1

Hematology Nursing – ■ Myeloid progenitor ○ Formed elements

cells ■ Erythrocytes
Ma’am Robles ○ Lineage committed cells ■ Leukocytes
Lesson 1 ■ B-cell progenitor cells ■ Platelets
■ T-cell and natural
Objectives: killed cell progenitor Plasma 55%
❏ Recall anaphy of blood cells Buffy Coat <1%
❏ Identify importance of basic CBC ■ Granulocyte and Red Blood Cells 45%
components monocyte progenitor
❏ Identify causes of anemia cells Functions of Blood
❏ Enhance clinical eye on the ■ Megakaryocyte ● Transport
assessment of anemia ○ Specialized blood cells ○ O2, CO2, nutrients, wastes,
❏ Formulate plan of care for patient ■ B-cells hormones, etc.
with anemia ■ Natural killer cell ● Protection
■ T-cells ○ WBC, antibodies, platelets,
■ Granulocytes compliment CHON
Review of Blood Formation: (basophils, ● Regulation
● Stem Cell Possibilities neutrophils, ○ Fluid and temperature,
○ A stem cell can become any eosinophils) regulation and buffering
one of the 220 different cells ■ Monocytes and ○ 37 degrees
in the body macrophages ○ pH 7.35–7.45
■ Kidney, liver, heart, ■ Thrombocytes
lungs, brain (platelets) Blood Volume
● Hierarchy of Stem Cells ● Adults have 4-6L of blood (7-10%)
○ Multipotent stem cells ● Plasma, a clear extracellular fluid
■ Hematopoietic stem Blood ● Formed elements (blood cells and
cell Composition of Blood platelets)
○ Primitive progenitor cells ● Blood is a fluid connective tissue
■ Lymphoid progenitor composed of:
cells ○ Plasma Plasma

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● Plasma – liquid portion of blood
○ Serum remains after plasma
clots
● 3 major categories of plasma
proteins
○ Albumin – keeps fluid in
your bloodstream
○ Globulin –  ● 175B/day
○ Fibrinogen – clotting factor
Other Components of Plasma
● Nitrogenous by-products of
metabolism
○ Lactic acid, urea, crea,
CHON, etc.
● Nutrients – glucose, carbohydrates,
amino acids
● Electrolytes – Na, K, Cl, HCO3
● Respiratory gases – O2 and CO2
● Hormones
Erythrocytes
Overview
● Biconcave disc, anucleated,
essentially no organelles
● 7-8 microns
● 5-7 days
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● Have a flexible cytoskeleton that
allows them to move through narrow
spaces
● Cytoplasm is filled with hemoglobin
for gas transport
● Are the major factor contribution to
blood viscosity
● 1-2% reticulocytes
● 5M/microliter (mm3)
● 120 days
Erythrocyte Functions
● Gas transport - major function
○ Due to loss of organelles
during maturation
○ Increase surface area/volume
ratio
● 26-33% of cytoplasm is hemoglobin
○ O2 delivery to tissue and
CO2 transport to lungs
● Important role in gas transport and
pH balance
Hemoglobin Structure Overview
● Protein globein: 2 alpha chain and 2
beta chain
○ Heme pigment centrally
bonded to each globin
2
● Iron atom in each heme can bind to 1
O2 molecule
● Each Hb molecule can transport 4
O2 molecule
● Men 14-16g/dl
● Women 12-14g/dl
Hematocrit
● RBC count and hemoglobin
concentration indicate an O2
carrying capacity of blood
● Hematocrit (packed cell volume) - %
of blood composed of cells
● Men 42-52%
● Women 37-48%
Erythrocyte Disorders
● Increased –
Erythrocytosis/Polycythemia
● Decreased – Anemia
Leukocytes
● 5,000-10,000 WBC/microliter
● Conspicuous nucleus
● Travel in blood before migrating to
connective tissue and lymphatic
tissue
● Protects against pathogens
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● Less than RBC ––
● Stored in RBM
● RES Basophils
● 9-12 microns Leukocyte Disorders
Leukocyte Classes ● 0.5-1% 1. Leukopenia – low WBC count
● Granulocytes ● Few hours-few days – damaged RBM
○ Neutrophils 50-70% ● Blue stain a. Causes: radiation, poisons
○ Eosinophils 2-4% 2. Leukocytosis: high WBC count
○ Basophils 0.5-1% a. Causes: infection, allergy and
● Agranulocytes Eosinophils disease
○ Lymphocytes 25-45% ● 10-14 microns 3. Leukemoid reaction – uncontrolled
○ Monocytes 3-8% ● 2-4% WBC reaction/overproduction
● 5 days 4. Leukemia ???
Leukocyte Functions (NBEML) ● 2 lobes
● Neutrophils – 1st responder, ● Bright red
bacterial infections Platelets
● Basophils – allergic reaction ● Small fragments of megakaryocytes
● Eosinophils – parasitic & allergic Lymphocyte cytoplasm
reaction ● 5-10/10-18microns ● Sticky, circulate in clumps
● Monocytes – chronic infections ● 25-45% ● 150,000-400,000/ml
● Lymphocytes – viral infections & ● 2-3 µ (micro)m, contain granules
chronic infection ● Secrete clotting factor and growth
Monocyte factors for vessel repair
● Kidney shaped ● Initiate formation of clot
Neutrophils ● 16-25microns (HEMOSTASIS)
● 9-12microns ● 3-8%
● 50-70% ● Months-years
● 3-5 lobes Erythropoiesis
● 1-2 days/6 hours ● Production of erythrocytes
● Blue and pink granules Reticulo Endothelial System ● Regulated by:

