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Peds Hematology
KYCOM Block 10
86
Medical
Graduate
09/19/2013

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Cards

Term
why do RBC look like they do on a peripherial smear
Definition
RBC have more Hb on the edges due to the biconcave disck so are lighter in the middle
Term
why do we do a peripherial smear (5)
Definition
confirm coulter printout values
RBC morphology
WBC differential and morphology
platelet morphology
immature precursor identification (have nuclei)
Term
RBC: primary function, what is the functional component
Definition
mediate exchange of respiratory gasses between lungs and tissues
intracellular Hb picks up oxygen
Term
what can affect binding of RBC to oxygen (3)
Definition
fever, acidosis, and 2,3-BPG decrease binding
Term
what stimulates release of erythropoetin
Definition
tissue hypoxia
Term
how much oxygen on a RBC is normally released to the tissues
Definition
25%
Term
what is a reticulocyte, where do you see them
Definition
RBC that is still loosing its nucleus, there a little bit in the circulation. when it raises it is a concern
Term
what changes Hb levels other than anemia (3)
Definition
age, sex, and race
make sure to check appropirate levels for the age
Term
what is the official diagnosis criteria
Definition
two standard deviations below the standard Hb level for the child - it is really a lab diagnosis
Term
describe the trends of Hb levels through out life
Definition
higher in the first two weeks then comes down to 11.5 then in the teens it jumps to 13.5-14
Term
what is the cause of aplastic / hypoplastic anemia, what is a good sign of this
Definition
decrease in marrow production of RBC
reticulocyte site would be lower than normal
Term
what is the cause of hemolytic anemia, what is a good sign of this
Definition
increased destruction of RBC peripherially
a sign is immature precursors in marrow (ineffective erythropoiesis)
Term
what is the cause of hemorrhagic anemia
Definition
nose bleed, GI bleed, trauma, etc
Term
what is the causes of hypochromic / microcytic anemia (4)
Definition
iron deficiency, thalassemia minor, lead poisoning, chronic infection
Term
what are some common general clinical signs of anemia (4)
Definition
fatigue, looks pale, pale conjunctiva, pale palmer creases
Term
other than Hb and reticulocites what are other signs on anemia (2)
Definition
MCV and MCHC
Term
what is the standard range of MCV for all kids
Definition
75-85 fl
Term
what is it called when the MCV is below the normal range
Definition
microcephic anemia
Term
what is it called when the MCV is above the normal range
Definition
macrocific anemia
Term
what is it called when the MCV is normal but a kid still has anemia
Definition
nonocific anemia
Term
how can you tell if anemia is caused by marrow hypoplasia
Definition
low reticulocyte count
Term
what does chromicity mean in RBC
Definition
amount of Hb
Term
how do you determine the MCV
Definition
(hematocrit x 10) / RBCmm^3
Term
how do you determine the MCHC
Definition
(Hb x 10) / hematocrit
Term
what is MCHC describing
Definition
level of Hb in RBC
Term
what is it called if you have low Hb in anemia
Definition
hypochromic anemia
Term
what is it called if you have normal Hb but still have anemia
Definition
nonochromic anemia
Term
what is the most common anemia
Definition
hypochromic microcific anemia (iron deficiency anemia usually)
Term
what are the three causes of iron deficiency anemia, how can ou figure out which
Definition
poor nutrition, hemorrhage, poor absorption

ask if the kid is getting milk fed, see if they have lots of diarrhea (poor absorption Fe causes diarrhea)
Term
when does iron deficiency anemia normally show up, why (2)
Definition
after 6 mo, usually 10-18 mo
milk decreases the Fe absorption so if mom feeds milk and not Fe fortified forumla you can bet a happy chubby anemia baby.

