Lecture 1: Hematopoietic System Part 1 (Anemia)

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INCREASE in #

_penia

DECREASE in #

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blood

bone marrow

• specialized connective tissue + many capillaries

• immature, intermediate, mature forms of blood cells

erythropoiesis

• RBC production

• stimulated by erythropoietin from KIDNEY

stem cell reproduction

• yolk sac = embryonic life

• fetal liver = 3-6mth gestation

• bone marrow = 6+ mth gest (ONLY site of blood cell production after birth, stores RBC/WBC)

• precursor cells to

  • RBC

  • Platelet

  • Granulocytes

  • lymphocytes

  • monocytes

Stem cell

• Erythroblast

• Myeloblast

• Monoblast

• Lymphoblast

• Megakaryoblast

Erythroblast (immature) 

Normoblast → reticulocyte → erythrocytes (RBC) (carry O2 via hemoglobin, CO2 back to lungs, acid-base balance)

Myeloblast (immature) 

• Basophilic → basophilic (allergy/inflammation = histamine)

• Eosinophilic → eosinophil (parasite defense + asthma) 

• Neutrophilic → neutrophil (1st response to infection, kill bacteria via phagocytosis)

Granulocytes (immature) 

• Basophilic (WBC)

• Eosinophil (WBC)

• Neutrophil (WBC)

Monoblast (immature)

• Monocyte (WBC) (long-term immune = macrophages + dendritic cells) 

Lymphoblast (immature) 

• Lymphocyte (WBC) (B (antibodies) and T lymphocytes (helper/killer)  = adaptive immunity) 

Agranulocytes

• Monocyte (WBC) (long-term immune = macrophages + dendritic cells) 

• Lymphocyte (WBC) (B (antibodies) and T lymphocytes (helper/killer)  = adaptive immunity) 

Megakaryoblast (immature)

• Thrombocytes (platelets for clotting/coagulation) 

Erythrocytes

• RBC

• Transport O2/CO2 + acid-base balance

• Bioconcave shape = flexibility = pass through tiny capillaries

• Thin cell membrane = diffusion of gases 

• Composed of hemoglobin

Leukocytes

• granulocytes

  • Basophilic (WBC)

  • Eosinophil (WBC)

  • Neutrophil (WBC)

• agranulocytes

  • Monocyte (WBC) (long-term immune = macrophages + dendritic cells) 

  • Lymphocyte (WBC) (B (antibodies) and T lymphocytes (helper/killer)  = adaptive immunity) 

Blood

• Connective tissue

• 3 major functions

  • Protection

  • Regulation

  • Transportation 

• 2 components

  • Plasma

  • Blood cells 

Plasma

• 55% of blood + serum (plasma withOUT water)

• Water mostly 

  • Proteins = Fibrinogen

  • Electrolytes

  • Gases

  • Nutrients

  • Waste

Plasma proteins

• Albumin

• Globuline

• Clotting factors (fibrinogen) 

Protection - Functions of the blood

• Maintaining homeostasis of blood coagulation

• Combating invasion of pathogens/foreign substances

Regulation - Functions of the blood

• fluid/electrolyte balance

• Acid-base balance (erythrocytes as a buffer) 

• Body temp

• Maintain intravascular oncotic pressure

Transportation - Functions of the blood

• O2 from lungs → cells 

• Nutrients from GI tract → cells

• Hormones from endocrine glands → tissue/cells

• Metabolic waste products (CO2, NH3 (ammonia), urea) from cells → lungs/liver/kidneys 

Fe processing

• digest (diet/supplement)

• transports via transferrin

  • bone marrow, liver, spleen (transferrin binds to Fe)

  • Fe → RBC, store if RBC adequate in Hb

    • RBC breakdown every 120days → liver/spleen, release bili

    • Fe REMAINS (lost via blood loss or recycled when RBC dies)

• recycling

  • macrophages in liver/spleen

  • Hemosiderin = Fe storage in tissue when RBC breakdown

RBC - Erythrocytes

• NO nucleus

• biconcave disk

• transport O2

amount of O2 transported in RBC-Erythrocytes

• # of RBC in circulation

• amount of Hb within

WBC

• granulocytes (neutrophil, esosinhil, basophil)

