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98 Cards in this Set
- Front
- Back
In what setting would you see hypersegmented neutrophils?
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In megaloblastic anemia as a result of folate or B12 deficiency.
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What are 4 etiologies of target cells?
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HbC disease, Asplenia, Liver disease, Thalasemia. HALT said the hunter to his target.
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A blood smear filled with Burr cells is associated with what pathology?
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Thrombotic thrombocytopenic purpura/hemolytic uremic syndrome
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A blood smear filled with acanthocytes (spur cells) is associated with what pathology?
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Abetalipoproteinemia, liver disease
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What diseases are associated with basophilic stippling on blood smear?
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Thalassemia, Anemia of chronic disease, Iron deficiency, Lead poisoning (remember: TAIL)
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What are Heinz bodies?
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Heinz bodies are accumulations of denatured hemoglobin in RBCs due to the oxidation of iron
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What diseases are associated with Heinz bodies?
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α-Thalassemia, G6PD deficiency
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What are Howell-Jowell bodies?
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Nuclear remnants found in RBCs
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When are Howell-Jowell bodies seen?
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In patients with asplenia or functional hyposplenia
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What are three causes of microcytic anemia?
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Iron deficiency, thalassemia, lead poisoning
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What lab findings would you expect in iron deficiency anemia?
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Low serum iron, high TIBC, and low ferritin
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What blood smear findings would you expect to see in thalassemias?
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Target cells and Heinz bodies (in alpha)
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Name three causes of macrocytic anemia.
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Vitamin B12 deficiency, folate deficiency, Grab bag of non-megaloblastic causes
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Name some causes of normocytic, normochromic anemia.
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Acute hemorrhage, G6PD deficiency, pyruvate kinase deficiency, RBC membrane defects, bone marrow disease, hemoglobinopathies, autoimmune hemolytic anemia, anemia of chronic disease
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What lab values suggest hemolysis as the cause of normocytic anemia?
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Decreased haptoglobin, increased LDH
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How do you differentiate between autoimmune and non-autoimmune hemolysis?
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Positive direct coombs test suggests autoimmune hemolysis
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What lab values are suggestive of anemia of chronic disease?
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Low TIBC, high ferritin, high storage of iron in macrophages in bone marrow
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What is the pathogenesis of microcytic anemias?
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Any defect in hemoglobin synthesis
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What are four diseases that result in microcytic anemias
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Iron deficiency, Sideroblastic anemia, thalassemia, lead poisoning
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What findings do you expect to see in a peripheral smear of somebody with iron deficiency anemia?
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Microcytosis and hypochromia
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What syndrome may iron deficiency anemia manifest as?
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Plummer-Vinson syndrome (iron deficiency anemia, esophageal web, and atrophic glossitis).
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Where is alpha thalasemia present?
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Asia and Africa
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In what gene is there a defect in alpha thalasemia?
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at least 3 the alpha-globin genes (1-2 of them result with no findings)
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What is the disease called that results from the deletion of 3 alpha-globin genes?
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HbH (beta-4)
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What is the disease called that results from the deletion of 4 alpha-globulin genes?
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Hb Barts
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What does Hb barts cause?
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hydrops fetalis
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In what population is B-thalasemia prevelent?
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Mediterraneans
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What kind of mutations are present in B-thalassemia?
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point mutations in splicing sites and promoter sequences. This leads to under production (just like the mutations in alpha-globulin)
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What is Beta-thalasemia minor and how would you detect it?
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It is a heterozygote with a B-globulin gene mutation. They are usually asymptomatic and would only yeild an increase in HbA2 (A hemoglobin with delta globulin, usually a very small amount of the total).
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What is B-thalasemia major?
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Homozygous mutation of the B-globulin gene.
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What does B-thal major lead to?
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Severe anemia requiring blood transfusions (secondary hemachromatosis)
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What finding would be present in a person with B-thal major?
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crew cut head xray-> skeletal deformaties, chipmunk facies. You would also have an increase in HbF (this would also be increased in b-thal minor)
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How does lead poisoning cause microcytic anemia?
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it inhibits ferrochelatase and ALA dehydratase which decreases heme synthesis. It also inhibits RNA degradation which causes basophilic stippling.
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What is sideroblastic anemia caused by?
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a defect in heme synthesis.
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What gene is defective in hereditary sideroblastic anemia?
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delta-aminoleuvulinic acid synthase (X-linked)
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How do you treat sideroblastic anemia?
