Red Blood Cell Disorders II-Usera Flashcards
What are the five types of normocytic anemia?
- acute blood loss
- early iron deficiency and ACD
- aplastic anemia
- chronic renal failure
- malignancy
What are the intrinsic defect causes of normocytic anemia?
- membrane defect
- abnormal HB
- enzyme deficiency
What are the extrinsic defect causes of normocytic anemia?
trauma (e.g. aortic stenosis, prosthetic valves)
immune destruction
How come you get extravascular hemolysis?
RBC phagocytosis by splenic and hepatic macrophages due to IgG bound with or without complement, or abnormal shape (e.g. spherocytosis, sickling)
What are the laboratory findings of normoncytic anemia?
increase unconjugates bilirubin
increased serum LDH
How come you get intravascular hemolysis?
RBC phagocytosis by splenic and hepatic macrophages
-hemolysis occurs within blood vessels (enzyme deficiency G6PD, mechanical trauma, complement/immune destruction)
What are the lab findings in intravascular hemolysis?
- increase unconjugates bilirubin (minimal)
- increased serum LDH
- decreased serum haptoglobin
- hemosiderinuria
(Blank) is an acute phase reactant that complexes with Hb?
haptoglobin
Is herediatary spherocytosis autosomal dominant or recessive?
autosomal dominant
What causes hereditary spherocytosis?
intrinisic defect caused by mutation in ankyrin Howvere can have problems in Band 3, spectrins, protein 4.2
What are the clinical features of hereditary spherocytosis?
jaundice
gallstones
splenomegaly
aplastic crisis in children
What are the lab findings of hereditary spherocytosis?
normocytic anemia
increase MCHC
increase osmotic fragility (rupture in hypotonic solutions)
elevated LDH and Bili
Are all spherocytes due to genetic diseases?
no
What are 2 non genetic causes of sphereocytes?
warm autoimmune hemolytic anemia
ABO incompatibility
How do you treat spherocytosis?
splenectomy.
How come in spherocytosis, MCV may vary?
due to reticulocytosis
What is this: autosomal dominant defect in spectrin tetramers or 4.1 mild anemia splenomegaly
Hereditary ellipotcytosis
What can hereditary ellipotcytosis cause?
mild anemia
splenomegaly
What percent of your peripheral blood must be elliptocytes to be considered hereditary elliptocytosis?
greater than 25%
How do you get sickle cell anemia?
autosomal recessive missense mutation
What amino acid causes sickle cell?
valine substitution for glutamic acid in beta-globin chain
A lot of people (mainly A.A) are heterozygotes for sickle cell anemia but are not (blank)
anemic
If you have more (blank) HBS is threshold for sickle cell anemia
60%
(blank) concentrations prevent sickling in newborns for 5-6 months.
HbF