  • Inflammation rxn

• lymphocytes

  • T and B lymphocytes

• monocytes

  • various rxn

platelets (thrombocytes)

• cytoplasmic fragments of megakaryocyte

• NOT cells

  • no nucleus/limited metabolic machinery

• granules of chemical mediators = clotting/inflammation

• must be replaced con’t

Anemia

• frequent and serious disease (1/3 of world population)

• deficiency in 1+

  • erythrocytes

  • quantity of Hb

  • quality of Hb

  • volume of packed RBCs

3 morphological classifications

1. normocytic, normochromic (normal size/colour)

2. macrocytic, normochromic (large size, normal colour)

3. microcytic, hypochromic (small size, PALE colour)

Normocytic, normochromic

• (normal size and colour)

• malnutrition

• acute blood loss

• chronic renal diseases

• cancers

• hemolytic anemia

• sickle cell anemia

• aplastic anemia (bone marrow does not produce enough blood cells)

Macrocytic, normochromic

• (large size, normal colour)

• vitamin B12-deficiency anemia (defective DNA syn)

• folic acid-deficiency anemia (defective DNA syn)

• liver disease

Microcytic, hypochromic

• (small size, pale colour)

• iron-deficient anemia (decreased hema syn)

• thalassemia (inherited hemoglobin synthesis disorder, chronic microcytic hemolytic anemia due to reduce/absent globin chain production) (decreased hema syn)

• vitamin B6 deficient

• lead poisoning

classification by etiology

• decreased erythrocyte production

• defective DNA synthesis

• decreased hemoglobin synthesis

• decrease number of erythrocyte precursors blood loss

• increased erythrocyte destruction

  • intrinsic or extrinsic

defective DNA synthesis (etiology)

• Cobalamin (vit B12) deficient

• folic acid deficient

decreased hemoglobin synthesis (etiology)

• Iron deficient

• Thalassemias (decrease globin synthesis)

• sideroblastic anemia (decreased porphyrin)

decrease number of erythrocyte precursors (etiology)

• aplastic anemia + inherited disorders

  • fanconi syndrome

• anemia of myeloproliferative disorders + myelodysplasia

• chronic disease/disorders

• medications (chemo)

• radiation

blood loss (etiology)

• acute

  • trauma

  • blood vessel rupture

  • splenic sequestration crisis

• chronic

  • gastritis

  • menstrual flow

  • hemorrhoids

increased erythrocyte destruction

• intrinsic

  • abnormal hemoglobin (sickle cell)

  • enzyme deficient (G6PD)

  • membrane abnormalities (paroxysmal nocturnal hemoglobinuria/hereditary spherocytosis)

• extrinsic

  • physical trauma (prosthetic heart valves)

  • acquired antibodies against RBCs

  • infectious agents, meds, toxins

  • disseminated intravascular coagulopathy (DIC)

  • HELLP syndrome

  • thrombotic thrombocytopenic purpura (TTP)

  • cancer

mild anemia manifestations

• Hb = 100-120 g/L

• palpitations

• exertional dyspnea

moderate anemia manifestations

• Hb = 60-100 g/L

• increased palpitations, bounding pulse

• dyspnea (roaring in ears)

• fatigue

severe anemia manifestations

• hb = <60 g/L

• pallor, jaundice, pruritus

• icteric conjunctiva/scarla, retinal hemorrhage, blurred vision

• glossitis (smooth tongue)

• tachycardia, increase pulse pressure, systolic murmurs, agina, HF, MI

• tachypnea, orthopnea, dyspnea at rest

• headache, vertigo, irritable, depression, impaired thought

• anorexia, hepatomegaly/splenomegaly (increase size), sore mouth, dif swallow

• bone pain

• sensitive to cold, weight loss, lethargy

inspection - assessment

• gen appearance

  • pallor, fatigue, weight loss

• skin/mucous mem

  • pale conjunctiva

  • pale oral muscosa

  • jaundice

  • petechia

  • delayed cap refill

• VS

  • tachycardia

  • tachypnea

  • hypotension (blood loss)