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○ Balance in erythrocytes a. Total amount of Hb in a
production and destruction given volume of RBC
○ Blood oxygen levels Nutritional Requirements 4. Mean Corpuscular Volume (MCV)
○ Hormonally controlled (EPO ● Adequate protein intake a. Average volume of RNC
and testosterone) **blood ● Adequate iron intake 5. Mean Corpuscular Hb (MCH)
doping** ● Adequate vitamin intake a. Average amount of Hb in
● Excessive effects: blood viscosity RBC
● Decreased effects: O2 carrying 6. Mean Corpuscular Hc
capacity Concentration (MCHC)
a. Concentration of Hb in a
given volume of RBC
Hematopoiesis 7. Normocytic cells
–– a. Normal sized cell
8. Microcytic cells
a. Small sized cell
Erythropoiesis 9. Macrocytic cells
–– a. Large sized cells
10. Normochromic cells
a. Normal color or normal
Leukopoiesis Complete Blood Count (Anemia) degree of hemoglobinization
● Chemically regulated by other Anemia Indicators 11. Hypochromic cells
WBC’s and other physiologic 1. Packed Cell Volume (PCV)/Hct a. Less degree of
functions a. Volume in a given blood hemoglobinization
● Colony Forming Units (CFU) occupied by RBC when 12. Hyperchromic cells
stimulate production of specific packed a. High degree of
leukocytes 2. RBC count hemoglobinization
a. Number of RNC in a given 13. Red cell Distribution Width
volume of blood a. Variations in the size of the
Thrombopoiesis 3. Hb/Hgb concentration cells
––

