is a defect in the rate of synthesis of one or more of the globin chains of hemoglobin |
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Definition
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Term
| What are the two broad classifications of hemoglobinopathies? |
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Definition
| Those which result from a structural alteration in the globin chain, like sickle cell disease; and those which are the result of defects in the rate of synthesis of the globin chain- the thalassemias. |
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Term
| Who discovered thalassemias, and when? |
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Definition
| Dr. Thomas Cooley, in 1925 |
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Term
| Where did the name thalassemia come from? |
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Definition
| the greek word for "the sea", because the first cases found were in people of Mediterranean background. |
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Term
| Is thalassemia rare, or common? |
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Definition
| It is one of the world's most common genetic disorders. |
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Term
| Thalassemia produces hematologic abnormalities similar to those seen in what other type of anemia? |
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Definition
| Severe iron deficiency anemia. |
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Term
| The globin genes are located on what two chromosomes? |
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Definition
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Term
| What is the globin chain structure of Hgb F? |
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Definition
|
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Term
| What is the globin chain structure of Hgb A? |
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Definition
|
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Term
| What is the globin chain structure of Hgb A2? |
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Definition
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Term
| What two hemoglobins are normally found in the newborn, and what percent? |
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Definition
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Term
| What three Hgb's are normally found in the adult, and what percents? |
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Definition
Hgb A- 92%
Hgb A2- 2%
Hgb F- 1% |
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Term
| When the amount of normal hemoglobin produced is decreased, what is the effect on the red cells? |
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Definition
| microcytic, hypochromic anemia. |
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Term
| What abnormal hemoglobins are formed in alpha thalassemia? |
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Definition
| Hgb Barts- G4, and Hgb H- B4 |
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Term
| What abnormal hemoglobins are formed in beta thalassemia? |
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Definition
| A2 precipitates formed from excess alpha chains- these aren't actually hemoglobins they are just abnormal precipitated chains. |
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Term
| In beta thalassemia, what is the cause of the chronic hemolytic process? |
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Definition
| A2 precipitates cause membrane damage; damaged cells are destroyed by macrophages and/or splenic pitting. |
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Term
| What are the two main variants of thalassemias? |
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Definition
| Alpha thalassemia and Beta thalassemia |
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Term
| Constant spring, hemoglobin lepore, and hereditary persistance of fetal hemoglobin are all what? |
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Definition
| Minor variants of thalassemia |
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Term
| In beta thalassemia, synthesis of which globin chain is absent or diminished? |
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Definition
|
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Term
| Does beta thalassemia affect newborns? |
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Definition
| No because newborns aren't making beta chains yet. |
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Term
| What thalassemia is characterized by elevated levels of Hgb A2 and F? |
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Definition
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Term
| How many genotypic variants are there of Beta thalassemia? |
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Definition
|
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Term
| How many different mutations are included in the B+ variant? |
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Definition
| Over 120 different mutations; extreme variability in amount of beta chain produced. |
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Term
| What are the four classifications of Beta thalassemia according to phenotype? |
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Definition
| Thalassemia major, Thalassemia intermedia, Thalassemia minor and Silent carrier. |
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Term
| Cooley's anemia is another name for what? |
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Definition
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Term
| Thalassemia major can be caused by what three genotypes? |
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Definition