  • orthostatic changes

palpation - assessment

• skin/perfusion

  • cool skin

  • delayed cap refill

  • dry skin

  • turgor

• pulse

  • tachy

  • weak or building

  • symmetry

• lymph nodes

• abdo

  • splenomegaly and hepatomegaly

RBC cell count lab test

• # of RBCs in the blood

• Female: 3.50–5.00 × 1012/L

• Male: 4.00–5.50 × 1012/L

Hemoglobin HgB lab test

• amount of Hb in RBC

• Female: 115–155 g/L

• Male: 125–170 g/L

mean corpuscular hemoglobin (MCH) lab test

• average amount of Hb in a single RBC

• Female: 25–34 pg

• Male: 27–34 pg

• pg = picograms

Mean corpuscular volume (MCV) lab test

• average SIZE of RBC cells

• 80 - 100 fL

• fL = femtoliters

hematocrit (HCT) lab test

• % of RBC in the blood

• 36 - 50%

Anemia Drugs (4)

1. Ferrous Salts and Injectables Iron Supplements

2. Cyanocobalamin (B12) Water-soluble B-Complex

3. Folic Acid/Folate Water-soluble B-Complex

4. Epoetin Alpha (Eprex) Darbepoetin (Aranesp)

Ferrous Salts and Injectables

• Ferrous Salts PO = ferrous gluconate, ferrous sulphate/sulphate, ferrous fumarate

• injectables = iron dextran, iron sucrose, ferric gluconate, ferumoxytol

• class = iron supplements

• MOA = Fe as O2 carrier in HgB and myoglobin (O2 carrying molecule in muscle tissue)

• Contra = drug allergy, hemochromatosis (Fe overload), hemolytic anemia, other anemia NOT associated with Fe deficiency

• AE = N/V, diarrhea, constipation, dark stool, stomach cramps = TAKE WITH FOOD

• Nursing = Fe absorption enhanced with Ascorbic Acid (Vit C), nut assessment (pt diet), energy level for ADLs, monitor constipation

• Time Action

  • PO Ferrous Salts = reticulocyte 3-5 days, Hb rise 1-2wks, normal 6-8 wks, replenished 3-6mths

  • injectable (parenteral) Fe = reticulocyte 2-4days, Hg rise 1 wk, normal 2-4wks, replenished faster than PO

Cyanocobalamin (B12)

• class = Water-soluble B-Complex

• DEEP IM

• MOA = coenzyme for metabolic pathways (fat/carbs) + protein syn. responsible for growth, cell replication, hematopoiesis, nucleoprotein, myeline syn

• indications = pernicious anemia, deficiency via malabsorption

• contra = drug allergy, COBALT sensitive, heredity optic nerve atrophy (Leber’s disease)

• AE = heart failure, PVT, pulmonary edema, flushing, optic nerve atrophy, diarrhea, pruritus, rash, HYPOKALEMIA

• Nursing = assess med order, route of admin, GI stat, decreased efficiency with anticonvulsants, aminoglycoside AB, long-acting potassium prep

• Peak plasma Conc = 8-12hrs

• half-life = 6 days

Folic Acid/Folate

Class = Water-soluble B-Complex

MOA = erythropoiesis and syn of nucleic acids (DNA/RNA)

Indications = folic acid deficient anemia, prevention of neural tube defects, tropical sprue (malabsorption syndrome)

conta = drug allergy, anemia not caused by folic acid deficient

AE = allergic rxn (rare), yellow discolouration of urine

Nursing = assess/monitor nutritional intake, blood work, interactions with PO contraceptives, corticosteroids, sulfonamides, dihydrofolate reductase inhibitors (methotrexate) and abx trimethoprim

peak plasma = 60-90 min

Epoetin Alpha (Eprex) Darbepoetin (Aranesp)

• injectable only (IV/SC)

• class = human recombinant hormone (erythropoietin) analogue

• MOA = biosynthetic form of natural hormone erythropoietin (excreted from kidneys in response to decrease RBCs) stims RBC manufacturing/maturation of bone marrow

• contra = drug allergy, uncontrolled HTN (hypertension), red cell aplasia, Hgb 100g/L + for cancer pt, Hgb 130g/L + for kidney disease pt