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● Mean Corpuscular Hb (MCH) ● Decreased red cell production
Anemia Indicators ○ Average amount of Hb in ● Increased red cell destruction
● Packed Cell Volume (PCV) / Hct RBC ● Blood loss
○ Volume in a given blood ■ Total hemoglobin
occupied by RBC when (Hgb) / total number Symptoms of Anemia
packed of RBC or Red cell ● Eyes
● Red Blood Cell or RBC Count count ● Skin
○ Number of RBC in a given ● Mean Corpuscular Hb ● Respiratory
volume of blood Concentration (MCHC) ● Muscular
■ 1 ml of blood = 0.45 ○ Concentration of Hb in a ● Intestinal
ml Hct and 0.55 ml given volume of RBC ● Central
Plasma ■ Total hemoglobin ● Blood vessels
■ 1 ml of blood = 5 (Hgb) / total volume ● Heart
million RBC of RBC or Hct ● Spleen
● Hb/Hgb concentration ● Red cell distribution width
○ Total amount of Hb in a ○ Variations in the size of the
given volume of RBC cells Nursing Diagnosis
■ 1 ml of blood = 1. Activity intolerance related to
0.45ml Hct, ⅓ is Hgb imbalance between oxygen supply
= 0.15 ml (g)/ml Anemia (delivery) and demand
■ 100ml of blood = Anemia According to WHO 2. Ineffective tissue perfusion
45ml Hct, ⅓ is Hgb = 3. Decreased cardiac output
15ml(g)/dL 4. Disturbed sensory perception
● Mean Corpuscular Volume (MCV) 5. Impaired oral mucous membrane
○ Average volume of RBC 6. Imbalanced nutrition: less than body
■ Total volume requirement
occupied by RBC or 7. Constipation
PCV or Hct / total 8. Deficient knowledge
number of RBC or 9. Low self esteem
Red cell count Different Types of Anemia 10. Fatigue

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11. Fear
12. Risk for infection
13. Risk for injury

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Lesson 2 – Anemia ○ Alcoholic intake
Folic Acid Rich Foods ○ Drugs - sulfa/phenytoin
Objectives: ● Leafy greens ○ Oral contraceptive pills
❏ Know and understand fully types of ● Asparagus ○ Surgical removal of jejunum
anemia due to decreased red cell ● Broccoli
production ● Papaya and oranges Vit B9 Deficiency Signs/Symptoms
❏ Enumerate specific signs and ● Avocado ––
symptoms of each anemia ● Seeds and nuts
❏ Make NCP on the different types of ● Brussels sprouts Vit B 9 Deficiency Anemia Lab Findings
anemia ● Beans, peas, lentils ● Decreased serum B9 level
● CBC – Low HCT
12 Foods Rich in Folate ● CBC – Low HGB
Different Types of Anemia ● Okra ● CBC – Low RBC count
● Decreased red cell production ● Cauliflower ● CBC – High MCV macrocytic
○ Macrocytic, megaloblastic ● Beets ● CBC – normal to hypochromia
anemia (B12 & B9) ● Bell peppers ● CBC – hypersegmented neutrophils
○ Microcytic (iron deficiency) ● Pancytopenia
anemia ● Bone Marrow – megaloblastic
○ Normocytic (aplastic anemia Vit B9 Deficiency Causes
& anemia of chronic disease) ● Reduced intake (most common): Vit B9 Deficiency Treatment
● Increased red cell destruction ○ Food preference ● Folic acid rich foods
● Blood loss ○ Heating ● FA supplement of 4-5mg daily PO
● Increased demand (most common): ● FA parenteral 1mg/day for 1 week
Macrocytic Megaloblastic Anemia ○ Pregnancy ● Folic acid 400 mcg
1. FA Deficiency ○ Lactation ○ 1 hour before or 2 hours after
2. Vitamin B 12 Deficiency ○ Rapid growth spurts meals
○ IDA ○ Nausea
Folic Acid Deficiency Anemia ○ Hemolytic anemia ○ Loss of appetite
–– ○ CA cells ○ Bloating
● Impaiared absorption ○ Mood changes

