Exam 3 Flashcards
Transferrin
Absorbed iron is carried by this
Ferritin
Stores excess iron
Patho/mechanism of disease of iron deficiency anemia
Blood loss, reproduction and growth, inadequate iron intake
Symptoms of anemia’s
Fatigue
Tachycardia
Palpitations
Dyspnea or tachypnea on exertion
Pica
Common in iron deficiency patients
Craving for specific food, even if it doesn’t contain iron
Cheilitis
Irritation at corners of mouth
Seen in iron deficiency anemia
Glossitis
Smooth red tongue, common in anemia’s
Koilonychia
Spoon nails, common in iron deficiency anemia
Plummer Vinson syndrome
Dysphagia due to esophageal webs, seen only in severe disease of iron deficiency anemia
Lab eval for iron deficiency anemia
Microcytic, hypochromic
May see elevated RDW early in disease
Ferritin levels in iron deficiency anemia
Decreased
**will be falsely elevated in inflammation
B12 characteristics
Cobalamin Produced in microbes 100% needed from diet Stores well in tissues Takes years to deplete stores
B12 deficiency anemia causes
Nutritional deficiency
Inadequate absorption
Pernicious anemia
Large prevelance in patients 60+
Autoimmune destruction of gastric mucosa
Decreased instrinsive factor, causing severe b12 malabsorption
General cobalamin metabolism
Made into transcobalalmin, goes into cells, bings to MMcoA, donates methyl to DNA RNA, proteins, etc.
Why does b12 deficiency cause macrocytic anemia?
Accumulation of genetic material
B12 deficiency anemia signs and symptoms
Can cause leukopenia/thrombocytopenia
Glossitis, anorexia, diarrhea
**neuropathy! Stocking/glove distribution
B12 anemia lab testing
Low cobalamin
MMA and/or homocysteine levels elevated
Macrocytosis
Folic acid deficiency general characteristics
Neural tube defects (spina bifida) in pregnancy, congenital heart disease
Abundant in citrus and leafy greens
2-3 month store
Folic acid deficiency causes
Increased demand, dietary deficiency, drugs
Folic acid deficiency anemia signs and symptoms
Similar to b12, often simultaneous
**no neural abnormalities!!!
Lab testing for folic acid deficiency
Low serum folic acid
Cobalamin to rule out b12 deficiency
Thalassemias pathophysiology
Defective globin synthesis
Leads to microcytic, hypochromic anemia
Asian, black, Mediterranean patients
Alpha thalassemia pathophysiology
Gene deletion reduces alpha globin chain synthesis
Beta thalassemia pathophysiology
Point mutation, decrease or absent beta chain synthesis
How can you distinguish alpha and beta thalassemia?
Beta has basophilic stippling (also seen in lead poisoning)
Severe thalassemia, consider…
Splenectomy
Autoimmune hemolytic anemia
Autoantibodies form against RBC membrane causing hemolysis
IgG binds at body temp- autoimmune hemolytic anemia, warm auto Abs
IgM binds at cooler temps- cold agglutinin disease
Coombs test diagnoses…
Autoimmune hemolytic anemia
Sickle cell disease general characteristics
Autosomal recessive, amino acid substitution on beta globin chain
Hb S is unstable and prone to polymerization with other Hb S, leads to suckling
What are common triggers of sickling?
Hypoxemia and acidosis, deoxygenated Hb S
Heterozygous sickle cell…
Are typically hematologically normal
Homozygous sickle cell disease…
Causes the disease
Sickle cell disease diagnosis
Chronic hemolytic anemia
Sickle D cells visible on smear
Elevated acute phase reactants
Hemoglobin electrophoresis** confirmatory test
Sickle cell disease manifestations
Vaso-occlusive episodes/ crises
Acute chest syndrome- respiratory failure due to emboli, thrombosis, sometimes infection
Sickle cell and the spleen
Spleen is very active in removing old RBC and RBC fragments
Splenomegaly, splenic sequestration, can lead to hypocalcemia shock
Can cause auto splenectomy
Sickle cell disease traetment
Stem cell transplant for kids
**pain management
Hydroxyurea
Paroxysmal nocturnal hemoglobinuria General
Rare
Gene on X chromosome, but not X linked
Abnormal RBC vulnerability to complement (Coombs negative)
PNH symptoms/signs
Hemoglobinuria, jaundice, bone marrow suppression, increases at night
PNH labs
Flow cytometry, sorts and identifies cell types
**severe disease needs to be treated
Aplastic anemia
Injury or suppression of heme stem cell- marrow failure and inability to make mature blood cells
Hereditary Spherocytosis
RBC membrane protein defect, leads to hemolysis due to membrane fragility
Spherocytosis diagnosis
Osmotic fragility testing
Spherocytes in peripheral smear
G6PD deficiency
G6PD decreases oxidative stress, so a deficiency leads to denatured proteins
G6PD deficiency RBC presentation
Heinz bodies
RBC membrane damaged
Bite cells in peripheral smear
**enzyme assay confirms low G6PD
Anemia of chronic disease
Microcytic
Commonly from inflammation, organ failure, old age
Extrinsic pathway general
Triggered by tissue damage, started from exposure to tissue factor
Quick, small steps to form thrombin, positive feedback loop
Prothrombin time evaluates this pathway
Intrinsic pathway general
Trauma to blood or exposed collagen, less potent enough than extrinsic pathway
PTT evaluates this pathway
Plasminogen is activated to…
Plasmin, which does fibrin degredation
Platelet range indicative of platelet transfusion
<10-20 k
Petechiae
No blanching, erythematous or violaceous lesion
<3 mm
Purpura
3-10 mm
Ecchymosis
> 10 mm
Hemorrhagic bullae
Fluid filled lesion >10 mm
Vasculitis, necrotizing infections, etc
Thrombocytopenia in pregnancy
Often due to expanded plasma volume, HELLP
Hemolysis, elevated liver enzymes, low platelets
Pseudo thrombocytopenia
Platelet clumping can occur in lab testing, but citrated platelet count can verify true vs. pseudo thrombocytopenia
Immune Thrombocytopenia general
Dz of young women
Circulating antiplatelet autoantibodies, insufficient platelet production
Thrombopoietin levels low
Immune thrombocytopenia manifestations
Some asymptomatic
Mucocutaneous bleeds, mild nosebleeds
Some severe bleeds