RBC Part 3 Flashcards
hemolytic anemias are due to what
increased rate of RBC destruction
premature RBC destruction
Acute hemolytic anemias present with what
fatigue fever pallor jaundice low back pain splenomegaly congestive heart failure
chronic hemolytic anemias present with what
same as acute plus
gallstones
abnormal bone growth
fractures
in hemolytic anemias what lab findings
increased unconjugated bilirubin
decreased serum hepatoglobin conc.
increased LDH
what is seen on a peripheral blood smear of hemolytic anemias
spherocytes
schistocytes
reticulocytosis
polychromasia
intravasular hemolysis leads to what
hemoglobinemia
methemoglobinemia
hemoglobinuria
hemosiderinuria
extravascular hemolysis usually occurs where
spleen, liver, bone marrow macrophages
extravascular hemolysis usually occurs in association with what
intrinsic/inherited hemolytic anemias hemolytic (defects in hemoglobins, RBC membrane, RBC enzyme)
what lab results would one expect from extravascular hemolysis
spherocytes in peripheral blood
increase unconjugated bilirubin
normal to slightly decrease haptoglobin
intravascular hemolysis is characterized by an increase in what
free hemoglobins
intravascular hemolysis occurs in association with what usually
extrinsic/acquired hemolytic anemias (immune hemolytic, microangiopathic anemias, infection, burns, hypersplenism
excess free hemoglobin in intravascular hemolysis leads to what
hemogloinuria
hemosiderinuria
decreased serum haptoglobin conc.
intrinsic hemolytic anemias can be due to what
defects in hemoglobin
defects in RBC membrane
defects in RBC enzymes
thalassemia are defined as what
reduced production of one or more globulin chain
beta thalassemia is common in what type of people
mediterranean origin
alpha thalassemia is common in what type of people
southeast asia or china
what is beta+ and beta 0
abnormal beta genes
beta + produces reduced beta-globin
beta 0 produces no beta globin at all
beta+ and beta 0 are seen in what
beta thalassemia major
beta-globin chain is on what chromosome
11
beta thalassemias are usually do to what
point mutations or partial deletions
in beta thalassemia major what alleles are affected
both alleles (B+/B+ or B0/B+ or B0/B0)
beta thalasseia majors onset is when
6-9 months after birth when switch from fetal hemoglobin occurs
what compensatory changes occur in beta thalassemia
distorted facies (chipmunk face)
abnormal bone growth
hepatosplenomegaly
damage to organs
chipmunk face and crew cut on X-ray are seen in what
beta-thalassemia major
patients with beta-thalassemia major are dependent on what for survival
transfusions
what is seen in peripheral blood of beta thalassemia major
microcytosis hypochromia anisopoikilocytosis target cells teardrop cells nucleated RBCs
what is seen on Hb electrophoresis or HPLC for beta thalassemia minor and major
minor- HbA is decreased, HbA2 and HbF are increased
major- no HbA and increased HbA2 and HbF
in beta thalassemia minor the alleles are?
one normal one abnormal gene (B/B+ or B/B0)
iron therapy does what to beta-thalassemia minor
worsens it
iron levels in beta thalassemia minor are what
increased or normal in serum and bone
what type of cells are seen in a peripheral smear of beta thalassemia minor
tear drop and target cells
alpha thalassemias are due to what
complete deletions of genes
the loss of all 4 alpha genes results in what
HbBarts- tetrameric gamma chains
no HbA, HbF or HbA2 at all
hydrops fetalis
loss of 3 alpha genes results in what
HgH- tetrameric beta chains
what is seen in HbH on peripheral smear (pre and post splenectomy)
post- heinz bodies
pre- teardrop cells and bite cells
what is seen on a chord blood smear in HbBarts
erythroblasts
polychromatophils
burr cells
loss of two alpha genes is called what
alpha thalassemia trait
altha-thal-1 is what
cis deletion of alpha genes
HbH inclusions in peripheral blood during life
HbBart (gamma4) present at birth
alpha-thal-2 is what
trans deletion of alpha genes
HbBart (gamma4) present at birth
no HbH inclusions
how is alpha-thal-2 confirmed
gene mapping
extrinsic hemolytic anemias are due predominately to what type of hemolysis
intravascular hemolysis
intrinsic hemolytic anemias are due predominately to what type of hemolysis
extravascular hemolysis
intrinsic hemolytic anemias can be due to what
defects in hemoglobin
defects in RBC membrane
defects in RBC enzymes
defects in hemoglobin include what
quantitative- thalassemias
qualitative- sickle cell, HbC
defects in RBC membrane include
hereditary spherocytosis
defects in RBC enzymes include
G6PD deficiency
sickled cells lead to what
increased viscosity of blood
obstruction in microcirculation
infarction (tissue death)
what occurs in sickle cell to the cytoskeleton
cytoskeletal damage leads to spectrin now dimers instead of normal tetramers
the life span of sickled cells is what
20 days
what is the best example of hemoglobinopathy
sickle cell disease
hemoglobinopathy (define)
structurally abnormal hemoglobin is produced
HbS is made how
valine replaces glutamic acid on beta globin chain
alpha globin genes are on what chromosome
16
sickle cell anemia patients have what medical issues
chronic hemolysis and vaso-occlusive crises
chronic leg ulcers
autosplenectomy
extramedullary hematopoiesis
salmonella osteomyelitis is associated with what
sickle cell
chronic leg ulcers and autosplenectomy are associated with what
sickle cell
what vast-occlusive crises occur in sickle cell anemia
musculoskeletal painful crises
hand and foot syndrome (dactylics)
avascular neurosis of femoral head
avascular necrosis of femoral head is seen in what
sickle cell
dactylitis is what and seen in what
swollen fingers
seen in sickle cell anemia
acute chest syndrome is a complication of what
sickle cell anemia