Normocytic-Normochromic Anemias Flashcards
What is the most common cause of aplastic anemia?
Idiopathic (unknown)
What are the four secondary causes for aplastic anemia?
Chemicals, Drugs (chloramphenical), Radiation, and infections (Chronic)
What is the most common congenital disorder associated with aplastic anemia?
Fanconi’s anemia
What is the BM cellularity of aplastic anemia?
Hypocellular
What is the CBC results of someone with aplastic anemia?
Pancytopenia Decreased in HGB and HCT, Normocytic normochromic.
What is the RBC morphology of someone with aplastic anemia?
No characteristic; few present.
What is the retic count for someone with aplastic anemia?
Decreased to absent.
What is the treatment for someone with aplastic anemia?
Take away offending agent, support therapy, immunosuppressive therapy. Cure = BM transplant
What type of Poik is in every hemoglobinapathy?
Targets
What is the amino acid substitution for someone with sickle cell?
Valine for glutamic acid
What are the 3 factors contributing to sickling process?
Hypoxia, acidosis (CO2) and Dehydration
What is the cause for painful crisis in SC?
Infection, fever, dehydration, exposure to extreme cold (tissue damage)
What are the causes for acute chest syndrome in SC?
Infarction of lungs
What are the causes for high risk of infection in SC?
Tissue damage in spleen
What is the difference from SC disease and SC trait in regards to inheritance?
SCD: inherited two sickle genes, one from each parent.
SCT: Inherited one normal gene from one parent and one sickle gene from the other
What is the difference from SC disease and SC trait in regards to hemoglobin nomenclature?
AS = Trait SS = Disease
What is the difference from SC disease and SC trait in regards to solubility results?
AS and SS remain turbid and are insoluble. only AA is soluble and is thus clear
What is the difference from SC disease and SC trait in regards to hemoglobin electrophoresis results?
AS: two bands A>S
SS: S>F (no A)
What is the difference from SC disease and SC trait in regards to RBC morphology?
SS: Targets, sickles, schistocytes, sphereocytes
AS: Slight targets, usually no sickles unless in crisis
What is the difference from SC disease and SC trait in regards to treatment?
SS: Hydration, Morphine, antibiotics, blood transfusions
AS: No treatment
What is the principle of the sickledex solubility test?
Reduction; S- HGB is insoluble, A is soluble
What is the reagent for the sickledex solubility test?
Sodium dithionite or sodium metabisulfite
What are the causes for false positives in the sickledex solubility test?
Protienemia, >18g/dL, other sickling HGBs
What are the causes for false negatives in the sickledex solubility test?
Newborn, HGB
What is the amino acid substitution for someone with hemoglobin C disease?
Lysine for glutamic acid
What is the difference from Hemoglobin C disease and Hemoglobin C trait in regards to clinical presentation?
CC: mildly hemolytic; splenomegaly
AC: Asymptomatic
What is the difference from Hemoglobin C disease and Hemoglobin C trait in regards to hemoglobin nomenclature?
CC = disease AC = trait
What is the difference from Hemoglobin C disease and Hemoglobin C trait in regards to hemoglobin electrophoresis?
CC = 100% C, (no A) AC = A>C
What is the difference from Hemoglobin C disease and Hemoglobin C trait in regards to RBC morphology?
CC: Targets +, Crystals, polychromes, (increase in retic)
What is the inheritance for SC disease?
Lysine sub from one parent, Valine sub from the other
What is the clinical presentation for SC disease?
Less sever than sickle but severe hemolytic anemia with painful crisis
What is the electrophoresis result for someone with SC disease?
S = C
What is the RBC morphology for someone with SC disease?
Tartget +, C-crystals, S-C Crystals, polychromasia, H-J, Pappenheimer, nRBC
Review worksheet 7 for electrophoretic patter in cord blood, CC, SC, AC, AS, and SS
Review!!