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pyridoxine (b6) therapy
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What are some reversible etiologies of siderblastic anemia?
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alchohol, lead
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What lab results would be present in sideroblastic anemia?
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increased iron, normal TIBC, increased ferritin.
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What finding would be present on peripheral smear of sideroblastic anemia?
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ringed sideroblasts (with iron-laden mitochondria)
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What are the 2 dietary causes of megaloblastic anemia?
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Folate Deficiency, B12 deficiency
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What is megaloblastic anemia?
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Macrocytic anemia which results from inhibition of DNA synthesisin RBCs. It will have many large dysfunctional RBCs in the marrow and hypersegmented neutrophils.
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What lab findings would be present in megaloblastic folate deficiency anemia?
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Hypersegmented PMNs, glossitis, decreased folate, increased homocysteine, NORMAL methylmalonic acid.
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What lab findings would be present in megaloblastic b12 deficiency anemia?
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hypersegmented PMNs, glossitis, decreased B12, increased homocysteine, increased methylmalonic acid.
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What are several etiologies behind megaloblastic folate deficiency anemia?
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Malnutrition (alcoholics), malabsorption, imparied metabolism (methotrexate, trimethoprim), increased requirement (preggers, hemolytic anemia)
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What are several etiologies behind megaloblastic B12 deficiency?
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malnutrtion (alcoholics), malabsorption (crohn's), pernicious anemia, tapeworm
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In terms of symptoms, what is the most obvious difference between folate and b12 deficiency megaloblastic anemia?
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B12 has neuro findings
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What are 5 causes of non-megaloblastic macrocytic anemia?
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1) Liver disease
2) alcoholism (non-nutrition related) 3) Reticulocytosis, 4) Metabolic disorder (purine, pyrimidine synthesis) 5) Drugs (5-FU, AZT, hydroxyurea) |
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What is the cause of macrocytic anemia in general?
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Impaired DNA synthesis -> the nucleus is delayed in maturity relative to maturation of cytoplasm
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Name 3 types of nonhemolytic normocytic anemia
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ACD, aplastic anemia, kidney disease
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Name 6 types of intrinsic hemolytic normocytic anemia
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hereditary spherocytosis, G6PD deficiency, pyruvate kinase deficiency, sickle cell anemia, HbC defect, paroxysmal nocturnal hemoglobinuria,
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Name 3 types of extrinsic hemolytic normocytic anemias
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Autoimmune, microangiopathic (DIC, TTP-HUS), infections.
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Name 3 types of intravascular hemolytic anemias
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Paroxysmal nocturnal hemoglobunuria, autoimmune (cold agglutinins), mechanical destruction (aortic stenosis, prosthetic valve)
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Name 7 types of extravascular hemolysis
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Hereditary spherocytosis, G6PD deficiency, pyruvate kinase deficiency, sickle cell anemia, HbC defect, Autoimmune (warm agglutinins), microangiopathic (DIC,TTP-HUS)
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What's the pathogenesis of ACD?
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Inflammation causes increased hepcidin which decreases the release of iron from macrophages.
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What lab findings will be present in ACD?
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decreased iron and TIBC, increased ferritin
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What is ferritin?
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It is an iron storage protein. If there is increased amounts of it, then the body has increased iron stores (may appear as decreased serum iron because it is being hoarded by macrophages).
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If ACD goes on for a long time, how will its presentation change?
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It will no longer be a normocytic normochromic anemia. It will instead become a hypochromic microcytic anemia.
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What is the cause of Aplastic anemia?
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Failure or destruction of myeloid stem cells due to:
1) radiation and drugs 2) Viral agents 3) Fanconi's anemia 4) idiopathic |
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What are the pathologic features of aplastic anemia?
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pancytopenia characterized with severe anemia, neutropenia, and thrombocytopenia. Normal cell morphology, but hypocellular bone marrow with fatty infiltration.
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What are the symptoms of aplastic anemia?
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fatigue, malaise, pallor, purpura, mucosal bleeding, petechiae, infection.
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How do you treat aplastic anemia?
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Withdrawal of offending agent, immunosupressive regimens, allogenic bone marrow transplantation, RBC and platelet transfusion, G-CSF, GM-CSF
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How does kidney disease cause anemia?
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decreased EPO decreases hematopoiesis
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What kind of anemia would kidney disease cause?
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normocytic, normochromic, nonhemolytic.
