hematology (2) Flashcards
what does blood arise from
bone marrow
what system regulates (direct or indirect) all other body functions
hematologic
what are the 2 major differences of bones in pediatrics compared to adults?
- all bones have red bone marrow
- all bones are capable of hematopoiesis
at what age range does bone growth cease?
adolescents
which bones contain red marrow (produce blood cells) in adolescents
flat bones (sternum, vertebrae, pelvis)
describe RBCs, WBCs, and platelet levels in neonate vs. older
RBCs and WBCs are higher in infancy, platelets are lower (all clotting factors are lower)
what is the most common anemia and nutritional deficiency
iron deficiency (anemia)
when are children screened for iron deficiency anemia?
9-12 mo
Case study:
3 y.o. is pale, lethargic, irritable. Always seems tired, can’t concentrate, ice craving. Assessment revelas 145 HR (tachy)
iron deficiency anemia
s/sx of iron deficiency anemia
pale, lethargy, irritable, tired, dec. LOC, dec. concentration, ice craving
what tests would be ordered for possible iron deficiency anemia?
H&H, CBC
what would a CBC show with iron deficiency anemia?
- inc. RBCs
- mean corpuscualr volume (MCV)
- mena corpuscular hemoglobin (MCH)
- inc. reticulocytes
what do cells with anemia look like under observation
pale, “bleached out”
what are the hct and hgb criteria to supplement iron in the diet
hct= <34%
hgb= < 11.3 g/dl
what is the dose of ferrous sulfate used to orally supplement?
3-6 mg/kg/day
what are the 3 causes of iron deficiency anemia?
inadequate diet intake
malabsorption in GI
chronic blood loss
what can lead to iron defiency anemia from chronic blood loss?
- cows milk protein allergy
- crohns
- celiac disease
- inc. physiologic demand (growth)
- menorrhage (period)
what should iron be given on (stomach, fluid)
empty stomach with OJ (any juice w/ vit. c)
what is an inherited autosomal recessive genetic disease that affects RBCs
sickle cell
in sickle cell, what is hemoglobin replaced or partially replaced with?
hemoglobin S
are cells chronicly or acutely sickle shaoed with the disease?
acute (crisis)
why do sickled cells occlude vessels
“sticky”
what is the first organ affected by sickle cell?
spleen
which races are affected by sickle cell
african, hispanic, middle eastern, southern european, asian indian
wha ttriggers cause sickling of cells
- infection/fever
- dehydrtion
- cold/high altitude
- overexertion
- alcohol
- stress, fatigue
(amnything that dec. oxygen)
to get sickle cell, the child must receive the gene from how many parents?
both
if both parents have the sickle cell gene, what are the chances of having, carrying, and not being effected?
25% having
50% carrying
25% not effected
what is the treatment of vaso-occlusive sickle cell crisis
- hydration (IV)
- aggressive pain management
- oxygen
- bed rest
how does hydration help sickle cell crisis
reduces dehydration (trigger)
decreases blood viscosity
is pain management suring a sickle cell crisis scheduled or PRN
scheduled
which immunizations can help sickle cell
- pneumococcal
- Hib
- hep b
- influenza
- 23-valent vaccine @ 2 and 5 yrs. after forst dose
what is the prophylactic antibiotic for sickle cell
low doses of penicillin
what ages is prophylactic penicillin used in sickle cell?
newborn - 5 y.o. (unless they ahd splenectomy)
what is the prophylactic dose of penicillin for sickle cell for ages newborn-3 y.o.
125 mg 2x/day
what is the prophylactic dose of penicillin for sickle cell for ages greater than 3 y.o.
250 mg 2x/day
which testing is done annualy for sickle cell
transcranial doppler testing
what haooens if transcranial doppler testing for sickle cell comes back abnormal?
prophylactic blood transfusion
which treatment of sickle cell improves blood and tissue oxygenation, reduces sickling, temporary suppression of RBCs with hgb S
transfusion
complication of blood transfusion for sickle cella and how to help
iron overload, use iron-chelating drugs
examples of iron chelating drugs
deferoxamine, deferasirox, with vitamin c
which sickle cell treatment is a cytotoxic drug that decreases production of abnormal blood cells (less pain)
hydroxyures
what is needed for a hematopoietic stem cell transplant for sickle cell?
human leukocyte antigen (HLA) compatible sibling
group of hereditary blleding disorders resulting from deficiency in specific clotting factors
hemophilia
what are the types of hemophilia
A,B, and C
which hemophilia is classic hemophilia
hemophilia A
which hemophilia is christmas disease
B
what is the most common hereditary bleeding disorder?
von willebrand disease
which hemophilia is caused by a deficiency of factor VIII (8)
hemophilia A
what gender does hemophilia A effect?
Males, X linked recessive
why does hemophilia A only happen in men?
x-linked recessive trait
which hemophilia is a deficiency of factor IX (9)
hemophilia B
what does the severity of hemophilia B range from?
mild to severe bleeding tendencies
what kind of genetic trait is hemophilia b
x-linked recessive trait (mostly male)
which population is most effected by hemophilia b
amish
which hemophilia is a deficiency of factor XI (11)?
hemophilia c
what kind of genetic trait is hemophilia c
autosomal recessive
what gender does hemophilia c primarily effect?
equally in males and females
how is bleeding in hemophilia c compoared to a and b
less bleeding in C than A and B
what is vWF (von willebrand factor)
plasma protein (usually increases in injury)
which blood disorder is poor platelet aggregation
von willebrand disease
what kind of genetic trait is von willebrand disease
autosomal dominant
what gender does von willebrand disease primarily effect?
M and F equally
what is von willebrand disease treated with?
DDAVP
what kind of cases is von willebrand disease usually seen in?
mouth injury, bloody nose, excessive menstrual bleeding
which hemophilias are sex-linked recessive clotting disorder
A and B
hemophilia A and B is carried by the _____ and passed to the ____
mother, sons
what are complications from hemophilia?
4 of them
- internal hemorrhage
- transfusion reaction
- hypovolemic shock
- death
what can not be given for pain in hemophilia?
aspirin/ibuprofen (thins blood)
what diagnostic tests are used to look for hemophilia?
3 of them
- chorionic villus sampling
- amniocentesis
- genetic testing (carriers)
what are the 3 treatments for hemophilia
- replacement of missing factor
- home infusions
- gene therapy (exploring it)
when is replacement of factors especially indicated in hemophilia?
when a child experiences mild to severe hemorrhage
why would factor replacement be scheduled for a hemophilia patient?
episodes can be controlled or avoided (earlier started, better outcome)
non-pharm method to help acute bleeding
RICE
rest, ice, compression, elevate
describe injections in hemophilia patients
- give sub cue if possible
- if not possible to avoid IM, administer replacement factor first
what kinds of replacement factors are used for hemophilia a or b
- pooled factor from blood products
- recombinant factor
- desmopressin acetate (DDAVP)
which replacement factor is made ina lab and does not contain blood?
recombinant factor
which repalcement factor increases factor VIII but does not effect factor IX
desmopressin acetate (DDAVP)
what is DDAVP used for
mild hemophilia A
what are the 3 signs of blood transfusion reaction
fever, chills, irritability
what fluid is blood given with fro infusions
NS, not D5W
what to do if child has transfusion reaction
turn off, disconnect, flush with NS and heparin (heparin only if central line)
