Anemias caused by Bld loss + RBC lysis (Pt2) Flashcards
Describe Acute Blood loss
Acute: pt rbc’s may not be indicative of loss, plasma is lost at same rate as blood/no time to respond by retaining fluid
Describe Chronic Blood loss
has decreased HCT/rbc counts with fluid retention
Describe Relative Anemia
How does pt appear?
Total ability?
What is decreased?
Causes:
pt appears anemic with an increased plasma
total RBC mass/O2 carrying ability uneffected
HCT/HGB decreased
Causes: pregnancy/hyperproteinemia/Massive IV/infusions
Indicators of RBC destruction? (hemolysis)
Increased bilirubin/LDH
Increased Iron/Hemosiderin (and urine)
Dec haptoglobin
schistocytes
Indicators of RBC production?
erythrohyperplasia
increased RPI
COOMBS DAT test
increased destruction/production
what is a Positive
What is a negative?
+ —> Ab mediated, immune hemolytic anemia
- —> peripheral blood smear
Intrinsic abnormalities causing rbc destruction
non-immune
What are the 4 groups of rbc membrane defects
Hereditary Spherocytosis (HS)
Hereditary Elliptocytosis (HE) (HPP/SE/SOA)
Hered. Stomatocytosis
Hereditary Xerocytosis
What is the vertical spectrin ankryn defect anemia?
What is the horizontal defects?
Vertical: HS
Horizontal: HE/HPP
Describe Hereditary Spherocytosis (HS)
RBC membrane
hemolysis ranges?
vertical spectrin ank. defect
Rbc’s lose membrane/low surface area/vol ratio/hemolyzed in spleen
Silent carrier to severe
What is the Presentation for HS?
Lab findings?
Hemolysis
DAT
Folic acid
MCV/MCHC
Retics/RDW
Presentations - jaundice, splenomegaly, anemia
Splenectomy resolves symptoms but defects persists
Lab: Extravascular Hemolysis
DAT -
Folic acid decreased (looks megaloblastic)
MCV dec
MCHC increased
Retics increased
RDW increased
Describe Osmotic Fragility
rbc placed in HYPOtonic solution, water drawn intracellular by osmosis
Rbc becomes more spherical/lyse (release HgB)
Spherocytes = increased fragility
T/F by-concave cells can take on more water
true
What is the normal rbc fragility
normal rbc lyse at .45% complete at 100%
T/F: use heprin tubes to dilute your sample (saline to water)
true
What is the calculation for % hemolysis
O.D sample/O.D of water x 100
What is the hereditary spherocytosis % hemolysis
.65 close to isotonic solution
Describe Hered. Elliptocytosis (HE)
defects in what
(micro….)
diff types linked?
alpha/beta chain spectrin ank defect, effects rbc deformability, failure to return to normal disc shape (microvasculature)
severity varies
DIFF TYPES LINKED TO DIFF BLOOD GROUPS
Lab findings of HE
elliptocytes
MCV norm to high
MCHC norm
Osmotic fragillity norm
some rbcs might be heat sensitive
What is Hereditary spherocytic HE
rare hybrid of HE/HS
Describe Southeast Asian ovalocytosis (SAO)
south asia/cape pop of south africa (against malaria)
spoon shaped ovalocytes/transverse bar avoids central pallor
treat w splenomectomy
Describe Pyropoikilocytosis
What kind of spectrin defects
what mutation
subtype of
thermal instability
what are most pts?
alpha/beta spectrin mutation/decrease synth of alpha spectrin
Subtype of HE/severe hem. anemia
Thermal Instability - heated to 45deg - fragment (norm at 49deg)
most pts are black
(face abnorm/gallbladder/growth retard)
Lab findings of Pyropoikilocytosis
MCV
MCHC
rbc shapes?
Bizzare?
MCV dec
MCHC incr
Elliptocytes
Spherocytes
Bizzare micro poik- rbc budding/fragments
Describe hereditary stomatocytosis
most pt def. in what
failure of
increased?
most pts deficient in stomatin
-membrane protein (theorized to regulate channel)
Failure of NaK pump
increased H2O in cells
Lab findings of Hereditary stomatocytosis
MCV
MCHC
cells
Increased?
What decreased?
MCV increased
MCHC decreased
stomatocytes
Increased osmotic fragility
2,3 BPG decreased (inc affinity for O2)
Describe Hereditary Xerocytosis
cells defect in what
what do cells look like?
Lab findings?
MCV
MCHC
Types of cells
2,3 BPG?
Osm fragility?
cells lose K+ due to unknown rbc perm. defect
cells are dehydrated
“Puddle cells”
MCV increased (macro)
MCHC inc
Stomatocytes
target cells
2,3 BPG dec (less aff for O2)
dec Osmotic fragility
Describe Acanthocytosis
abnormal what
shapes?
Causes?
A
L
M
C
V
abnormal membrane lipids - inc cholesterol
shape defect not present in young rbcs
phospholipids
Causes: Abetalipoprotenima (Hereditary)
liver disease
malnutrition
certain bld groups
Vit E def
Describe Anemia of liver disease
Lab findings?
cholesterol/lipids increased
Lab:
target cells
macro/ROUND
acanthocytes
hemolysis
Anemia of Alcholism
hemolytic/megaloblastic anemias
megaloblastic folate defect (poor diet/ethanol/folate)
Ethanol - toxic effect on rbc precursors, cellularity/morph
