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206 Cards in this Set
- Front
- Back
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How much oxygen can one gram of hemoglobin hold?
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1.34 mL
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What form of iron is normally in unbound heme?
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Ferrous
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What is methemoglobin? How much does a normal person have?
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when the iron is oxidized to the ferric (3+). A normal person has 1.5% methemoglobin- becomes cyanotic at 10%
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How does the cell reduce methemoglobin that is formed naturally?
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Different reduction systems: the most important is the NADH-cytochrome B5 reductase. Also, ascorbic acid, glutathione, and NADPH methemoglobin reductase, which needs methylene blue as a cofactor.
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What is Hb M?
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A kind of methemoglobinemia in which alternate hemoglobins that have a tendency to be in the ferric (methemoglobin) state. Reducing enzyme systems in the cell are intact, and treatment with methylene blue doesn't work.
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Drugs that oxidize Hb to methemoglobin (Hi)?
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nitrates, nitrites, chlorates, and quinones.
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People who have mild decreases in NADH cytochrome B5 reductase?
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May be more sensitive to oxidizing drugs (Hb more readily becomes Hi).
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How should you treat acute toxic methemoglobinemia?
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methylene blue (other things like glutathione or ascorbic acid) are better for people with genetic deficiencies of the reducing enzymes.
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Cyanide posioning?
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Give an oxidizing agent (like nitrites) to oxidize Hb to Hi. Hi will bind to cyanide and can be excreted.
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Sulfhemoglobin?
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Formed by inrreversible oxidized hemoglobin. Turns blood blue-green.
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Affinity of Hb for CO vs. O2?
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200 times greater.
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How does the hemiglobincyanide method for determining Hb concentration work?
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The sample is oxidized to make Hi, and then reacted with CN to make HiCN which has a broad absorbance peak at 540 nm.
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What kind of hemoglobin cannot be measured by the cyanohemiglobin method?
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Sulfhemoglobin. This method oxidizes hemoglobin to Hi so it can make HiCN which is measured at 540 nm. Sulfhemoglobin is too far oxidized to do this.
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What is hematocrit?
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Volume of erythrocytes divided by the volume of whole blood.
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What happens to MCV and hematocrit for a sample that has been sitting around at room temp?
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They both increase because the red cells begin to swell.
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What is the MCHC? How do you calculate it?
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The average amount of hemoglobin in a fixed volume of blood. [Hb]/Hematocrit
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1 mm^3 equals?
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1 microliter.
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What are reticulocytes? Why are they called that?
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Nonnucleated RBCs with lots of RNA making Hb. If you incubate the sample with methylene blue, the RNA precipitates out and make a blue meshwork in the cell.
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What is a Miller disk?
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A way to rapidly count cells per RBC- has two squares of different sizes.
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Manual method of counting leukocytes?
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Hemocytometer method. Dilute, lyse the red cells, put in a special slide and count.
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Two things that can cause an increased MPV?
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hyperthyroidism and hematopoietic disorders,
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Least reproducible blood cell count?
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Manual platelet count.
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Capillary vs venous blood re: red cell count and hemoglobin?
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Capillary counts are higher due to loss of fluid.
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Diurnal variation of leukocytes?
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Highest in the afternoon, lowest in the morning.
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Plasma factors that influence ESR?
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Fibrinogen or globulins promote rouleaux formation which accelerates ESR. Albumin and lecithin decrease ESR.
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Red cell factors that influence ESR?
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Anemia: more plasma proteins relatively, so increase the ESR.
Microcytes sediment slower than larger cells. Funny-shaped cells (sickle, etc) inhibit rouleaux formation and slow ESR |
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Reference method for ESR? Caveat?
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Westergren method. Can't use whole blood with a hematocrit greater than 35%, have to dilute.
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Difference between Westergren and modified Westergren methods?
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Modified Westergren can be done with EDTA anticoagulated blood (Westergren needs blood in citrate).
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How does Coulter counter work?
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Cells go through an aperature which causes impedance to the current going through it. Machine measures:
Impedance (# of deflections) = number of cells (RBC, WBC, plt) Magnitude of impedance = volume of cells (MCV, MPV) Conductivity and side scatter = cells opacity and complexity. |
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In the coulter counter, what is conductivity good for?