• AE = HTN, fever, pruritus, rash, N/V, arthralgia (pain at joint), injection site rxn

• nursing = check bloodwork (Hgb), VS, allergy rxn

• onset = 7-10 days

• half life = 4-13hr

• peak plasma concentration = 5-24hr

hemolytic anemias

• RBC prematurely destroyed by mononuclear phagocytes

• hyperplastic marrow leads to reticulocytosis (elevated immature RBCs in blood)

• pt are NOT depleted of Iron

hemolytic anemias may be caused by…

• production of defective RBCs

• events that affect normal cells

sickle cell anemia

• altered sequence of a.a. in globin molecule

• person with 2 hemoglobin S genes

• RBC collapse into sickle shape

• more susceptible to rupture/premature death

• can sludge obstruct small blood vessels

manifestations of sickle cell anemia

• most present healthy but are anemic

• may have chronic conditions/pain due to organ/tissue hypoxia (not enough O2)/damage

• ± jaundice

sickling episode

• pain = mild-excruicitating

• impacting ANY area of the body

• ± fever

• ± swelling

• ± tenderness

• ± tachypnea

• ± hyPERtension

• ± N/V

complications of sickle cell anemia

• brain = trhombosis/hemorrhage = paraylsis/sensory deficit/death

• eye = hemorrhage/retinal detachment

• lung = acute chest syndrome/pulmonary hypertension/pneumonia

• heart = failure

• liver/gallbladder = hepatomegaly/gallstone

• kidney = hematuria/renal failure

• spleen = atrophy

• bones/joints = hand-foot syn/osteonecrosis

• penis = priapism

• skin = ulcers

collaborative care for sickle cell anemia

• preventing dequalea from disease

• alleviate manifestations from complication

• minimize end-target organ damage

• promptly treating serious sequelae

acute care for sickle cell anemia

• O2

• NB assess resp stat

• fluids/electrolytes

• collab care for pain management

• rest

• DVT prophylaxis

• ± antibiotics

pt education for sickle cell anemia

• avoid triggers

  • high altitude

  • extreme temps

  • stress

• adequate fluid intake

• preventing/treating infections

• screening

  • retinopathy (eye)

pharmacotherapeutic for sickle cell crisis

hydroxyurea (PO) - NOT in sealock

hydroxyurea (PO)

• class = antineoplastic (myelosuppressive agent)

• MOA = antimetabolite reduces vaso-occlusive pain crisis, needed for transfusion of sickle cell pt by reducing marrow production of neutrophils, reticulocytes, and platelets (all mediators of inflammation)

• indications = vaso-occlusive sickle cell crisis, squamous cell carcinoma, chronic myeloid leukemia, acute chest syndrome, severe symptomatic anemia, hemolytic antibodies/anticoagulants

• contra = preg, active liver disease (HBV/HCV)

• AE = headache, GI, nausea, skin hyperpigmentation/darkening of nails

• nursing = check VS, bloodwork, opreg test women of reproductive age

• peak plasma concentration = 1-4hrs

• 76yr + anemia (no feel toes/tips fingers walking, tired, fatigue, SOB)

• cause of s/s

adequate amounts of Hb needed to carry O2 for tissue metabolism

• 32yr female pt (fatigue, dizzy, SOB exertion 2mths, heavy menstrual, veg diet)

• VS

  • BP = 110/70

  • HR = 104 bpm

  • RR 20/min

  • SpO2 = 98%

• what diagnostic test nurse expect order?

• Hgb = 90 g/L (normal 115-155 g/L)

• Hematocrit (Hct): 26% (normal: 36–50%)

• Mean corpuscular volume (MCV): 72 fL (normal: 80–100 fL)

• Ferritin: 10 ng/mL (normal: 15–150 ng/mL)

iron deficiency anemia consistent findings

• low MCV

• fatigue

• tachycardia

• heavy menstrual bleeding

• NOT

  • elevated ferritin

  • hypotension

which intervention needed for 32yr female pt (fatigue, dizzy, SOB exertion 2mths, heavy menstrual, veg diet)

• encourage intake of vit C

• admin oral ferrous as prescribed

• monitor Hg + hematocrit levels

• NOT

  • Iron fortified milk

  • blood transfusion