Exam 1 Flashcards
granulocytes: what precursor and which cells?
myeloid
basophils, eosinophils, neutrophils, mast cells
problem with AIDS patients
low CD4 T cell numbers, can’t fight off infections
senescence
cells die of old age
immature neutrophils are sent out when and called?
when reserve runs out
bands
what do bands indicate
acute infection
where does blood cell development begin fetally?
embryonic yolk sac
where do adults have BM?
axial skeleton, proximal femur and humerus
Where are bone marrow Bx taken from?
iliac crest or sternum
diseases that stress marrow capacity
marrow fibrosis, myeloproliferative diseases, severe hemolytic anemia (thalassemia major)
what will BM do in response to infection?
increase neutrophil production
what will BM do in response to hemorrhage or hypoxia?
increase production of erythrocytes
what will happen if BM can’t keep up with the amount of RBCs needed?
release premature, reticulocytes
what is a blast?
precursor cell to white blood cells in the myeloid and lymphoid cell line
eosinophil characteristics
parasites and allergic rxns
have pink granules and C-shaped nucleus
basophil characteristics
allergic rxns (contain histamine), contain anticoagulants, extremely large granules that obscure the nucleus
neutrophil
3-5 lobe nucleus, 1st responder in infections
monocytes
large nucleus, large area of cytoplasm
underproduction of mature cells
aplastic anemia
overproduction of mature cells
myeloproliferative disorders
failed differentiation of mature cells with production of excessive numbers of immature forms
myelodysplasia, acute leukemia
what happens in acute leukemia?
WBC increases but they are premature and nonfunctional
an example of myeloproliferative disorder
COPD
- ruddy complexion
- O2 carrying capacity decreases making BM think that the body needs more cells
- blood becomes “sludgy”
define aplastic anemia
failure of hematopoietic stem cells to produce mature RBCs
pathogenesis of aplastic anemia
few stem cells present (those that are there can produce little amounts of normal RBCs) other stem cells either can’t produce healthy precursor cells or just slow down and don’t work
characteristics of AA
pancytopenia and hypocellular bone marrow
congenital causes of AA (mechanisms)
failure of DNA repair proteins or defect in maintenance of telomere lengths
Acquired AA; dose related
chemotx (wipes out good and bad) antibiotics such as chloramphenicol and trimethoprimsulfamethoxale
idiosyncratic drugs AA
not dose related; gold, chloramphenicol, NSAIDs, cimetidine, sulfonamides, anticonvulsants, antifungals, penicillamine,
toxins that cause AA
benzene, insecticides
viral infections AA
hepatitis, E-B virus, HIV
immune disease AA
host vs graft, hypogammagloburinemia
other causes of AA
radiation, pregnancy, paroxysmal nocturnal hemoglobinuria
causes of toxicity to BM in AA; how do they damage?
radiation, cancer drugs, chemicals, cytotoxic chemotherapy
damage proliferating stem cells and differentiating SCs by producing DNA damdage
hypothesis of cause of AA
host lymphocytes are responsible for destroying normal hematopoiesis
suggested b/c AA can result from viral and immunological disorders
our own Ags are triggering cytotoxic T cells to destroy stem cells
how does AA come on?
insidiously
symptoms of AA with erythocytopenia
weakness, fatigue, dyspnea, palpitations
symptoms of AA with leukocytopenia
recurrent infections
symptoms of AA with thromobocytopenia
gingival bleeding, nose bleeds (epistaxis), petechiae, purpura
what will bone marrow bx show in AA
confirm hypocellularity (only 5-15%) decreased progenitor cells, increased fat precursor cells morphologically normal but are nonfunctional
DDx of AA
myelodysplastic disease (see dysplastic hematopoietic cells), (acute leukemia see increased blasts)
AA treatment if mild
monitor
AA if severe and not treated
poor survival rate of 2-6 months if untreated. die from overwhelming infections, need antifungals and antivirals which have bad side effects
AA severe (but not severe AA) treatment
RBC/platelet transfusions
Severe AA reticulocyte count
> 1%
Severe AA; neutrophil, platelet counts, bone marrow cellularity
N < 500 n./microliter (<200 if very severe), P < 20,000 p./microliter, BM cell < 5%
treatment of severe AA
bone marrow transplant
what you need to get a BM transplant
HLA compatible donor
after BM transplant what do you need?
immunosuppressant drugs
antithymocyte globulin (ATC)
cyclosporin (t-cell inhibitor)
BM transplant success in ___% of patients with a ___ year survival rate of ___%
70-80%; 5 year; 90%
side effects of immunosuppression therapy
anaphylaxis, serum sickness (violently ill, can be fatal)
what diseases are AA pts at his for?