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Cells that are the same size but have different granularity (internal characteristics)
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Spurious increase in WBC?
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Nucleated RBCs, platelet clumping.
Heparin, monoclonal antibodies, and cryoglobulin |
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Spurious increase in hemoglobin?
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Carboxyhemoglobin, high white count, hemolysis, cryoglobulin, lipemia, hyperbilirubinemia, heparin
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Spurious decrease in hematocrit?
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Clotting, hemolysis, autoagglutination.
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Spuriously high platelets?
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Hemolysis, microcytic red cells, red cell inclusions, white cell fragments.
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When using the wedge method to make a blood smear, what are two ways to make the film thicker?
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1) Increase the angle of the spreader slide, 2) Push the spreader slide faster. Also, if the hematocrit is low, then increase the angle of the spreader slide (since the blood is thin, you'll want to make a thicker smear).
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What's in Wright's Stain?
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Eosin, methylene blue, azure B
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How do you stain with Wright's stain?
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Fix in methyl alcohol, immerse in stain for 2 minutes, wash, air dry
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What is Wright's stain is too blue? Too pink?
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Too blue: Too much dye, not enough washing, buffer too alkaline. Too pink: Not enough dye, not enough washing, buffer too acidic (or methyl alcohol is turning into formic acid)
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Reference Romanowsky stain?
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Eosin Y and azure B.
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MCH and MCHC in: 1) megaloblastic anemia?, 2) Spherocytosis?
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1) MCH is increased but MCHC is normal. 2) MCH is normal but MCHC is increased
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MCV of spherocytes?
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Should be normal: they have a smaller diameter, but should have the same volume.
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Besides hereditary elliptocytosis, when else do you see a lot of elliptocytes?
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Iron deficiency anemia, myelofibrosis, megaloblastic anemia, and sickle cell anemia.
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When do you see spherocytes?
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Hereditary, AIHA, or when there has been damage to red cells, like body burns.
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When do you see Target cells?
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Hypochromic anemias, liver disease, Hemoglobin C disease, and post-splenectomy.
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Difference between acanthocytes and echinocytes?
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Acanthocytes: irregularly spiked with rounded/bulbous ends. Echinocytes are regularly spiked with sharp edges.
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Fine vs coarse basophilic stippling in red cells?
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Fine: just RNA, indicating increased red cell production. Coarse: lead posioning, megaloblastic anemia
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Pappenheimer bodies?
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A few blue granules in the red cell: are inorganic iron granules. Rarely seen in the blood except after splenectomy.
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Howell-Jolly bodies?
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Chromatin remnants- can see post-splenectomy, megaloblastic anemia.
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Fine granules seen in red cells that have malaria in them?
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Schuffner granules.
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What is leukoerythroblastosis and what does it mean?
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When you see immature red cells and white cells in the peripheral circulation. Indicates some sort of space-occupying lesion in the bone marrow: myelofibrosis, carcinoma, myeloma, Gaucher's disease, etc.
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One advantage of coverslip method over the wedge method of making blood smear?
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cells (like monocytes, blasts) tend to be unevenly distributed in the wedge method.
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neutrophils in black people?
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Can be lower than in whites, down to 1.1 x 10^9
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What does the Rumke table show?
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That as you count more and more cells, a difference in cell number is less likely to be due to sampling error and more likely to be real.
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A normal platelet count, one platelet per how many red cells?
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10-30
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What fixative should you use for bone marrow core and clot?
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B5 for 1-2 hours or Zenkers for 6-18 hours.
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What does the Perls' test for iron stain?
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It is a Prussian blue stain that stains ferritin and hemosiderin (but not the iron in hemoglobin).
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Other non-hematopoietic kinds of cells that you can see in bone marrow aspirate?
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mast cells, osteoblasts, osteoclasts.
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Bony trabeculae with osteoid seams?
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Osteomalacia (Vitamin D deficiency)
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What is ferritin?
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A ferric salt with apoferritin protein surrounding it.
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WHat is CD71?
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It is the transferrin receptor on normoblasts.