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○ Bitter or unpleasant taste in
the mouth
○ Don’t give in large doses
Vit B 12 Deficiency
● Vit B12 + Animal CHON = Pepsin
● Vit B12 + R Binder = Pancreatic
enzyme
● Vit B12 + IF = absorb
Vit B12 Deficiency Causes
● Reduced Intake
○ Vegan diet
○ Breastfeed baby with vegan
mother
● Increased demand
○ Uncommon
● Impaired absorption (most common)
○ Gastrectomy
○ Intake of corrosives
○ Elderly - achlorhydria
○ Pt w/ PEG or gastrostomy
tube
○ Pancreatitis
○ Tapeworm infestation
○ Bacterial overgrowth
○ Defective ilium -
sprue/Crohn's/TB/sx
● Pernicious anemia (most common)
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2. IM B12
Pernicious Anemia 3. Collect urine x 24 hours
● Macrocytic, megaloblastic anemia 4. B12 + IF
○ Due to vit B12 deficiency 5. Collect urine x 24 hours
○ Due to IF deficiency 6. AB/anti parasitic x 7 days
○ w/c must be due to 7. B12, then collect urine x 24 hours
○ Autoimmune destruction of 8. B12 + pancreatic enzyme
gastric fundic mucosa 9. Collect urine x 24 hours
● Common after age 40 --- 60 to 80
years old Other diagnostic examinations to rule out
● Sister diseases that run in the family TB, lymphoma, Crohn's disease, IBS
○ Hashimoto thyroiditis
○ Autoimmune Addison’s Vit B12 Deficiency Anemia Treatment
disease ● Increase intake of B12 rich foods
○ Vitiligo ○ Animal products
● Oral Vit B12 supplement
Vit B 12 Deficiency Anemia Lab Findings ○ 1mg daily
● Decreased serum B12 level ● IM Vit B12 supplement
● CBC – low HCT ○ 1 mg weekly for 8 weeks
● CBC – low HGB ○ 1mg monthly for LIFE
● CBC – low RBC count
● CBC – high MCV (macrocytic)
● CBC – normal - hypochromia Microcytic Anemia
● CBC – hypersegmented neutrophils ––
● Pancytopenia
● Bone marrow – megaloblastic Iron Rich Foods
● Schillings test ● Heme iron
● Non-heme iron
Schilling Test
1. Oral radio labeled B12 Total Iron in the Body
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● Iron 4-5 GMS ● Vertigo ■ Green leafy
○ Functional 80% ● Tinnitus vegetables
■ HGB 70% ● Alopecia ● Ferrous sulfate 325ng TID
■ Myoglobin 5% ● Amenorrhea ○ May cause GI upset,
■ Others 5% ● Functional murmur constipation and black stool
○ Storage 20% ○ Normal w/in 2 months
■ Ferritin 15% Iron Deficiency Anemia Lab Findings ○ Cont. 3-6 months
■ Hemosiderin 5% ● Decreased hemosiderin ● Parenteral iron
● Decreased serum ferritin ○ Risk of anaphylaxis
Causes of IDA ● Decreased serum iron
● Diet ● Increased transferrin Vit C – enhances absorption of iron
○ Poor diet ● Decreased transferrin saturation Coffee, tea, carbonates, calcium rich foods
○ Strict vegan ● Increased TIBC – blocks absorption of iron
● Impaired absorption ● CBC – Low HCT
○ Cow’s milk ● CBC – Low HGB Iron Replacement
○ Gastric bypass ● CBC – Low RBC count ● Best taken on empty stomach
● Increased demand ● CBC – Low MCV (microcytic) ● Absorption can be enhanced by vit.
○ Growth spurts ● CBC – Low HGB content C and amino acids
○ Pregnancy (hypochromic) ● Decreased absorption with tea,
○ Lactating ● Anisocytosis and poikilocytosis cereals, wheat and foods/drinks
● Blood loss ● Increased platelets containing phosphates
○ Heavy menstruation ● Decreased absorption due to ab. and
○ Colon cancer Iron Deficiency Anemia Treatment other meds
○ PUD ● Treatment of underlying cause ● Constipation
● Increase intake of iron rich foods ● Dark stool
Signs & Symptoms ○ Heme ● Loss of appetite
● Exertional dyspnea ■ Pork ● Nausea
● Tachycardia ■ Red meat
● Tachypnea ■ Poultry
● Weight loss ○ Non heme Normocytic Anemia