myelodysplasia, serum sickness, paroxysmal nocturnal hemoglobinuria
myeloproliferative disorders
leukocytosis, thrombocytosis, erythrocytosis, splenomegaly, bone marrow hypocellularity
pathogenesis of myeloproliferative disorders
occurs due to failure of stem cells to respond properly to feedback mechanism, system produces more and more RBCs
cause of polycythemia vera
increased blood cell mass (amount) due to defect in stem cell’s ability to stop producing RBCs
what is first identified in P.V.?
elevated Hgb and then increased RBCs
normal reasons for increased RBC production?
hypoxemia, anemia, hemolysis, acute blood loss
signs P.V
elevated red cell mass (sludges up blood because more cell mass, less liquid), splenomegaly (spleen is overworking)
thrombocytosis, leukocytosis, elevated leukocyte alkaline phosphatase, elevated serum B12, low serum EPO
adverse effects of PV?
increased risk of thromboembolic diseases
cerebral, coronary, mesenteric circulation
arterial and venous thrombosis
stokes, MI
symptoms of PV
headache, visual disturbances, mental disturbances, pruritus after bathing, CVA, TIA, MI, digital pain/emboli with paresthesia (emboli=clots, paresthesia=tingling, dusty digits)
hemorrhagic events-platelets aren’t working properly even tho increased amount
ruddy cyanosis
what will peripheral smear PV look like?
microcytic
bone marrow PV?
hypercellular
treatment for PV and risk factor
intermittent phlebotomy
thrombosis or splenomegaly
if phlebotomy doesn’t work for PV what is next? and side effect of this??
cytoreductive therapy with allopurinol
hyperuricemia
other treatment for PV
low dose chemo (chlorambucil, busulifan) radioactive phosphorus cytotoxic agents IFN-alpha aspirin
prognosis of PV
transformation to myelofibrosis or myeloid leukemia is 5-20% over 20 yrs
unique about RBC sturcture
malleable to fit thru small spaces
most CO2 stored as? secondarily?
carbonic acid, carried unbound by hgb
bound to globular part of hgb molecule
bohr effect
decrease in amount of oxygen associated with hgb in response to a lowered blood pH resulting from an increase in CO2 in the blood
examples of diseases with abnormal hgb
sickle cell
thalassemia
what is hemolytic anemia
more cells go to spleen for destruction than the BM produces
2 reasons for anemia
decreased production
increased hemolysis
MCV
size of the RBC
example of microcytic anemia
Fe deficiency
example of macrocytic
pernicious anemia
MCH
how much hgb is in each RBC; determined by color
MCHC
avg concentration of hgb in RBCs
% of RBC that the hgb makes up
hematocrit
cellular portion of the blood
when bleeding rapidly what occurs in the cardiovasculature
HR will go up to increase CO but with decreased BP
cardiac index
cardiac output per body mass
signs of low CI
murmur, conjunctiva, mucosa, nails are pale
fatigue, decreased exercise tolerance, dyspnea
reticulocyte count in instances where blood loss is occurring
increases
why does reticulocyte count increase
when RBC count decreases, stimulus sent to the kidney to release EPO to send to the BM to produce more RBCs; but RBCs can’t be made so reticulocytes are sent out
reticulocyte index
reticulocyte count x (pt HCT/normal HCT)
what do spherocytes indicate?
immune hemolysis
schistocytes indicate?
microangiopathic hemolysis
aniocytosis?
big range in size of RBC, large RDW
basophilic stippling
erythrocytes have small dots at periphery
signs of Fe deficiency anemia
hypo chromic and microcytic, target cells, elongated
what is iron incorporated into?
heme
what does alcohol abuse cause?
enzyme inhibition in heme synthesis
what does lead poisoning cause?
iron deficiency because lead binds to heme instead
what causes thalassemia?
failure of globin synthesis
how does iron absorption occur?
in the proximal small intestine; Fe binds to transferrin to be incorporated into RBC; rest is stored in other places with ferritin
other places Fe is stored
liver, spleen, bone marrow, muscle
TIBC in Fe def
total iron binding capacity
high because more spots are available
transferrin saturation Fe def
percentage of transferrin molecules saturated with iron
low in Fe deficiency
ferritin in Fe def, chronic disease and why
low in Fe def. because not a lot of iron, so liver stops producing
high in chronic disease because liver sends it out to remove iron so that bacteria don’t use it as a food source
bone marrow stores of Fe to confirm dx
Fe in BM? not Fe def
no FE in BM? confirms Fe def
treatment for Fe def
oral supplementation FeSO4 or Fe gluconate
if it doesn’t work sublingual supplementation
side effects of Fe supplementation
diarrhea, constipation, bloating, gas,
issues with Fe absorption indicated by?
dark stools