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Why do you see elliptocytes in iron-deficiency anemia?
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Because the cell membranes are abnormally stiff.
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Things that can decrease TIBC?
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chronic disease. Also, pregnancy and oral contraceptives.
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What can you see normal/high ferritin levels, but the person is still iron deficient?
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If the person has chronic inflammation or malignancy, because ferritin is an acute phase reactant.
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What causes megaloblasts?
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Cells have diminished capacity to make DNA, but not RNA: thus the cytoplasm can grow and develop and will be big.
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Most common autoantibodies seen in patients with pernicious anemia?
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First: antiparietal cell antibodies. Second: anti-intrinsic factor antibodies.
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Anatomic site of absorption for cobalamin and folic acid?
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Cobalamin is absorbed in the lower small bowel and folic acid is absorbed in the upper small bowel.
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What parasite is associated with B12 deficiency?
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Diphyllbothrium latum (fish tapeworm: seen in Denmark)
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Methylmalonic acid?
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Builds up in cobalamin deficiency and can see it in the urine.
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Deoxyuridine suppression test? Why is it good?
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A test for cobalamin and folate deficiency. Incubate cells in vitro with labeled thymidine. If cells can't convert deoxyuridine to thymidine by themselves, they'll use the labeled thymidine and will see more in the DNA. Good because it's very sensitive and doesn't use many cells (can use for children and babies).
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How does the Schilling test work?
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Give oral radioactive B12. Then inject with regular B12. If the B12 is low in the urine (should be pretty high now), the person's not absorbing it. So then repeat but give with pig IF. If the urine excretion increases then the person just has low IF. If it doesn't, then there's some intestinal reason they're not absorbing it.
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methotrexate?
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A folic acid antagonist.
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What are the microbiologic assays for cobalamin and folate?
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For cobalamin: Euglena gracilis. For folate: lactobacillus casei.
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Urinary FIGLU test?
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Give someone a bunch of histidine: if they have folate deficiency, then FIGLU (which is a metabolite of histidine) will build up and you can see in the urine.
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Serum folate levels and folate deficiency?
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Low serum folate only shows a negative folate balance, not necessarily a tissue deficiency.
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Acute megaloblastic anemia?
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Nitrous oxide anesthesia.
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Indices in anemia of chronic disease?
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Serum iron is low, TIBC is normal or elevated, serum ferritin is elevated.
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Neocytolysis?
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Youngest red cells are destroyed first. Seen with relative EPO deficiency (as in anemia of chronic disease).
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Indices necessary to call "severe aplastic anemia"?
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granulocytes less than 0.5 x 10^9, platelets less than 20, reticulocytes less than 40 000 per microliter.
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Things that can cause a toxic dose-dependent aplastic anemia?
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ionizing radiation, benzene, mustard compounts, busulfan, urethane, antimetabolites.
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Things that can cause a hypersensitivity aplastic anemia?
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chloramphenical, many others.
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Fanconi anemia?
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A familial aplastic anemia. 1/300 carrier frequency. Have anemia, hyperpigmentation, short, small head, radial aplasia. Due to increased chromosomal fragility. See especial chromosomal breakage when cultured cells are subjected to alkylating and DNA cross-linking agents such as mitomycin C.
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Mutated gene in amegakaryocytic thrombocytopenia?
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Gene for thrombopoetin receptor.
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Dyskeratosis congenita and Schwaumann-Diamond syndrome?
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Both inherited forms of aplastic anemia. DC have a lot of skin stuff. SD have excorine pancreatic insufficiency and neutropenia.
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Transient erythroblastosis of childhood?
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Anemia and leukopenia, but thrombocytosis often. Usually post-viral. Recover on their own.
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Diamond-Blackfan Anemia (and vs. TEC)?
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A congenital red cell aplasia. Usually, increased HbF, antigen i is present, red cell adenosine deaminase is increased (all in contrast to Transient erythroblastosis of childhood)
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Association of acquired pure red cell aplasia?
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thymoma. 5-10% of those with thymoma have the anemia. 50% of the anemia is associated with thymoma.
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What are some drugs/toxins that can cause sideroblastic anemia?
