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Terms in this set (211)
Image reviewing RBC destruction
What should be considered when 1) Establish the prescence of RBC Destruction
diagnosing a hemolytic state? What - indirect bilirubin (serum)
lab tests can be used in this process? - hemoglobin (serum/urine)
- haptoglobin (serum)
- hemosideron (urine)
- Schumm's test
- reticulocyte count
- RPI
2) Determine Cause of RBC destruction
- Coomb's Test (DAT)
- RBC morphology
,Which heriditary hemolytic anemia is Hereditary Spherocytosis
usually due to a deficiency in
spectrin? What does it cause to occur defect in action of spectrin w/ acrin (2.1), decrease in
in the cell? vertical interaction, causes membrane to start to pull
away from the cytoskeleton, leading to a round
shape
Which heriditary hemolytic anemia is Hereditary Spherocytosis
the most common heriditary
hemolytic anemia in Europe?
What are the clinical symptoms of Jaundice
Heriditary Spherocytosis? Anemia
Splemomegaly (enlarged spleen)
Which hereditary hemolytic anemia is Hereditary Spherocytosis
due to a vertical defect?
What are the laboratory findings Hemoglobin: normal to slight decrease
associated with Hereditary Retic > 8% (RPI > 2.5)
Spherocytosis? MCV: 77-87 fL
MCHC: > 36 g/dL
Increased indirect serum bilirubin
Increased urobilinogen
DAT negative
,What does the PBS and bone marrow PBS:
appear as in the case of hereditary - spherocytes
Spherocytosis? - polychromatic
- few NRBCs
Bone Marrow
- normoblastic erythroid hyperplasia
What is meant by intercorpuscular Intrinsic Defects
defects?
Defects in which the integrity of cell itself is effected,
inherent to the cell itself, RBC is not normal
structurally and /or functionally
What is meant by extracorpuscular Defects outside the cell, extrinsic defects
defects?
Defects in the structure components Pokilocytes
of RBCs lead to the formation of
what?
What is the purpose of the osmotic Measure of RBC's resistance to osmotic stress
fragility test? Explain how the test is
performed. 1) RBCs placed in varying concentrations of NaCl
2) Water rapidly enters cells and equilibrium is
achieved
3) RBCs swell and eventually lyse
*****Spherocytes can tolerate less swelling and lyse
at higher concentrations of NaCl (less water) due to
decreased surface area-to-volume ratio
, What are the four heriditary Common HE
elliptocytosis phenotypes? HE with infantile pokilocytosis
HPP (Hereditary pyropokilocytosis)
SAO (Southeastern Asian Ovalocytosis)
Describe the symptoms and Symptoms:
appearence on the peripheral blood Asymptomatic, Non-hemolytic
smear for Common HE.
PBS:
Elliptocytes
Describe the symptoms and Symptoms:
appearence on the peripheral blood Severe hemolysis up to 2 years of age
smear for HE with infantile
pokilocytosis? PBS:
Elliptocytes
Pokilocytes