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anemia
| Question | Answer |
|---|---|
| anemia | decrease in hemoglobin, decrease in amount of oxygen reaching tissues, causing fatigue, weakness, and pallor |
| what is needed for anemia diagnosis | heredity (sickle cell), ethnic background (thalassemia), exposure to drugs/ chemicals (reducing substances) |
| most important lab results for anemia | Hemoglobin and hematocrit (H&H) with indices |
| decreased reticulocyte count | defective heme synthesis, replacement of normal BM with tumors, failure of BM to produce normal RBC's |
| increased reticulocyte count | increased RBC destruction, blood loss, bone marrow is capable of responding |
| M/E ratio should be | 3:1 to 4:1 |
| ineffective erythropoiesis has _______________ erythroid and BM and ____________ retics | increased; normal indicates a defect in maturation |
| serum tests in anemia: LDH, serum ferritin, bilirubin, haptoglobin | increased LDH in hemolytic anemia serum ferritin is directly proportional to body stores increased bilirubin in hemolytic anemia decreased haptoglobin in hemolytic anemia |
| hematopathology | study of diseases of the blood |
| hypoproliferative | not making an adequate number of RBCs |
| ineffective erythropoiesis | non effective production or maturation of RBCs |
| hemolytic | increased destruction of RBCs |
| we need _____ Fe/day to produce RBC's | 20-25 mg |
| ____% of iron comes from recylced RBCs | 95% |
| what is needed for iron absorption? | vitamin C |
| what blocks iron absorption | phosphate |
| hemosiderin | granules of excess Fe |
| sideroblasts | RBC precursors containing hemosiderin granules. |
| causes of IDA | poor diet, decreased absorption, distribution defects, increased requirements, iron loss |
| clinical picture of IDA | fatigue, lethargy, dizziness, HCT less than 30%, hypochromia, microcytic |
| hemochromatosis | inherited disorder that causes the body to absorb too much iron from food |
| sideroblastic anemias | Group of disorders characterized by a hypochromic anemia, ineffective erythropoiesis, serum and tissue Fe and the presence of ringed sideroblasts in BM; involve abnormalities of enzymes regulating heme synthesis |
| clinical picture of sideroblastic anemia | weakness, pallor, fatigue, over 50 yrs old, dimorphic RBC's with hypochromia, Anisopoikilocytosis, hypercellular BM, more than 15% ringed sideroblast |
| thalassemias | Group of hereditary anemias produced by either a defective production of alpha- or beta-hemoglobin polypeptide |
| megaloblastic anemia | DNA and nuclear maturation abnormalities with the production of large blood cell precursors with mature cytoplasm and immature nuclei |
| what does b12 need to be absorbed? | intrinsic factor (IF) |
| vitamin b12 is also referred to as | cobalamin |
| B12 function | Participates directly in DNA synthesis by acting as a methyl receptor for methyltetrahydrofolate (MTHF) |
| pernicious anemia (PA) | autoimmune condition where the stomach's parietal cells or intrinsic factor (IF) are attacked by the immune system. This prevents the body from absorbing vitamin B-12, leading to macrocytic anemia and potentially permanent neurological damage |
| symptoms of PA | lemon yellow skin, red and sore tongue, diarrhea, nausea, CNS degeneration of white matter causing numbness and pins & needles |
| peripheral blood picture of PA | pancytopenia, macrocytic + normochromic, basophilic stippling, nRBC's, H-J bodies, hypersegmentation of neutrophils |
| Schillings test | Part I: inject patient with B12, then orally administer radioactive B12, collect urine, in PA none will be recovered in the urine Part II: Give the patient oral radioactive Vit B12 – IF complex. Collect urine. if 7% is recovered, issue is with IF. |
| how much folate is needed per day | 50 ug/day |
| body stores how much folate | 4 months worth |
| aplasia | without tissue, bone marrow has shut down |
| hypoplasia | decreased tissue, bone marrow has decreased production of cells |
| pancytopenia | Decrease in all cell lines, reds, whites and platelets |
| aplastic anemia symptoms | bleeding (thrombocytopenia), normochromic, normocytic anemia with fatigue and weakness, |
| fanconi's anemia | genetic disorder that impairs the body's ability to repair damaged DNA, leading to bone marrow failure and a high risk of developing blood disorders |
| reference intervals for RBC's, WBC's, and PLT's | RBC: 4 - 5.5 x10^6/uL (women) or 4.5 - 6 x10^6 /uL WBC: 4-9 x10^9/uL PLT: 100-440 x10^3/uL |
| two important factors in deteminring anemia | heredity and ethnic background |
| significant WBC diff findings in anemia diagnosis | neutropenia (aplastic anemia) monocytosis hypersegmented neutrophils |
| increased LDH indicates | RBC destruction or abnormal production |
| decreased serum ferritin indicates | decreased iron (IDA) |
| increased serum bilirubin indicates | premature RBC destruction |
| normal haptoglobin | issue in RBC production |
| 4 blood forming organs | bone marrow, spleen, lymph nodes, thymus |
| what foods are food for non heme iron | egg yolks, legumes, veggies |
| IDA is most often characterized by what blood picture | microcytic and hypochromic |
| ferroportin | carrier for iron as it is absorbed in the intestine |
| koilonchia | thin and concave nails |
| pica | cravings for things that aren't food |
| pagophagia | craving for ice |
| sideroblasts vs siderocytes | both have hemosiderin granules, but blasts are nucleated and cytes are not |
| what is most often seen in RBCs in lead poisoning | basophilic stippling |
| hemosiderosis | buildup of hemosiderin granules |
| ferritin | storage form of iron |
| what blood hemoglobin abnormality exhibits the same blood picture as IDA | thalassemia |