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antiTB drugs, lead poisoning, chloramphenicol, copper def or zinc overloas (also causes vacuolated marrow cells), ethanol-induced anemia, primary pyridoxine deficiency.
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Increased urobilinogen in urine or feces?
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Means extravascular hemolysis.
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MCV and MCHC in hereditary spherocytosis?
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MCV is normal and MCHC is increased.
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Osmotic fragility test in spherocytosis?
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The cells have a limited capability to expand with hypotonic solutions, so they will burst at a higher concentration of NaCl (at a less hypotonic solution) than normal. Called: "increased osmotic fragility".
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Aplastic anemia and PNH?
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40% of people with AA at the time of diagnosis have evidence of PNH clones.
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Defect in pNH?
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Defect in GPI which links certain proteins to the cell membrane (like DAF and MIRL).
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Why does PNH cause thrombosis?
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Lack of CD59 on platelets causes them to externalize phosphatidyl serine on their membranes which is prothrombotic.
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Describe the Ham test for PNH.
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It is the acidified serum test. Add normal serum, HCl, and pt's blood and watch for hemolysis. Heating the normal serum will eliminate hemolysis (because it will denature complement proteins).
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Why does HbF have a higher affinity for oxygen than HbA?
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It has less affinity for 2,3-DPG.
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What % should HbF be at 6 months? 1 year?
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8%, less than 5%
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What is HgbA2? When do you see it?
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It has two alpha and two delta chains. Seen almost exclusively in beta-thalassemias.
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What are zeta and eta globin?
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Zeta is the embryonic analogue of the alpha globin and eta is the embryonic analogue of the beta globin.
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On which chromosomes are the alpha and beta globin genes?
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Alpha is on chromosome 16 and beta is on chromosome 11.
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Method for initial screening for hemoglobin variants?
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Cation-exchange HPLC.
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Alkali denaturation test for fetal hemoglobin?
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HbF is more resistant to alkalinity than HbA. Use this test to get rid of adult Hgb and quantitate HbF.
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What interferes with HbA2 quantification on cation-exchange HPLC?
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The presence of HbS
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How does the sickling test work?
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Add a reducing substance to red cells (which enhances deoxygenation) and look for sickling.
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With a structural variant of the beta chain TRAIT, how much of the variant Hb will you see? What is the ramification of this?
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With a strucutural beta variant, usually doesn't complex with alpha so well, so only 40%. With alpha thalassemia, it has an even harder time competing, so the variant Hb will be even less.
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What is the order and what comigrates with what on alkaline and acid electrophoresis?
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Alkaline: A, F, S (with D, G, Lepore, and Hasahron), and C (with A2 and E).
Acid: C, S (with Hasharon), A (with A2, E, D, G, Lepore), F |
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How much Hb A is produced in Hb SC disease?
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None (there is no normal beta globulin produced)
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When does Hb S polymerize?
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when it is deoxygenated.
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What is the clinical for Hb SC disease? The peripheral smear?
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Clinically kind of like sickle cell anemia, but milder. Peripheral smear has lots of target cells and some sickle cells.
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How can you tell Hb S disease from Hb S/beta-0?
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In Hb S/beta-0, MCV and MCH are decreased and A2 can be markedly elevated. Also, in Hb S/beta-0, the spleen remains enlarged throughout life.
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How can you tell Hb S/beta+ from HbAS?
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Hb S/beta+ has more Hb S than Hb A (in the Hb AS, there is more Hb A and Hb S is only like 40%)
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What other entity cannot be distinguished from Hb S/ beta-0 disease?
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HB SS/alpha+ thal
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What other entity clinically looks like Hb SC disease?
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Hb SD disease.
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Clinical for Hb C disease?
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A mild hemolytic anemia with splenomegaly.
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Hexagonal crystals in blood smear: why don't they cause vaso-occlusive diease?
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Hb C. They melt at low pO2.
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Most common Hb D variant in African Americans?
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Hb D Los Angeles (Punjab)
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Hb G?
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An alpha chain variant. Will see a little blip next to A2 (because there is alpha2delta2 and alphaG2delta2)
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Who gets hemoglobin E disease the most?
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Thai and southeast asians.
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Unstable hemoglobin that is okay in vivo?