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Anemia of Chronic Disease Causes ● Genetic alteration Aplastic Anemia Lab Results
● Thyroid DSE ○ Hereditary ● CBC – low RBC CT
● Collagen Vascular Disease (RA, ■ Fanconi’s anemia ● CBC – low WBC CT
SLE) ● Acquired/environmental ● CBC – low platelet count
● Inflammatory bowel Disease ○ Chemical subs (medications) ● MCV – normal
(Colitis, Chron’s) ■ Dose related ● MCH – normal
● Malignancy ■ Idiosyncratic way ● Bone marrow aspirate
● Chronic Infectious Diseases (OM, ○ Radiation – hypoplastic/aplastic
TB, HIV, etc.) ○ Virus ● Reticulocytopenia
● Renal disease ○ Idiopathic causes 65% ● Yellow bone marrow
● Myeloid stem cell surrounded by
IDA vs. ACD Pathophysiology of Aplastic Anemia immune cells (lymphocytes)
● IDA – decreased serum FE – Mitosis
increased transferrin – decreased ● Oncogene - cell replication Aplastic Anemia Treatment
ferritin ● Tumor suppressor gene - stops cell ● Young Adult – allogeneic bone
● ACD – decreased serum Fe – replication marrow transplant
decreased transferrin – increased ● >50 = ATG + cyclosporine
ferritin Aplastic Anemia Signs and Symptoms ● Blood transfusion
● Anemia ● Neutropenic regimen
Increased Hepcidin Level ○ Fatigue, DOB, tachypneic, ● Treatment of underlying cause
tachycardic, dizziness, HA, ● Removal of thymus
ACD Treatment pale skin, thin hair etc.
● Treat underlying disease ● Leukopenia
● EPO ○ Frequent and prolonged, Nursing Intervention
● Transfusion recurrent infections ● Neutropenic regime
● Chelation ● Thrombocytopenia ○ Provide well cooked meat, no
● No iron supplement ○ Petechiae, ecchymosis, nose fresh fruits, vegetables, no
and gum bleeding, prolonged fresh flowers
bleed ○ Limit visitors
*Aplastic Anemia* ○ Wear mask

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○ Avoid crowded areas
○ No bathing of pets
○ Drink only pasteurized milk
● Thrombocytopenia
○ Do not take any medicine,
including over-the-counter
drugs
○ To avoid aspirin and other
drugs that contain aspirin
○ Use a soft-bristle toothbrush.
Do not use dental floss
○ Do not have dental work
without your doctor’s
approval
○ Do not do heavy lifting,
contact sports, or strenuous
exercise
○ Do not walk in bare feet
○ When shaving, do not use a
razor blade. Use an electric
razor instead
○ Be careful when using
household tools, such as
knives and scissors
○ Do not blow your nose
forcefully
○ Do not wear tight clothing
○ Prevent constipation
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○ If you are a woman having ● Administer erythropoietin as
your period, do not use prescribed
tampons ● Reduce activities and stimuli that
cause tachycardia
● Allow for rest between periods of
Nursing Diagnosis activity
1. Activity intolerance related to ● Elevate the patient’s head on pillows
imbalance between oxygen supply during episodes of shortness of
(delivery) and demand breath
2. Ineffective tissue perfusion ● Provide extra blankets if the patient
3. Decreased cardiac output feels cold
4. Disturbed sensory perception ● Teach the patient/family about
5. Impaired oral mucous membrane underlying disease and how to
6. Imbalanced nutrition: less than body manage the symptoms of anemia
requirement
7. Constipation
8. Deficient knowledge
9. Low self esteem
10. Fatigue
11. Fear
12. Risk for infection
13. Risk for injury
Nursing Actions for a Patient who is
Anemic
● Administer oxygen as prescribed
● Administer blood products as
prescribed

