Normocytic Anemia Flashcards
two different subtypes of anemia
normocytic w/ low reticulocyte count
normocytic wth high reticulocyte cout
anemias w/ low reticulocyte count
anemia of chronic disease
aplastic anemia
chronic kdney disease
hypoprolfeirative normocytic anemia
ACD is typically ___, but can also be ___
normochromic
normocytic
hypoproliferative
hypochromic
microcytic
ACD presents with
normal to inc ferritin (all Fe in MOs)
decreased to nm transferrin
inc sed rate
dec epo production and diirect inhibition of erythopoiesis in bone marrow
treatment ACD
underlying disorder
transfusions, epo –>if necessary
aplastic anemia
pancytopenia (w/ empty bone marrow)
- leukopenia (low WBC)
- thrombocytopenia (low platelets)
- anemia
primary causes aplastic anemia and prognosis
idiopathic, fanconi anemia
–poor
secondary aplastic anemia and prognosis
radiation, chemotherapy, alkylating agents
drug- chloramphenicol, bezene
viruses-epstein-barr, parvo
treatments of aplastic anemia include
G-CSF
GM-CSF
anemia of chronic kidney disease can be due to
- decreased epo (due to decreased renal cortical cells)
- blood loss
- shortened RBC survival
why do RBCs have shortened survival?
uremic RBCs are less deformable and more prone to mechanical destruction
treatment of anemia of chronic kidney disease
epo
4 types of normocytic anemia w/ increased reticulocyte countjjjj
hemolysis
membranopathies
enzymopathies
hemoglobinopathies
hemolysis
anemia due to destruction of RBC before usual 120 days lifespan
normal RBC lifecycle
survival of the fittest–>weaklings engulfed by macrophages that line splenic sinusoids–>hemoglobin broken down–>Fe recovered and stored in macrophage ferritin or transported back to marrow & heme ring metabolized to bilirubin, transported to liver, and excreted into bile
Haptoglobin and intravascular hemolysis
haptoglobin binds hemoglobin released into circulation–>complex removed by RES
–prevents Fe utilizing bacteria from benefiting from hemolysis
what does intravascular hemolysis have that extravascular hemolysis does not
hemoglobinemia
hemoglobinuria
blood-tinged plasma
blood-tined urine
seen in both intravascular hemolysis and extravascular hemolysis
decrease RBC
increase indirect bilirubin
increase haptoglobin, LDH
increase reticulocyte
spherocytes
RBC that have part of their membrane removed by RES and as a result lose the donut shape and have no central pallor
schistocytes
fragment of RBC
immune hemolytic anemias
caused by antibodies directed aganst RBC antigens
what do you use to distinguish immune hemo anemia
Coombs test
warm antibodies bind to red cells at ___ and produce ___
37 degrees
IgG
what are warm hemolytic anmias due to
1/3 lymphoproliferative diseases
1/3 CT diseases like lupus
1/3 idopathic
spleens role
destroys cells coated with IgG
treatment of warm antibodies
underlying disease
steroids
immunosuppression if those fail
splenectomy can be curative
formation of spherocyte
antibodies attach to RBC
Fc R on splenic macrophage binds to antibody
splenic macrophage pits antibody-antigen complex fragmenting RBC
damaged RBC reseals
cold anemia
IgM antibodies bind in cold areas (nose, ears, finger tips etc)–>pick up C3b–>lyse in circulation or in liver
Raynaud’s phenomenom
fingers turn white then red and painful on exposure to cold
cold anemia occurs most frequently after
viral and mycoplastic infection
three drugs that induce drug-induce hemoyltic anemia
penicilin
quinidine
methyldopa
how does penicilin cause it?
it is a hapten [[small molecule that can elicit an immune response only wen attached to larger carrer)
–ab targeted against RBC+drug–>complement removes RBC via spleen
Quinidine drug induced hemolytic anemia mech
innocent bystander- drug combines with Ab that happens to land on RBCj
methyldopa mech
drug incoporated into RBC membranej
microangiopathic hemolytic anemia & three types
non-immune intravascular hemolytic processes with prominent red cell fragmentation (schstocytes)
- TTP-HUS
- DIC
- malfunctioning heart valves
thrombotic thrombocytopenia purpura-HUS
thrombocytopenia and RBC fragmentation
TTP-HUS can be associated with
ADAMTS13 mutation, HIV, shiga toxin producing Ecoli infections
treatment for TTP HUS
plasmarpheresis
why do malfunctioning heart valves cause fragmentation?
turbulence and shearing forces from high pressure gradients
membranopathies
decreases in spectrin, ankyrin ,or cyto protein–> RBC fragility
types of membranopathies
hereditary spherocytosis
paroxysmal nocturnal hemoglobinuria
hereditary spherocytosis epi
congenital auto dominant
common in N europeans
problem wth hereditary spherocytosis
membrane defect so red cells processed by spleen form spears–>lyse–> inc bili, decreased haptoglobin, inc retculocytes
MCHC elevated
test for hereditary spherocytosis
osmotic fragility test (lysis of cells in hypotonic salt)
if need to cure hereditary spherocytosis
splenectomy
paroxysmal noctural hemoglobinuria is caused by
acquired (rather than inherited) intrinsic defect in cell membrane
defect in PIG-A gene–>missing membrane molecule GPI–>RBCS become vulnerable to complement medaited destruction
PNH is an example of
intravascular hemolysis
clinical signs PNH
dark urine
thrombocytopenia
anemia
DVT
diagnosis of PNH
flow cytometry
treatment of PNH
transfusion
anticomp drug Eculizumab, stem cell transplantation
example of enzymopathy
G6PD de
G6PD genetics
present on x chromosome
what does G6PD
helps keep hemoglobin molecule intact when faced wth oxidant stressed
2 syndromes associated with G6PD
hemolysis AFTER oxidant stress (drugs, infection ,diabetic ketoacidosis, eating fava beans)
chronic, on-going hemolysis w/o drug stress
-drugs that induce sulfonamides, acetysalicylc iacd, vt K, nitrofuranotoin, prmaqune, qunne, chlorampehnicol
reticulocytes and G6PD
may possess higher levels of enzyme, obscuring diagnosis
G6PD may cause
heinz bodies, bite cells
hemoglobinopathies
qualitative disorders of hemoglobin caused by mutated (rather than deleted) globin genes
example of hemogloinopathy
sickle cell disease
genetics of sickle cell disease
single AA sub on beta chain on chrom 16
if both chromosomes affected, sickle cell disease- if one affected, sickle cell disease
Hb in SSD
hemoglobin is poorly soluble when deoxygenated (Hemo S) and when polymerizes, RBCS form a sickle shape–>less deformable, vasooclussve plaques occur
why is SC trait adaptive?
R against malara
treatment SCD
propylaxis-hydration, oxygenation, warm temp, algesics–treat pain!
longterm treatment SCD
hydroxyurea–increases Hg F and decrease Hg S
stem cell transplant?
