week 3- hematologic abnormalities

Question 1

erythropoeisis

Answer 1

1. stimulated by erythropoietin (EPO), which is secreted by the kidneys in response to hypoxia
2. it stimulates myeloid stem cells to divide, forming erythroblasts
   - proerythroblasts (no hgb) and erythroblasts are found in bone marrow
3. erythroblasts lose their nucleus to fit more hgb in the cell, forming reticulocytes
4. reticulocytes lose their mitochondria and ribosomes, forming a RBC
   - reticulocytes and erythrocytes are found in the blood

Question 2

anemia

Answer 2

• reduction in the quality or quantity of RBCs
• impacts 1/3 of global pop
• diverse etiology, occurs when RBC loss is greater than RBC production

Question 3

severity of anemia

Answer 3

• hgb 100-120 g/L: mild, may be asymptomatic or symptoms with exertion
• hbg 60-100 g/L: moderate, symptoms at rest and with activity
• hgb ≤60 g/L: severe, multi-system symptoms

Question 4

clinical manifestations of anemia

Answer 4

a) integumentary: pallor, jaundice and pruritis
- pallor from dec hgb and dec blood flow to skin
- jaundice from inc hemolysis leading to bili accumulation
b) resp: tachypnea, dyspnea, orthopnea
c) cv: tachycardia, angina, heart failure, MI
- tachy from heart inc CO to improve oxygenation
d) neuro: dizziness, vertigo, agitation, impaired thought process, fatigue

Question 5

nursing assessment for anemia

Answer 5

a) integ: pale skin and mucous membranes, dec skin turgor, spoon-shaped finegrnails, jaundice, petechiae, bruising
b) resp: tachypnea
c) cv: tachy, edema, postural hypotension
d) neuro: headache, irritability, imparied judgement, lethargy, loss of vibration sense
e) GI: hepatomegaly, splenomegaly, glossitis, abdominal distension
f) pt history: blood/clotting disorders, recent infections, smoking, endocrine or renal disease, recent surgery, diet
g) medications: anticoags, iron, oral contraceptives, ASA, NSAIDS

Question 6

nursing planning and implementation for anemia

Answer 6

a) goals: engage in normal ADLs, adequate nutrition, prevent complications
b) implementation: blood transfusions, replace blood volume, medications, oxygen, dietary modifications

Question 7

anemias from dec erythrocyte production

Answer 7

1. decreased hgb synthesis: iron-deficiency anemia, thalassemia
2. defective DNA synthesis: megaloblastic anemia
3. decreased erythrocyte precursors: aplastic anemia, anemia of chronic disease

Question 8

iron deficiency anemia

Answer 8

• most common nutritional disorder, caused by dec RBC production
• inadequate iron to make hgb, so RBCs are microcytic (small) and hypochromic (pale)
• those at risk for IDA are premenopausal and pregnant women, low SES, older adults, exp blood loss, GI disorders of absorption

Question 9

iron deficiency anemia: etiology

Answer 9

• iron is obtained through diet and supplements
• inadequate diets may not contain enough iron or vitamins (B12, folic acid) for erythropoeisis
• GI malabsorption can impact iron avail such as with chron’s, diverticulitis or celiac
• can also be derived from hemorrhage or slow bleeding ulcers

Question 10

iron deficiency anemia: symptoms

Answer 10

a) pallor: dec hbg and blood flow to skin
b) glossitis: inflam of tongue
c) chelitis: skin bd in corners of mouth
d) burning sensation on tongue: tongue sloughs off epithelium and body is unable to regenerate it
e) pressure headaches: vasodilation from CO2 accumulation
f) paresthesis: dec myelin sheath prod leads to tingling
g) pica: iron is imp for dopamine synth, chewing on hard things wakes you up
h) restless legs: dec O2 to legs

Question 11

iron deficiency anemia: diagnosis

Answer 11

CBC, reticulocyte count, blood smear, serum iron, serum ferritin/transferrin, stool occult blood