the iron stores that came from mom last until around this time
Term
what are Fe rich foods
Definition
meat, spinach, apples, rasins,
Term
what are 4 signs specific to Fe deficient anemia
Definition
low IQ, asymptomatic, irritability if severe, spooning of finger nails
Term
what is the AAP recommendation for time to check Hb level in a healthy child
Definition
9 mo
Term
what is the cause of thalassemia
Definition
abnormal Hb gene affects the a or B globin chain synthesis causing a or B thalassemia
Term
how is thalassemia minor diagnosed (7)
Definition
Hb <9g/dl
hypochromic very severe
mentzer index
serum ferritin
theraputic trial of Fe
TIBC
serum Fe
Term
thalassemia major treatment
Definition
transfusion every 6-8 weeks (severe anemia)
Term
mentzer index: how is it calculated, what does the number mean
Definition
MCV/(RBC/10^6)
>13.5 suggests Fe deficiency anemia
<11.5 suggests thallasemia minor
Term
how does a theraputic trial of Fe help diagnose anemia
Definition
if Fe deficient anemia they will get better. make sure to see the child back in 3 mo (it takes a while for the Hb to return to normal) or check the reticulocyte count it should go up
Term
how do you diagnose a vs B thalassemia
Definition
electrophoresis
FHb should go down around 3 mo so if they have it after that they have thalassemia
Term
signs of thalassemia major (6)
Definition
severe anemia
expansion of marrow - maxillary hyperplasa (flat bones try to help)
signs of chronic hypoxia
basophillic stippling
hepatosplenomeagly - spleen and liver try to help make RBC
Term
lead poisioning: 4 neurological complications
Definition
psychomotor slowing, seizures, encephalopathy, lower IQ
Term
what are the 3 GI symptoms of lead poisoning
Definition
abdominal pain, vomiting, constipation
Term
what is a late finding of lead poisoning, why
Definition
papilledema, due to increased ICP
Term
what is the cause of lead poisoning (5)
Definition
leaded gasoline, old paint, aersolized paint, PICA
Term
how is lead poisoning confirmed (3)
Definition
serum lead
lead lines on x-rays
some states mandate that all kids at 1 yo they need lead check
Term
what is the cut off for concern in serum lead levels
Definition
10 mcg
Term
macrocytic anemia: aka, two qualifiers
Definition
megaloblastic anemia
MCV > 100
Hb is low
Term
macrocytic anemia causes (5)
Definition
B12 deficienciey
folate deficiency: goat milk fed
medication: phenytoin (anticonvulsant)
congenital hypoplastic anemia
transient erythroblastopenia of childhood
Term
what are some microscopic signs of megaloblastic
Definition
hypersegmented polymorphic neutrophils
low Hb
MCV > 100
Term
what is the difference in the Hb, cellular antigens, reticulocytes, MCV, and RBC enzymes in congenital hypoplastic anemia and transient erythroblastopenia
Definition
CHA: increased FHb, low reticulocytes, antigen i, increased MCV, normal or high RBC enzymes

TEC: notmal FHb, normal reticulocytes, antigen I, normal MCV, low RBC enzymes
Term
what is the cause of congenital hypoplastic anemia, what is the treatment
Definition
switch from FHb to adult is broken. marrow aplasia
need marow transplant
Term
what is the cause of transient erythroblastopenia of childhood, what is the treatment
Definition
switch from FHb to adult is fine but the marrow becomes supressed by virus
might need a few bridge transfusions but dont need marrow transplant
Term
hereditary spherocytosis: inheritance, caue
Definition
autosomal dominant disease with abnormal RBC membrane due to deficiency of contractile spectrums making spherical RBC so the cells cannot squeeze through the sinusoids of the spleen and the RBC get lysed so they only liv 30-40 days
Term
what are some signs of spherocytosis (5)
Definition
hemolytic signs: marrow overdrive expands marrow sites, increased reticulocytes, RBC lysed in the spleen cause splenomeagly, jaundice due to hemolysis, urobillinogen in urine
Term
spherocytosis treatment
Definition
spleen removal
Term
why does hemolysis cause jaundice
Definition
bilirubin is released during lysis
Term
what are 5 causes of hemolytic anemia
Definition
sickle cell hemoglobinopathies
hereditary spherocytosis
G6PD deficiency
autoimmune hemolytic anemia
hemolytic disease caused by microangiopathy
Term
G6PD deficiency: cause, who gets it, what can trigger it
Definition
enzyme that prevents oxidation of RBC membrane is deficient