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Term
| What disorder is characterized by bone changes secondary to extremely hyperplastic bone marrow? |
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Definition
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Term
| What microcytic anemia is usually diagnosed in the first year of life, with symptoms like failure to thrive, diarrhea, fever, hepatosplenomegaly and skeletal deformities? |
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Definition
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Term
| Why do Thalassemia major patients develop hemochromatosis? |
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Definition
| Hemochromatosis is iron overload, they develop it because of repeated blood transfusions. |
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Term
| Hemoglobin electrophoresis shows that the majority of hemoglobin is F, A2 is increased, and A is decreased or absent. What thalassemia is this? |
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Definition
| Beta thalassemia- Thalassemia major |
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Term
| What is the prognosis for Thalassemia major patients? |
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Definition
| Death in second or third decade from cardiac siderosis- iron overload |
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Term
| In thalassemia major, the spleen is sometimes removed- why? |
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Definition
| To decrease hemolysis and increase red cell survival. |
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Term
| What inclusions are found in thalassemia major? |
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Definition
| basophilic stippling, heinz bodies. |
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Term
| Are Howell Jolly bodies associated with Thalassemia? |
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Definition
|
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Term
| What genotypes could cause Thalassemia intermedia? |
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Definition
|
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Term
| What is the Hgb concentration in thalassemia intermedia? |
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Definition
| Hgb is 7 g/dL or greater. |
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Term
| What is the percentage of hemoglobin F in thalassemia intermedia? |
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Definition
|
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Term
| What poikilocytes would you see in thalassemia intermedia? |
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Definition
| target cells, basophilic stippling, microcytic hypochromic RBC's |
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Term
| What is the level of hgb A2 in thalassemia intermedia? |
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Definition
|
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Term
| What genotypes could cause thalassemia minor? |
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Definition
|
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Term
| How many abnormal beta genes are present in thalassemia intermedia? |
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Definition
|
|
Term
| How many abnormal beta genes are present in thalassemia major? |
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Definition
|
|
Term
| How many abnormal beta genes are present in thalassemia minor? |
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Definition
|
|
Term
| How many abnormal beta genes are present in silent carrier of beta thalassemia? |
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Definition
|
|
Term
| What is the percent of hgb A2 in thalassemia minor? |
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Definition
|
|
Term
| What is the percent of hemoglobin F in thalassemia minor? |
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Definition
|
|
Term
| What hematologic value differentiates thalassemia minor from Fe deficiency? |
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Definition
| Red cell count is normal to slightly increased in thal minor; in Fe deficiency it's decreased. |
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Term
| How is the Mentzer index calculated? |
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Definition
|
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Term
| What is the Mentzer index used for? |
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Definition
| To differentiate between Thal minor and Fe deficiency. |
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Term
| What is indicated by a Mentzer index of less than 13? |
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Definition
|
|
Term
| What is indicated by a Mentzer index of greater than 13? |
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Definition
|
|
Term
| What is the genotype of a beta thalassemia silent carrier? |
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Definition
|
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Term
| What happens in delta-beta thalassemia? |
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Definition
| Lack of db genes; g genes left intact and overcompensate. |
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Term
| Homozygous delta-beta thalassemia is clinically similar to what other thalassemia? |
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Definition
|
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Term
| What hemoglobins are present in homozygous delta-beta thalassemia? |
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Definition
|
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Term
| If a patient can only make hgb F, what is the diagnosis? |
|
