hematology

aplastic & cooley anemia

100

types of cells that are decreased due to complete bone marrow suppression. what will red bone marrow replace itself with?

1. RBCs, WBCs and platelets will decrease (all of them)

2. yellow bone marrow (fatty)

200

in cooley anemia, name the type of heme that is deficient

beta

300

name the result from beta thalassemia & what manifests?

lysing of RBCs

enlarged forehead & cheekbones from facial bones being under chronic stress, hepatosplenomegaly, and growth deceleration

400

what type of bone marrow is replaced in aplastic anemia?

yellow bone marrow replaces red

500

name the initial treatment for severe anemia, explain why chelation therapy is necessary for beta thalassemia and #1 nursing goal (3 answers)

1. they will receive blood transfusions

2. a side effect of transfusions is hemochromatosis (excess iron in blood) so chelating agent is needed to excrete excess iron = which can cause diabetes, heart, problems & liver disease

3. compliance

hemophelia

100

name the 2 traditional types of hemophilia and their factor

A (factor 8) and B (factor 9)

200

explain how symptoms may vary

depends on the amount of factor made

300

name the lab finding and precautions with hemophilia

•  prolonged PTT, normal PT

•  do not take meds that impair clotting abilities (NSAIDS/ibuprofen), no contact sports (not even track = hard on the joints), but low-impact instead (swimming), as a nurse, be prepared when performing skills like starting an IV = hold pressure longer

400

describe the patho of hemophilia

based on a complex “clotting cascade”, different clotting factors react to some stimuli, when it is activated, it will lead to the activation of Factor I, which is fibrinogen → fibrin (basically it impairs the ability to clot and leads to excessive bleeding even from minor injuries)

500

treatment!! (2)

• synthetic clotting factors (injections routinely), if severely deficient, they might have an allergic response b/c the body will recognize that it’s synthetic

• desmopressin (DDAVP) a synthetic ADH works with factor VIII to trigger factor X = boost effectiveness of the factor VIII present.

sicky cell

100

name what sickled cells are poor carriers of?

oxygen

200

when does a sickle event occur after birth and why?

within the first year of life, when the hemoglobin is being replaced - plan accordingly (hydration, immunizations, prophylactic antibiotics, recognize signs of infection = receive medical care for crisis, and assess for splenomegaly)

300

potential curative measures?

hematopoietic stem cell transplantation or gene editing

400

best ways to manage a crisis:

• hydration & infection avoidance, IN CRISIS: rest to minimize O2 requirements, hydration (oral or IV), electrolyte monitoring, pain control, blood replacement to correct anemia (body produces abnormal RBCs that will sickle again, not permanent tx), and replace abnormal Hgb, treat underlying the infection

500

list crisis symptoms of sickle cell (broad - potential effects)

• Greatest concern: accumulation in the brain & heart, oxygenated blood cannot enter these areas - anywhere these occlusive crises occur = pain, decreased oxygenation = potential cellular death

• *If abnormal blood cells pass through the spleen & liver = may go into hypovolemic shock

iron-deficient

100

list some great sources of iron:

iron-fortified cereals/breads, dark leafy greens, & red meats, poultry, & seafood

200

name food item that competes and/or causes GI upset, what is the recommended amount, what med helps with absorption of iron?

cow's milk, 2-3 cups/day, and vitamin C

300

what way should iron be administered to school-aged kids?

a syringe

400

list 3 of the common RF for iron-deficient anemia

• adolescent due to poor nutrition, rapid growth, & menstruation

500

this is the most common in the world due to 

parasitic infections draining nutrients

immune thrombocytopenia (ITP)

100

what is the body doing with ITP & what does this tend to cause?

the body is destroying its own platelets, 

• prolonged bleeding, purpura, & petechiae

200

when does immune thrombocytopenia present?

after a viral illness

300

name two interventions to reduce this problem, which one is not done lightly & why

• reduce injury/chances of bleeding

• remove spleen that is getting rid of marked platelets if chronic (not done lightly b/c it is an immune organ = prone to infection, decreased immune memory)

400

what could help prevent something like this?

vaccinations, but not always

500

priority management for prolonged bleeding?

stop the bleeding, hold down firm, consistent pressure without peeking