more in males

if exposed to medicies (anti-malarial, fava beans) can trigger hemolysis
Term
hemolytic disease caused by microangiopathy: cause
Definition
when cells go through the narrow capillaries they are destoried
Term
what are three chrisis that people with sickle cell get
Definition
sequesteration chrisis, vasoocclusive chrisis, aplastic chrisis
Term
what causes sequesteration chrisis
Definition
when RBC are exposed to hypoxia or acidosis the Hb crystalizes and causes sickling. the RBC get trapped in the capillaries (especially in liver and spleen) and cause hypovolemia because this trapped blood is not available for use
Term
what is vasoocclusive chrisis
Definition
active child gets hypoxia in phalanges or tores
Term
what is a aplastic chrisis
Definition
kid with sickle cell gets 5th disease and marrow is supressed due to virus on top of anemia making anemia worse
Term
what is part of a sickle cell that is characteristic and seen on a peripherial smear (3)
Definition
howell-jolly body, bluster cells (when Hb condenses), sickle cells
Term
signs of sickle cell
Definition
maxillary hyperplasia, dactylitis (hand foot syndrome, more common in toddlers), priapism (12h erectionin adolescent)
Term
what substances can cause hemolysis in someone with G6PD deficiency (4)
Definition
antimalarials, sulfa drugs, fava beans, antipyretics/analgesics
Term
malaria: cause, pathology, 3 symptoms
Definition
Plasmodia vivax lives intracellilarly and can be seen on smear
RBC are more fragile causing hemolysis, fever, chills, anemia
Term
what does it mean when band cells are seen
Definition
neutrophilia (shift left). mobilization of neutrophils to fight bacterial infection allows immature neutrophils into the blood.
if there is lots think sepsis
Term
what does lymphocyte elevation indicate
Definition
viral infection
Term
what does monocyte elevation indicate
Definition
infectous mono
Term
what does eosinophilia indicate
Definition
parasite infection, allergies (allergic rhinitis, bronchial asthma)
Term
what are the causes of thrombocytopenia (2)
Definition
destruction: ITP
consumption: DIC, massive hemangioma
production: TAR syndrome
Term
what is a coagulation disorder
Definition
hemophilia
Term
what is a normal platelet count, if it gets low what happens
Definition
1500,000 - 450, 000 mm^3

<50,000 get petechiae
<20,000 get purpura purple spots
Term
what is a petechiae and purpura
Definition
patechi are small
purpura are many patechiae and are larger
Term
what is wet purpura
Definition
purpura on the oral mucosa or retinah
Term
how does hemangomia cause low platelets, what disease
Definition
kasabach-merritt syndrome

lyming sticking platelets on walls of hemangioma causes low platelet in circulation
Term
what is the CC of someone with ITP
Definition
low grade fever that began a week ago, bluish rash
Term
ITP cause
Definition
viral particles attach to surface of platelets and are not recognized as ours in the spleen so it destories them
Term
what are some lab values of ITP (2)
Definition
WBC norma, platelets <20,000
Term
what is the treatment of ITP
Definition
not platelet transfusion unless life is iin danger because they get destoried too

corticosteroids supress immune reaction, gamma globulins coat sites in spleen and saturate them so it cannot grab platelets, give rh positive and coat the spots in the spleen with anti-b immunoglobulin
Term
what do you do if the ITP treatments dont work
Definition
suspect leukemia, get marrow aspirate
Term
how does a marrow aspirate distinguish between ITP and leukemia
Definition
ITP: overdrive like in hemolytic anemia
megakaryocytes are hypoplastic

leukemia: megakaryocytes decrease because lukemia cells take up all the room
Term
what is TAR syndrome
Definition
abnormalities of the radius due to hypoplastic anemia
Term
vitamin K deficiency complications
Definition
needed for production of clotting factors
increases bleeding
seen in chronic liver disease in adults
Term
vitamin K deficiency prophylaxis
Definition
within 24 hours kids get injection to avoid hemorrhagic disease of the newborn
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