Hematology Ch 9 + 10: IDA & ACD/AOI – Flashcards
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Aplastic Anemia
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reduced proliferation of red blood cells, can be acquired (drug related) or inherited. Exposure to oil wells burning of benzene. Hep B & C infections.
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Panctytopenia
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Bone Marrow Applasia. Reduction off all cell lines: RBC, WBC, platelets
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Red Cell Applasia
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hypoplastic proliferation of the RBC cell line
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In aplastic anemia, what are the lab findings?
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normocytic/normochromic, thrombocytopenia, increased iron,
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Treatment for aplastic anemia?
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immunosuppresive drugs, bone marrow transplant, and splenectomy
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Describe Fanconi's anemia
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Congenital/ Inherited, skeletal abnormalities, pancytopenia
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What are telomeres and how do they relate to aplastic anemia?
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short sequence of DNA located at the end of the chromosome that get shorter until the cell can no longer divide (apoptosis). Aplastic anemia and AML are associated with inherited mutations that inhibit in telomere repair = bone marrow failure
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What is shelterin?
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DNA protein that covers the telomere
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What are the molecular techniques used to identify telomeres in Aplastic Anemia?
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flow- FISH cytometry (most common), southern blot. Used to detect residual disease in bone marrow after treatment
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what are the lab findings in Bone Marrow Failure?
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macrocytosis (may be masked if patient has thalassemia or IDA), micromegkaryocytes, pelger-huet anomaly, nuclear/ cytoplasmic asynchrony
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What are the lab findings in Diamond Black Fan Anemia?
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anemia appearing prior to the first birthday, decreased neutrophil count, macrocytosis,
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Red Cell Aplasia?
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hypoproliferative RBC's, serum erythropoietin increase. Can be caused by infection and hemolytic anemia
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Black- fan Anemia?
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proapoptoic hematopoesis, bone marrow failure, birth defects, thrombocytosis, ribosomal disorder caused by deletion and translocation
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Causes of IDA?
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decreases iron intake, increase utilization( pregnancy, growth), excessive iron loss( menstruation), decreased absorbtion
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In a patient that has IDA, what might cause decreased absorbtion?
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H. pylori, autoimmune gasteritis, cancer, GI bleeding
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What is the avg. total iron in a normal adult? What is the normal iron loss per day?
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3.5-5.0g ; 1mg/day
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What may cause daily iron loss
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exfoliation of epithelial cells, skin cells, urinary excretion
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What is operation iron?
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Iron used for O2 binding and found in the heme portion of Hb and myoglobin
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What is transferrin?
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Glycoprotein produced by the liver, transports iron for the production of hemoglobin
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what is ferritin?
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Stores iron. Acute-phase reactant (class of proteins whose plasma concentrations increase in response to inflammation)
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How much of the iron in the body is made of iron salts?
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90% (iron salts is the non-reduced Non-heme iron) Fe3+
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What are the inhibitors and the enhancers of iron absorption?
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Enhancers: Ascorbic acid. Inhibitors: tanins intea
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Where is iron absorbed and stores
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Duodenum/ hepatocytes
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What are the 3 stages in IDA?
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pre-latent (decrease in storage of iron), latent( decrease in iron for erythropoiesis), Anemia (clinical)
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In IDA, describe the levels of serum iron, Total iron binding capacity, and serum ferritin?
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levels of serum iron decrease, Total iron binding capacity increases, and serum ferritin decreases
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What are the normal levels of levels of serum iron, Total iron binding capacity, reticulocyte, and serum ferritin?
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Iron 60-170g/dL, Total iron binding capacity 240-450 ug/dL, serum ferritin 12-300 ug/dL, reticulocyte >2.5%
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what is the relationship between total iron and transferrin?
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As total iron decreases, transferrin increases
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Describe the Soluble transferin receptor test?
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test for transferin receptors in body. detects increases erythropoesis. as the level of iron decreases, production of sTfR increases in cells. Provides differentiation of IDA from anemia of chronic inflammation
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What causes Anemia of Chronic Disease?
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second most prevalent after IDA, hypoproliferative, decreased erythropoietin caused by hepcidin which blocks release of iron
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What is the pathogenesis of Anemia of Chronic Disease?
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increased hepcidin induces iron sequestration and blockage of iron for erythropoiesis . Hemoglobin & Hematocrit will be low BUT transferrin & serum ferritin will be normal
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In ACD, describe the levels of serum iron, Total iron binding capacity, serum transferrin, and serum ferritin?
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levels of serum iron decrease, Total iron binding capacity decreases, serum transferrin decreases, and serum ferritin increases
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Constitutional Aplastic Anemia
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congenital or genetic predisposition to bone marrow failure
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iatrogenic
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a disease produced secondary to the treatment of the patient; often it is the result of side effects of the drug therapy chosen by the physician
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Exposure to this led to Aplastic Anemia
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Benzene from the burning oils in kuwait
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This antibiotic led to Aplastic Anemia
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Chloamphenicol
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These viruses have caused Aplastic Anemia?
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Hepatitis B &C, measels, Epstein Bar virus, cytomegalovirus
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What are the three phases of Aplastic Anemia?
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onset of disease, recovery, and late disease
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Describe the serum iron in aplastia anemia?
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Increased becasue the lack of iron turnover
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Describe the function of the enzyme telomerase?
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cause telomeres to shorten with age
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which leukemia is assosiated with aplastic anemia and the cause?
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AML and mutation in telomere
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What are the most common hematological abnormality in Bone Marrow Failures?
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macrocytosis, pelger huet anomallies
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This type of anemia occurs most frequently 25-30% of childhood Aplastic Anemia cases
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Fanconi's Anemia
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chelation therapy
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chemical process in which a synthetic solution—EDTA (ethylenediaminetetraacetic acid)—is injected into the bloodstream to remove heavy metals and/or minerals from the body. Chelation means "to grab" or "to bind.
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When total body iron is low, the levels of this___ increases, but the relative and absolute aounts of serum decline?
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Transferrin
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A patient with IDA would exhibit increased or decreased souluble trnsferrin receptors?
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increased
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In ACD/AOI one would expect the serum iron and the iron binding capacity to be?
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serum iron low, iron binding capacity to be high
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In ACD/AOI one would expect the ferritin to be?
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high