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Hemoglobin E
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What do abnormally unstable hemoglobin variants cause?
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They precipitate out at Heinz bodies and the RBCs are damaged and get removed by the spleen.
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What is the isopropranol precipitation test?
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A test for unstable hemoglobin variants. Based on the principle that nonpolar solvent can weaken the intermolecular bonds of hemoglobin, so if a variant is less stable than normal, it will precipitate out faster. HbF can give a false positive result, though.
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What kind of mutations are commonly seen in beta thalassemia? Alpha thalassemia?
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point mutations in beta thal. Large deletions in alpha thal.
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What is the difference between beta-0 and beta+ thal?
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Beta-0 gene means that the whole gene is knocked out. Beta+ means that there is some B being made, but very reduced in amount. Beta thal major means you get two abnormal genes. Beta thal minor means you get one abnormal gene.
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What chromosomes are the hemoglobin genes on?
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Alpha and zeta are on 16.
Beta and beta-like are on 11. |
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Hb Constant Spring?
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A mutation in alpha causes it to be longer that normal, and the mRNA is unstable, so there is less alpha chain made. Clinically, looks like alpha thalassemia.
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Why causes disease in beta thal?
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Excess alpha, which does not form tetramers, but damages the red cell membrane. Ineffective erythropoiesis.
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X-ray findings of thinned cortex and enlarged bone marrow cavity?
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Beta thalassemia (bone marrow hyperplasia due to ineffective erythropoiesis).
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Bizarre target cells, extreme poikilocytosis, and Howell-Jolly bodies?
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Beta-thalassemia.
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What does the bone marrow look like in Beta thalassemia?
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Ineffective erythropoiesis: increased storage iron, sideroblasts, Gaucher-like cells, normoblast hyperplasia.
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Indices in beta thal trait?
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A mild anemia. RBC count is increased, MCV is low, MCH is low.
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Beta thal trait but really elevated HbF?
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Think of the gene for Hereditary Persistent Fetal Hemoglobin.
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A2 greater than 10% on HPLC?
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Probably not because of beta thal: think of something else: A variant hemoglobin that is coming off the column at the same time as A2.
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Thalassemia indices with really elevated HbF?
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Think of (deltabeta)0 thalassemia. Missing a delta gene can mask beta-thal. You expect someone with beta thal to have increased Hgb A2, but if they are missing a delta gene, then it won't be elevated in them.
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What is hemoglobin Lepore?
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An abnormal globin gene produced by a fused delta beta gene (from improper crossing-over).
They have BOTH a hemoglobinopathy AND a thalassemia (because the fused gene is under the control of the delta promoter and is produced in limited quantities). |
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Overall clinical picture of alpha thal compared to beta thal?
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Alpha thal is more of hemolytic anemia (bc gamma and beta tetramers are somewhat stable). Beta thal is more of an ineffective erythropoiesis picture.
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Thalassemia that is uncommon in black people?
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Alpha-0 and Hb H disease.
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Where do Hb Bart and Hb H migrate on alkaline elctrophoresis?
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Far: they are fast migrators.
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Incubating RBC is cresyl blue and there are many round inclusions attached to the membrane?
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Think precipitated Hb H.
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Why is the HMP shunt important to red cells?
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Replenishes NADPH for reduction (G6P dehydrogenase and 6-phosphogluconate are involved).
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What happens if there is an enzyme deficiency in the HMP shunt pathway in the red cell?
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If there is oxidant stress, the HMP pathway cannot make things to reduce hemoglobin fast enough. It gets oxidized, precipitates out as Heinz bodies and causes the cell to lyse.
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Deficiencies of glycolysis pathway in the red cell?
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Also cause a hemolytic anemia.
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Pattern of inheritance of G6PD deficiency?
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X linked
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Cells that are preferentially destroyed in G6PD deficiency and why?
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Old cells because they usually have less G6PD activity anyway then young cells.
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What stain do you use to show Heinz bodies?
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Methyl violet or crystal violet
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What gives a positive ascorbate cyanide test?