| diagnosing features in hemolytic anemia | elevated retics, LDH and bilirubin, decreased haptoglobin, hemoglobinuria |
| where is b12 absorbed into the blood | the ileum |
| lab picture of PA | pancytopenia, macrocytic and normochromic |
| what is ascorbic acid and what does it do | vitamin C, which enhances absorption of non-heme iron |
| two stains for retics | new methylene blue and brilliant blue cresyl |
| 2 globin chains in normal hemoglobin A | 2 alpha and 2 beta |
| reference intervals for MCV, MCH, MCHC | MCV: 80-100 fL MCH: 27-31pg MCHC: 33-36 g/dL |
| what cell inclusions are stained with crystal violet | heinz bodies |
| 4 types of intracorpuscular defects | 1. sickle cell 2. thalassemia 3. G6PD deficiency 4. hereditary spherocytosis defect |
| thalassemias are characterized by what | a malfunction in the mRNA that eventually decreases the production of what |
| what happens in hereditary spherocytosis | RBC's lose their biconcave shape and become rigid, leading to them being destroyed |
| sugar water test tests for | fragile RBC's and anemia |
| what type of hemoglobin is resistant to aced elution | fetal hemoglobin (HbF) |
| hemosiderin is stained with what? positive cells in blood and BM | prussian blue siderocytes in blood sideroblasts in BM |
| ABO, Rh immunity causes what | alloimmune hemolytic anemia |
| in PNH, RBCs are sensitive to | activation by complement |
| why are red cells small in sideroblastic anemia | cant fully mature due to a lack of an enzyme causing a defect in hemoglobin synthesis |
| the peripheral blood smear in aplastic anemia shows | pancytopenia |
| which population of cells is thought to be damaged in the BM, which results in pancytopenia in the peripheral blood in aplastic anemia | pluripotent stem cells |
| BM in aplastic anemia shows | hypoplasia |
| congenital disease associated with aplastic anemia | fanconi's anemia |
| whats a drug that causes aplastic anemia | phenylbutazone |
| RBC indices in aplastic anemia | normocytic/ normochromic |
| what is not going to be in the peripheral blood of a patient with aplastic anemia | reticulocytosis |
| BM aspiration of a patient with aplastic anemia results in | dry tap |
| bone biopsy in aplastic anemia reveals | hypocellularity |
| patient younger than 50 with aplastic anemia is going to be treated with | BM transplants |
| bone marrow transplants often have the complications of | graft rejection and acute graft versus host disease |
| patient has associated aplastic anemia syndrome. BM has normal cellularity with absence of erythroid precursors. | purre red cell aplasia |
| what is a characteristic of congenital dyserhtyropoietic anemia (CDA) | ineffective erythropoiesis |
| CDA type 1 is characterized by | thin internuclear chromatin bridges between 2 erythroblasts, megaloblastic synchrony, and multinuclear erythroblasts |
| CDA type 2 is characterized by | positive HEMPAS test |
| CDA type 3 characteristic finding | giant nucleated erythroblasts |
| what is an example of an acquired intracorpuscular defect | paroxysmal nocturnal hemoglobinuria |
| the abnormal red cell membrane in PNH is highly sensitive to | complement |
| which lab findings are associated with chronic hemolysis of PNH | leukopenia and thrombocytopenia |
| a positive Ham's test results shows | hemolysis occurs with a patients cells but not control cells |
| the defect in PNH is associated with | abnormal clone of hematopoietic stem cells |
| which classes of PNH is deficient in both decay-accelerating factor (DAF) and membrane inhibitor of reactive lysis (MIRL) | PNH II and III |
| the presentation of hemoglobinuria in a PNH patient is due to | intravascular hemolysis |
| which condition will lead to iron deficiency anemia masking the diagnosis of PNH | hemosiderinuria |
| one of the major complications of PNH is | venous thrombosis |
| the diagnosis of PNH depends on the detection of complement sensitive ___________ in the peripheral blood | erythrocytes |
| most PNH patients present with a normocytic/normochromic anemia and | cytopenia |
| slight macrocytosis or polychromasia may be seen in the peripheral blood of a PNH patient due to increased | retics |
| schistocytes or fragmented red cells seen on a peripheral blood smear of a PNH may indicate | intravascular thrombosis |
| what helps to determine the presence of PNH | decrease in acetylcholinesterase |
| typical PLT count in PNH | <50 x 10^9/L |
| a person with PNH may have __________________ in the urine | hemoglobin casts |
| the bone marrow in a PNH patient reveals | erythroid hyperplasia |
| in differentiating PNH red cells from normal red cells in the sugar water test, PNH red cells will be | lysed |
| what percentage of cell lysis in a sugar water test is considered negative for PNH | <5% |
| what test is used to confirm the diagnosis of PNH | ham's test |
| what membrane deficiency renders PNH erythrocytes more sensitive to lysis by complement | GPI-anchor proteins |
| what immunophenotype is associated with a diagnosis of PNH | deficiency of CD55 and 59 |
| the most common cause of death in a PNH patient is | thromboembolism |
| what is the function of decay accelerating factor (DAF) as a complement protein | accelerates the spontaneous decay of C3 convertase |
| what cell lineage is affected by megaloblastic change | lymphocytic |
| what is the primary cause of B12 deficiency | malabsorption |
| in megaloblastic anemia, the absolute retic count is decreased from | ineffective erythropoiesis |
| what is the leading cause of anemia in an alcoholic | folate deficiency |
| the presence of polychromatophilic macrocytes in megaloblastic anemia is due to | megaloblastic dyspoiesis |