Definition
homozygous delta-beta thalassemia
or
hereditary persistance of fetal hemoglobin |
|
|
Term
| heterozygous delta-beta thalassemia phenotypically presents as what? |
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Definition
|
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Term
| What thalassemia has 5-20% hgb F, 90% hgb A, and normal A2? |
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Definition
| Heterozygous delta-beta thalassemia |
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Term
| Which hemoglobinopathy is thought to be a product of delta-beta fusion genes which arise by unequal crossing over between delta and beta globin loci during meiosis? |
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Definition
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Term
| Hgb Lepore migrates to the same position as what other hemoglobin at pH 8.4? |
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Definition
|
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Term
| What hemoglobins are present in homozygous hemoglobin lepore, and what percentages? |
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Definition
| 20% Hgb Lepore, 80% Hgb F |
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Term
| In heterozygous hemoglobin lepore, what percent of the total hemoglobin is hemoglobin lepore? |
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Definition
|
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Term
| What is the name of this form of delta-beta thalassemia in which gamma genes are not switched off and are able to fully compensate for lack of delta and beta chain production? |
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Definition
| HPFH- hereditary persistance of fetal hemoglobin |
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Term
| In HPFH, do excess alpha chains accumulate and precipitate? |
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Definition
| No, because gamma chain production is increased. |
|
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Term
| In homozygous HPFH, what hemoglobins are made? |
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Definition
|
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Term
| Do patients with homozygous HPFH have severe anemia? |
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Definition
|
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Term
| When does alpha thalassemia manifest itself? |
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Definition
| At birth. Hgb F is not made. |
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Term
| Is alpha thalassemia usually caused by deletions or by point mutations? |
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Definition
|
|
Term
| What thalassemia is characterized by globin tetramers? |
|
Definition
|
|
Term
| How many alpha genes are located on each chromosome 16? |
|
Definition
|
|
Term
| How many alpha genes does a normal person have? |
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Definition
|
|
Term
| Thalassemia with 100% Hgb F; mild to moderate anemia. |
|
Definition
| Homozygous delta-beta thallasemia. |
|
|
Term
| Thalassemia with 100% Hgb F; little or no anemia. |
|
Definition
| Hereditary persistance of fetal hemoglobin- HPFH |
|
|
Term
| What two thalassemias have 100% Hgb F? |
|
Definition
| HPFH and Homozygous delta-beta thalassemia |
|
|
Term
| What is the genotype of an alpha thalassemia silent carrier? |
|
Definition
|
|
Term
| Does the alpha thalassemia silent carrier produce Hgb Barts? How much and when? |
|
Definition
|
|
Term
| Hydrops Fetalis is a fatal type of what thalassemia? |
|
Definition
|
|
Term
| The genotype --/-- is associated with what? |
|
Definition
| Hydrops Fetalis/ fatal form of alpha thalassemia. |
|
|
Term
| What causes Hgb H disease? |
|
Definition
| Hgb H disease is a form of alpha thalassemia with 3 gene deletions or 3 affected genes. |
|
|
Term
| How many gene deletions does a-thal minor have? |
|
Definition
|
|
Term
| What inclusions are present in a-thal minor? |
|
Definition
|
|
Term
| What is the most difficult type of thalassemia to identify clinically and genetically? |
|
Definition
|
|
Term
| Why is a-thal minor usually diagnosed at birth? |
|
Definition
| it is one of very few causes of microcytosis at birth; also Hgb Barts is present until about 3 months. Hgb H inclusions may be present. |
|
|
Term
| What is the O2 affinity of Hgb H compared to Hgb A? |
|
Definition
| The O2 affinity of Hgb H is 10x that of Hgb A. |
|
|
Term
| What stain is used to visualize Hgb H inclusions and heinz bodies? |
|
Definition
|
|
Term
| What is the fastest migrating hemoglobin on cellulose acetate at pH 8.4? |
|
Definition
|
|
Term
| Accelerate Fast Slow Crawls. What is this referring to? |
|
Definition
| The order of hemoglobins migrating in electrophoresis. |
|
|
Term
| In hemoglobin electrophoresis, are the hemoglobins going toward the positive or negative? |
|
Definition
|
|
Term
| A patient has a microcytic hypochromic anemia. Serum iron is decreased; TIBC is increased; % saturation is decreased. Anisocytosis and poikilocytosis, and target cells, are present on peripheral smear. Diagnosis? |
|
Definition
|
|
Term
| A patient has microcytic hypochromic anemia. Serum iron is decreased, TIBC is slightly decreased, % saturation is decreased. No anisocytosis or poikilocytosis is noted on the peripheral smear. Diagnosis? |
|
Definition
| Anemia of chronic disease. |
|
|
Term
| A patient has microcytic hypochromic anemia. Serum Fe is increased; serum ferritin is increased, ringed sideroblasts present in BM; % saturation is increased. The smear shows anisocytosis and poikilocytosis, target cells, and basophilic stippling. Diagnosis? |
|
Definition
|
|
Term
| Lead poisoning is associated with what anemia? |
|
Definition
|
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