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A test for G6PD deficiency mostly. H2O2 is formed, and cyanide inhbits catalase, so the peroxide oxidizes the hemoglobin to methemoglobin. In G6PD deficiency, the HMP shunt reduction system is decreased, so can't go back. Also positive in pyruvate kinase, PNH, and unstable hemoglobins
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Recommended screening test for G6PD deficiency and how does it work?
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Fluorescent spot test. Basically, if you have G6PD, you will make NADPH on the spot and it will fluoresce. If it doesn't, then that is a positive result.
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Why does pyruvate kinase def patients tolerate their anemia so well?
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2,3-DPG increases since there is a block in glycolysis, so there is enough tissue unloading of oxygen.
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Caveat with using fluorescent spot test for PK deficiency?
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Have to remove the leukocytes: they have a shit ton of PK. In PK deficiency, red cells lack PK, but the white cells do not.
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How do you diagnose Fanconi's Anemia?
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Increased chromosomal breakage in lymphocytes cultured in the presence of DNA-crosslinking agents such as mitomycin C
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Things the Coulter counter can tell you directly?
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# of cells (RBC, WBC, plt)
size of cells (MCV and MPV) distribution of size (RDW) |
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Wintrobe method?
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The old school way of measuring hematocrit.
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RBC indices that are indirectly measured, and how are they measured?
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Hct = MCV x RBC
MCH = Hgb/RBC MCHC = Hgb/Hct |
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How does the coulter counter determine the MCV?
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The mean of the magnitude of the impedance divided by the number of deflections.
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Things besides Hb SS that will give a positive sickling test?
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Hgb AS, Hgb SC, C harlem, Hgb Barts (anything with an S)
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Why would a Hgb AS neonate give a negative sickling test?
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Because they have so much Hgb F, that there Hgb S is less than 10% so looks negative.
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What is the alkaline denaturation test for Hgb F?
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Hgb F is resistant to alkaline denaturation, so treat the blood with this to get rid of the Hgb A and then requantitate the Hgb.
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Some reasons for increased Hgb F?
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SS, beta thal, childhood CML, B12/FA deficiency, epogen
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Pneumonic for alkaline and acid hemoglobin electrophoresis?
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Alkaline:
origin C/A2/E S/D/G/Lepore F A + P(an), a frozen section conundrum! Acid: F A/everything origin S C + Call surgeon, Ask forgiveness |
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What is a normal A2?
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Less than 3%. It's at the end of the HPLC graph.
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Dye that is used for reticulocytes?
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Methylene blue
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What are "thalassemic indices"?
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Low hct
low MCV increased RBC |
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Features of May-Hegglin anomaly
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Autosomal dominant thrombocytopenia with giant platelets and dohle-like bodies.
Often asymptomatic and usually no treatment is required. associated with deafness. |
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Platelet aggro for Bernard-soulier and Glanzmanns?
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B-S: aggregates to everything except risto.
G-T: only aggregates to risto and nothing else. |
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Features of Chediak-higashi?
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Autosomal recessive, bad disease with oculocutaneous albism and recurrent bacterial infections.
Big crazy granules (all different sizes) in granulocytes and platelet dense core storage dysfunction. |
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Granulocyte anomaly seen in Hunters/Hurlers/mucopolysaccharidosis
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Alder-reilly anomaly. Large prominent granules in the granulocytes.
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What kind of red cells do you see in pyruvate kinase deficiency?
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Echinocytes
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Three categories of things that cause intravascular hemolysis?
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1) Complement (PNH, PCH, cold)
2) MAHA (DIC, TTP, mech. valves) 3) Toxic environment (bacteria, malaria, drugs) Generally, other things cause extravascular hemolysis |
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Other causes for stomatocytes?
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Rhnull and liver disease.
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Differential diagnosis of Heinz body/blister cell/bite cells?
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G6PD deficiency
Unstable hemoglobins (Hb H, Barts) Sulfhemoglobinemia |
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Differential for echinocytes?
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Artifact, pyruvate kinase deficiency, uremia, burns
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Causes for acanthocytes?
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Liver disease, abetalipoproteinemia, and Mcleod phenotype.
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Three things to know about congenital dyserythropoietic anemia?
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They have multinucleated erythroid precursors.