What is the inheritance for someone with sickle cell-Beta thal?
Valine suc from one and B^o or B^+ from the other
What are the clinical presentations for someone with sickle cell-Beta thal major vs. minor?
Major: severe hemolytic anemia
Minor: Mild to moderate
What are the hemoglobin electrophoresis results for someone with sickle cell-Beta thal major vs. minor?
Major: S>F (^)> A2 (^) (no A)
Minor: S>A>F (^)>A2
What is the RBC morphology for someone with sickle cell-Beta thal major vs. minor?
Targets +, sickles, nRBC, hemolytic poik
Sickles may be absent in minor
What are the two ways that Hemoglobin D disease is differentiated from Hemoglobin S?
Solubility and Citrate electrophoresis
What is the worlds third most common abnormal hemoglobin and where does it occur?
Hemoglobin E disease, SE Asia`
What is the mechanism for hereditary sphereocytosis?
Decrease in spectrin = Increase permeability of Na+ into cell
What is the mechanism for hereditary elliptocytosis?
Decrease in cholesterol causing HGB to polarize to opposite sides of cell
What is the mechanism for hereditary stomatocytosis?
Defect in Na+/K+ pump
What is the clinical presentation for hereditary sphereocytosis?
Anemia, jaundice and splenomegaly
What is the RBC indices for hereditary sphereocytosis?
HGB ~ 12g/dL
MCHC = 36-38%
What is the RBC morphology for hereditary sphereocytosis?
Variable number of spheres, polychromasia, increased retic
What is the principle for the osmotic fragility test?
Blood to series of hypotonic salt, hemolysis observed
Conditions that show increased osmotic fragility?
Sphereocytosis
Conditions that show decreased osmotic fragility?
Thals, sickles, hypochromia
Conditions that show increased resistance to hemolysis?
Thals, sickles, hypochromia
Conditions that show decreased resistance to hemolysis?
Sphereocytosis
What is the NaCl [ ] when hemolysis should begin?
0.45-.50% NaCl
What is the NaCl [ ] when hemolysis should be completed?
0.3-0.35% NaCl
What type of pork demonstrate the greater resistance to hemolysis?
Targets (hypochromia and sickles)
In someone with any hemolytic anemia, what are the expected results for plasma hemoglobin?
Decreased
In someone with any hemolytic anemia, what are the expected results for retics?
Increased
In someone with any hemolytic anemia, what are the expected results for serum bilirubin?
Increased
What is the deficient enzyme in G-6-PD?
Reduced Glutathione
What is the deficient enzyme in PK deficiency?
Pyruvate Kinase, = Burr
What is the deficient enzyme in methemoglobin reductase?
Methemoglobin (ferric) Cyanotic
What is the triggering factors in G-6-PD?
New drug, Infection, Feva beans, ingestion of moth balls
What is the etiology of PNH?
RBCs are more sensitive than normal to lytic action of complement.
What is the Clinical presentation of PNH?
Sleep induced hemolytic action, bloody first urine
What is the CBC for PNH?
Panocytopenia
What is the RBC morphology for PNH?
None
What test is done to confirm PNH and what is its result?
Positive HAMS test
What is the principles of Hams test?
Complement to alternative pathway = lysis of PNH cells
What are the three conditions that may cause illumine hemolytic anemia?
Transfusions, Pregnancy and organ transplant
What are the differences between autoimmune and alloimmune hemolytic anemia?
Auto = self Allo = antibodies one one react with antigens of another
What is the etiology of cold agglutination?
??????? Clumping of RBC cut to IgM antibody
What is the CBC results of Cold agglutination?
Break rule of 3 = high HCV
What antibody is associated with Paroxysmal cold hemoglobinuria?
Donath-lansteiner antibody
What are the two disorders associated with MAHA?
hemolytic uremic syndrome and Thrombotic thrombocytopenic purpura
What is the predominate poik in MAHA?
Schistocytes