Question 12

iron deificiency anemia: treatment

Answer 12

• aim for nutrition from food, then supplements
• oral supplements preffered, ferrous fumarate
• 150-200mg elemental iron per day
• requires continued monitoring through bloodwork, every 3 months
• iron is absorbed in the duodenum

Question 13

considerations for iron supplements

Answer 13

• best absorbed in acidic environment, take w vitamin C on empty stomach
• avoid taking w calcium (chelating agent) or antacids
• AE: GI disturbances, stain teeth (liquid forms), toxic in large amounts
• parenteral supplements are used w malabsorption disorders

Question 14

thalassemia

Answer 14

• inad production of hgb from reduced globulin protein (alpha or beta)
• more prevalent in people of mediterranean, south asian, middle eastern, south russia, india, pakistan or chinese descent (near the equator)
• autosomal recessive disorder

Question 15

heterozygous thalassemia

Answer 15

• thalassemia minor, asymptomatic
• mild to mod anemia with microcytosis, hypochromia, mild splenomegaly and some BM hyperplasia
• normal gene compensates for thalassemia gene

Question 16

homozygous thalassemia

Answer 16

• thalassemia major, severe and life-threatening
• impacts physical and mental dev
• pale, jaundice, BM hyperplasia, hepatomegaly, splenomegaly, cardiomegaly

Question 17

bone marrow hyperplasia

Answer 17

1. low O2 from dec hgb prod leads to stimulation of erythropoeisis in the BM
2. BM creates lots of erythroid precursors but important globulins are missing, so the precursors die in the BM
3. the demand for RBC prod continues and we see an inc in BM space (hyperplasia)
4. bony appearance can appear altered, esp cranium or maxilla

Question 18

thalassemia treatment

Answer 18

a) minor: body adapts, minimal treatment rqrd although genetic counselling is imp
b) major: transfusions and chelating agents
- transfusions to keep hgb >100g/L to avoid splenomegaly
- vitamin C given w transfusions to inc excretion of iron
- bone marrow transplant is curative

Question 19

megaloblastic anemia

Answer 19

• large RBCs are characterisitc
• DNA synthesis is impaired, RBCs that mature are defective and large, with fragile cell membranes
• often occur from deficiency in folic acid or vitamin B12, medications and erythroleukemia

Question 20

cobalamin (B12) deficiency

Answer 20

• more common in people of africa or scandinavian descent
• often begins in middle age
• develops from perniscious anemia (lack of IF)
• symptoms are mainly from hypoxia and include sore/red tongue, N/V, abdo pain, neurologic changes from demyelination of nerve tissue (paresthesia, weakness. dec ability to feel vibration, impaired though process)

Question 21

intrinsic factor (IF)

Answer 21

• lack of IF leads to perniscious anemia
• needed for B12 absoprtion in the ileum
• IF secretion can be impacted by autoimmune destruction of mucosal cells, GI surgery, celiac dosease, diverticulitis of small intestine, chron’s disease

Question 22

cobalamin deficiency: diagnosis and treatement

Answer 22

a) diagnosis: CBC and blood smear (macrocytic, thin walled), serum B12, serum anti-IF ab
b) treatment
- if gut intact: high dose oral/SL B12
- if absorption imp: 100mcg/d B12 for 14d then q week until hgb is normal,then q month for life

Question 23

anemia from acute blood loss

Answer 23

• caused by surgery or trauma, can lead to hypovolemic or hemmorhagic shock
• important to monitor signs and symptoms as lab values may take time to change
• if gradual, the body compensates by expanding plasma volume to dec hemoconcentration (imp bp but RBCs still low)

Question 24

nursing priorities w anemia from acute blood loss

Answer 24

1. replace blood volume: IV fluids first (NS, RL), then blood
2. find source of bleeding
3. monitor drain output
4. may need supplemental iron