Lesson 3 – Anemia cont.
*Hereditary Spherocytosis*
● Autosomal Dominant disease of the
Erythrocytes
● Mutations on the genes related to
membrane protein formation
● 200-300 per million in Northern
Europe
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● 1/5000 in United States 3. Cholelithiasis – bili stone
● 1ª Extravascular and Intravascular – cholecystectomy
Hemolysis
Hemolysis – Intracorpuscular – Hereditary
H. Spherocytosis Signs and Symptoms – Enzyme Defect G6PD Deficiency
● Anemia
● Splenomegaly Glucose 6 Phosphate Dehydrogenase
● Mild Jaundice Deficiency
● Pigment stones ● Sex linked recessive disorder
● *most common enzyme deficiency in
H. Spherocytosis Lab Results humans
● Low RBC ● Affects 400M people worldwide
● Low HB ● Africa – affects 20% of the
● Low HCT population
● Low MCV ● Mediterranian – 4%
● Normal MCH ● Southeast – 30%
● High MCHC ● Intravascular Hemolysis –
● High reticulocytes extravascular
● Increased RDW
Glucose 6 Phosphate Dehydrogenase
H. Spherocytosis Treatment Deficiency Signs and Symptoms
● Splenectomy ● Signs and symptoms of anemia
● Exchange transfusion ● Jaundice
● Dark colored urine
H. Spherocytosis Complications ● Kernicterus
1. Aplastic crisis – parvovirus – blood ● Death
transfusion
2. Hemolytic crisis – any DSE - spleen Glucose 6 Phosphate Dehydrogenase
– exchange blood transfusion Deficiency Lab Results
● High reticulocytes
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● High LDH ● Menthol
● Presence of heinz bodies and bite ● Artificial blue food coloring
cells ● Gluten free products
● High bilirubin ● Ampalaya
● Low haptoglobin ● Chinese herbs
● Hemoglobinemia ● black/green tea
● Hemoglobinuria ● Soya
● Acute tubular necrosis
List of Antioxidants
Glucose 6 Phosphate Dehydrogenase ● Berries walnut
Deficiency Treatment ● Sunflower seeds
● Prevent oxidative stress/damage ● Ginger
● Infections ● Grapes Sickle Cell Disease
● Medications ● Orange ● Autosomal recessive genetic disorder
● Foods ● Pineapple ● The most common form of an
● Mothballs or naphtaline ● Prunes inherited blood disorder that causes
● Spinach the production of abnormal
Some Medications That Should be ● Tomatoes hemoglobin
Avoided ● Garlic ● In the United States, about 1,000
● Chloramphenicol babies are born with sickle cell
● Flutamide Nursing Actions disease each year
● Nalidixic acid 1. Encourage newborn screening ● 70,000-100,000 Americans have
● Nitrofurantoin 2. Educate the parents and significant sickle cell anemia
● Primaquine others about the condition ● 1/365 Black or African-American
● Sulfa drugs 3. Avoid exposure to oxidative stress births
● Aspirin 4. Let the child understand the ● 1/13 Black or African-American
● Paracetamol condition at earliest possible age babies is born with sickle cell train
5. Any signs and symptoms of acute ● Nigeria - 45,000-90,000 babies with
Some Foods That Should be Avoided hemolysis, please consult a doctor sickle cell disease are born each
● Fava beans and other legumes immediately.