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Name the 6 causes of intrinsic hemolytic normocytic anemia
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Hereditary spherocytosis, G6PD deficiency, Pyruvate kinase deficiency, Sickle Cell anemia, HbC defect, paroxysmal nocturnal hemoglobinuria
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In HS, what proteins are deficient?
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spectrin, ankyrin, band 3.1
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What is the pathogenesis of HS?
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less membrane causes small round RBCs with no central power (increased MCHC and RDW). These defects cause the spleen to remove the cells prematurely.
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What labs would you expect to be positive in HS?
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positive osmotic fragility test
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What findings would be present in an HS patient post-treatment?
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Howell-Jolly Body (basophilic nuclear remnants in RBCs)
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What's the pathophysiology for G6PD deficient patients?
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The lack of functional G6PD, which recycles glutathione, This leaves the cells susceptible to oxidant stress.
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When will G6PD patients experience symptoms
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After oxidative stressful events (sulfa drugs, infections, fava beans)
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What labs would you find on a G6PD patient?
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blood smear with Heinz bodies and bite cells
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What is the inheritance of G6PD?
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X-linked
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What kind of anemia would somebody with pyruvate kinase deficiency show?
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intrinsic hemolytic normocytic anemia
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What's the pathophysiology of pyruvate kinase deficiency? What is the genetic heritence?
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Defect in pyruvate kinase leads to decreased ATP which causes rigid RBCs. Autosomal recessive
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When will pyruvate kinase deficiency present?
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in newborns.
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What is the genetic inheritance of pyruvate kinase deficiency?
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Autosomal recessive
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What type of anemia is sickle cell anemia?
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intrinsic hemolytic normocytic
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When does sickle cell anemia manifest?
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After the newborn period (they have increased fetal hemoglobin which compensates for the shitty HbS)
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What precipitates sickling of HbS?
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deoxygenation (low O2) or dehydration
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What are potential complications in sickle cell patients?
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1) Aplastic crisis (stemming from parvovirus)
2) Autosplenectomy- >increased risk of infection with encapsulated organisms 3) Salmonella osteomyelitis 4) Painful crisis due to vasocclusion 5) Renal papullary necrosis (low o2) 6) splenic sequestration crisis |
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What's the treatment of sickle cell anemia?
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Hydroxyurea (increased HBF), bone marrow transplant
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What is HbC?
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a B-chain mutation (like sickle cell),, but this time a lysine is substituted for glutamic acid.
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What is the mutation in sickle cell anemia?
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Valine for glutamic acid on B-globulin gene
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Which is worse: HbC/HbSC (combined) or HbSS?
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HbSS is worse
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What is the pathogenesis of Paroxysmal nocturnal hemoglobunuria?
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intravascular hemolysis due to increased complement-mediated RBC lysis (impaired synthesis of GPI anchor/decay-accelerating factor in RBC membrane)
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What lab values will you find in paroxysmal nocturnal hemoglobinuria?
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increased hemosiderin
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What are the 3 categories of extrinsic hemolytic normocytic anemia?
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Autoimmune hemolytic anemia, microangiopathic anemia, Infections
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Autoimmune hemolytic anemia is considered to be an extrinsic hemolytic anemia because....
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factors outside of the blood cell lead to the anemia (compliment or whatever).
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SLE, CLL or cerain drugs such as alpha-methyldopa can cause what type of anemia?
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Warm-agglutinin (IgG) mediated autoimmune hemolytic anemia
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What antibodies mediate cold-agglutinin autoimmune hemolytic anemia? What triggers it?
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IgM, the cold (temperatures below 28-31)
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What kind of anemia is erythoblastosis fetalis?
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autoimmune hemolytic anemia
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What test can be used to detect autoimmune hemolytic anemias?
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Coombs
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How is a direct coombs test preformed?
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anti-Ig antibody added. Patient's RBCs will agglutinate if they are coated with Ig.
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How is an indirect coombs test preformed?
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Lab RBCs are mixed with patient's serum. If the serum contains the anti RBC surface Ig, the cells will agglutinate.
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What is the pathogenesis of microangiopathic anemia?
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RBCs are damaged as they pass through an obstructed or narrowed vessel lumin.
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What blood smear finding is indicitive of microangiopathic anemia?
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schistocytes (helmet cells).
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In addition to DIC, TTP-HUS, SLE and malignant hypertension, what can cause microangiopathic anemia?
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prosthetic heart valves and aortic stenosis.
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Which infections typically cause extrinsic hemolytic normocytic anemia?
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Babesia, malaria species.
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