Type II is the most common. They have a lot of I antigen. |
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What is the function of clathrin?
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receptor-mediated endocytosis.
(trans-Golgi --> lysosomes, and plasma memb --> endosomes) Used to transport proteins between cells |
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Differential for Zinc ProtoPorphyrin (ZPP) elevation?
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Lead posioning, iron deficiency anemia, anemia of chronic disease, and sideroblastic anemia.
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Differential for basophilic stippling?
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Lead poisoning, thalassemias, hemoglobinopathies, sideroblastic anemia, and pyrimidine-5' nucleotidase deficiency.
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Hemoglobin H?
Hemoglobin Barts? |
H is four beta.
Barts is four gamma. |
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Most common cause of hydrops fetalis in southeast asians?
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Alpha-thalassemia
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Hermansk-Pudlak syndrome?
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Problem with granule-making. Oculocutaneous albinism, storage pool defect, and pulmonary fibrosis.
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Cabot's ring?
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Nuclear remnant. Seen in lead toxicity, pernicious anemia, and hemolytic anemia.
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Half-life of a RBC?
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Based on 51 Chr it's 30 days, but it's really 60 days (nl lifespan is 120 days)
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What's in a red/gray top tube?
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A clot activator and silicon separator.
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Royal blue tube?
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For trace element studies.
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Yellow top tube?
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ACD (citrate)- for samples that must be kept at room temperature and shipped (like immunology reference, etc)
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WHat does pyropoikilocytosis look like on smear?
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Elliptocytes.
Hereditary. Red cells will lyse at 45 degrees (normal RBCs can stand up to 49 degrees). |
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What's in a Dohle body?
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Free ribosomes from rough ER with glycogen
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Best way to differentiate TMA of Downs with AML?
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Clinical observation
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HB indices for Hb beta+/S
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HbS- 60-80%
Hb A- 15-30% HBA2- 3-8% (slightly elevated) HBF- 2-20% |
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Features of sideroblastic anemia?
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A group of disorders characterized by anemia and sideroblasts in the BM. Bone marrow with DIMORPHIC appearance. Also in the BM: erythroid hyperplasia +/- dysplasia, increased iron stores.
Serum iron: high Ferritin: high Transferrin saturation: high TIBC: normal to low |
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How do you calculate transferrin saturation?
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Serum fe/TIBC X 100
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How do you measure transferrin?
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TIBC is an indirect measure of.
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Soluble transferrin receptor?
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Measures RBCs putting transferring receptors on their membranes. This count is not affected by inflammation!
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Smear findings with lead posioning?
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Hypochromic normocytic anemia with basophilic stippling.
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Difference in screening between inorganic and organic lead poisoning?
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Inorganic: Screen with increased zinc protoporphyrin.
Organic: Screen with decreased urine ALAD. |
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Protoporphyrin in Fe-deficiency anemia?
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Both Free ereythrocyte protoporphyrin and zinc protoporphyrin are elevated.
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Positive sickledex test?
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A positive test is turbidity. A negative test is clear.
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Defect in hereditary elliptocytosis?
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Spectrin defect.
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MMA in the urine?
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In B12 deficiency, but not folate deficiency.
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Where is heme produced?
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In the mitochondria.
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When you break hemoglobin down, what are it's components?
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Heme (which protoporphyrin plus iron) and globin (protein part).
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Do you see sideroblasts in anemia of chronic disease?
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No.
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RBC have which MHC molecules?
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None (they are not nucleated).
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RDW in iron def anemia vs. heterozygous thalassemia?
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It is elevated in iron deficiency anemia and normal in heterozygous thalassemia.
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Falsely elevated Hgb A1c?
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Iron def anemia, hemoglobinopathies (and thalassemia), and B12/folate deficiency.
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Beta thal minor and iron def?
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May decreased A2 and patient may look like alpha thal.
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What happens with stomatocytes?
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They will give a hemolytic anemia. If you take out their spleen, they have an increased risk of thrombosis.
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Which anemia is associated with an increased LDH?
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Hemolytic (including megaloblastic).
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Things with reduced osmotic fragility?
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States where there are codocytes and stiffer cells: thalassemia, Hb SS, iron deficiency anemia
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