Question 25

anemia from erythrocyte destruction

Answer 25

a) intrinsic: from factors w/in RBC b) external: from factors external to RBC

Question 26

sickle cell disease

Answer 26

- autosomal recessive disease w abnormal hgb on RBC - causes RBC to stiffen, elongate and sickle - occurs in response to low O2 in the blood - drastically reduces life expectancy and causes irreversible damage to kidneys, lungs, brain, retina and bones - more prevalent in african descent but also high rates in south/central american, saudi arabian, indian and mediterranean descent

Question 27

sickling episodes

Answer 27

1. sickled RBCs cannot pass through capillaries leading to vascular occlusion 2. acute or chronic tissue injury occurs, as well as local hypoxia/deoxygenation of RBCs 3. this results in inc sickling and sickled cells are hemolyzed by spleen 4. this leads to anemia

Question 28

sickle cell crisis

Answer 28

1. severe sickling and vasoocclusive crisis leads to vasospasm and resitricted bloodflow, inc capillary hypoxia 2. this causes changes in membrane permeability and plasma leaks out of blood vessels, leading to hemoconcentration 3. hemoconcentration inc clotting risk and thrombi can lead to reduced BF, ischemia and infarction

Question 29

sickle cell disease: clinical manifestations

Answer 29

chronic pain/illness, pallor, fever, edema (fluid leaves vessels), tachypnea/tachycardia, infection (spleen cannot phagocytose foreign bacteria/infarcts and becomes dysfunctional)

Question 30

sickle cell disease: diagnosis/treatment

Answer 30

a) diagnosis: blood smear, hemoglobin, electrophoresis (how much sickled hgbS is present) b) treatment: goals to prevent complications, dec manifestations and organ damage, and treat complications quickly - patient teaching (avoid smoking, cold env, stay hydrated, avoid strenuous activity) - hospitalize for O2 admin - comprehensive H2T - fluid and electrolytes to dec blood viscosity - multimodal pain management

Question 31

disseminated IV coag

Answer 31

- bleeding and thrombotic disorder from abnormally initiated and accelerated clotting and anti-clotting processes - occurs from disease or injury - TREAT THE CAUSE

Question 32

DIC patho

Answer 32

1. something causes release of TF and inflammatory cytokines, leading to uncontrolled activation of clotting 2. systemic thrombin generation causes fibrinogen to convert to fibrin and microvascular clots forms within circulation 3. clots lodge in organs and cause imp perfusion and organ dysfn 4. as clotting continues, platelets and clotting factors are consumed 5. fibrinolysis ensures, breaking down fibrin clots and releasing FDPs and D-dimer 6. this leads to more bleeding

Question 33

DIC treatment

Answer 33

- stabilize the pt w oxygen, IV fluids - treat underlying cause and sequelae of DIC - support w blood products if currently bleeding (weigh pros and cons of inc clotting factors in blood)

Question 34

hemophillia

Answer 34

- x-linked recessive disorder w defective coag factors - **hemophillia A:** factor 13 deficiency (80% of cases) - **hemophillia B:** factor 9 deficiency

Question 35

hemophillia: clinical manifestations

Answer 35

- slow, prolonged bleeding from minor trauma - uncontrollable bleeding after more invasive trauma - bloody nose - ulcer/gastric bleeding - subq hematomas causing compartment syndrome - hemoarthritis causing joint deformity - neurologic symptoms from nerve compression (from hematoma)

Question 36

hemophillia: treatment

Answer 36

a) factor replacement therapy - used prophylactivally to dec bleeding episodes and joint deformity - used during bleeding episodes to control bleeding b) DDAVP: acts on platelets and endothelial cells to release von willebrand factor (binds to factor 8) - lasts for 12h c) emicizumab - monoclonal antibody that helps activate other factors and improve clotting

Question 37

hemophillia: nursing considerations

Answer 37

- genetic counselling - immunizations given SC instead of IM (reduced efficacy) - can apply topipcal hemostatic medication/dressing and apply direct pressure - administer appropriate clotting factor - if joint is bleeding, rest it, pack with ice and give tylenol