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Sickle Cell Disease Pathophysiology
Sickle Cell Disease Signs and Symptoms
Causes - Hypoxic Condition
● High altitude
● Temperature changes
● Severe exercise
● Unpressurized cabin
● Anesthesia – operation
● Infection Severe Hypoxic Condition
● Dehydration
● Inflammation
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● RBM expansion
● Any hypoxic condition Sickle Cell Crisis
● Beta globin abnormal protrusion ● Vaso occlusive crisis
● Hemoglobin will precipitate into gel ● Aplastic crisis
● Hemoglobin structures formed ● Sequestration crisis
fibrous polymer ● Hemolytic crisis
● Hemolysis – sickle cell anemia
● Splenomegaly & hepatomegaly Treatment
● High unconjugated bilirubin ● Hydration
● Lemon yellow color ● Folic acid and penicillin
● Dark stool ● Analgesics
● Bili stones ● Exchange or blood transfusion - iron
● Enlargement of the bones in the face loading?
and skull ● Chelation
● Reticulocytosis ● Hydroxyurea
● Pain attacks ● Arginine
● Infarcts ● Bone marrow or stem cell transplant
Nursing Interventions
● Pain 1. Hydration
● Ulcers 2. Managing pain
● Delayed growth 3. Preventing and managing infections
● Visual problems 4. Vaccinations
● Autosplenectomy 5. Minimizing deficient knowledge
● Frequent infections 6. Promoting coping skills
7. Monitoring and managing potential
complications
● Pain attacks 8. Promoting home and community
● Vaso occlusive attack based care
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9. Stem cell transplant and gene ● Thalassemia minor ● Exjade
therapy ● Thalassemia intermedia ● Desferal
● Thalassemia major
Thalassemia ● Asymptomatic increased – HB A2 & Exjade: Drug Administration
● Autosomal recessive genetic disorder HB F ● Oral Drug
● Thalassemia affects approximately ● Moderate anemia – may require ● DO NOT break, crush, or allow
4.4 of every 10,000 live births episodic blood transfusion patient to swallow whole
throughout the world ● Severe anemia – blood transfusion ○ Dosages <1 gram should be
● African-American, Mediterranean for life dissolved in 100 mL of liquid
countries ○ Dosage >1 gram should be
dissolved in 200 mL of liquid
Alpha Thalassemia ■ Drop tablet into water
● Silent carrier or juice
● Thalassemia trait ■ Stir until completely
● HB H Disease dissolved
● Hemoglobin barts ● Take on an empty stomach at least
● Asymptomatic 30 minutes before food
● Milk anemia ● Take daily at the same time
● Severe anemia (extravascular
hemolysis) Exjade: Recommended Ranges
● Hydrops fetalis ● Initial daily dose is 20 mg/kg/day
Treatment ● Dose should not exceed 30
Treatment ● Folic acid mg/kg/day
● Folic acid (FA) ● Exchange blood transfusion iron
● Exchange or blood transfusion - iron loading Exjade: Advantages
loading ● Chelation ● Once a day oral administration
● Chelation ● Bone marrow, gene therapy or stem ● May be dissolved in water or juice
● Bone marrow or stem cell transplant cell transplant ● Patient compliance

Beta Thalassemia Chelation Therapy Exjade: Disadvantages

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● Lack of long term data
● Extremely expensive
● Increases serum creatinine levels
● Potential to cause:
○ Agranulocytosis
○ Neutropenia
○ Thrombocytopenia
○ Auditory disturbances
○ Ocular disturbances
Desferal: Drug Administration
● May be given intravenously,
subcutaneously, or intramuscularly
Desferal: Recommended Ranges
● IV Administration:
○ 40-50 mg/kg/day over 8-12
hours for 5-7 days per week
● Subcutaneous Administration
○ 1-2 grams over 8-24 hours
● Intramuscular Administration:
○ 0.5-1 gram every day
○ Do not exceed 1 gram daily
Desferal: Advantages
● Long term data available
● May be used to treat acute
iron-overload
Desferal: Disadvantages
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● A minimum of 5 days per week is ● Cardiac evaluation
needed to attain adequate excretion ● Observe administration site for
● Adversely affects skeletal maturation irritation and infection
and growth development ● Explain common side effects
● Orange to red urine ● Instruct patient and family on drug
● Patient compliance administration and equipment
○ Desferal infusion must be
administered over 8-24 hours Nursing Consideration
● Aseptic technique
Desferal: Adverse Effects ● Rotate sites
● Hypotension with to rapid IV ● Encourage patient to increase fluid
infusion intake
● Tachycardia ● Provide support to encourage
● Shock compliance with treatment and
● Acute Renal Failure reporting adverse occurrences
● Observe administration sites for
Complications of Iron Chelation Therapy irritation or infection
● Nausea
● Diarrhea Long-term Follow-up
● Rash ● Liver function test
● Renal insufficiency ● CNS evaluation
● Liver fibrosis ● Liver biopsy
● Growth retardation ● 24 hour urinary iron excretion
● Sensorineural toxicity ● EKG, echocardiogram
● Ocular toxicity ● Audiology evaluation
● Agranulocytosis ● Ophthalmology evaluation
● Endocrine evaluation
Continuing Care
● Audiology evaluation Paroxysmal Nocturnal Hemoglobinuria
● Ophthalmology evaluation ● Acquired intrinsic hemolysis

● Disease of myeloid stem cell –
pancytopenia
● Mutation on X chromosomes – pig a
gene
● Defective CHON on cell membrane
– against complement CHON
● Prone to attack by complement
protein
● RBC will be destroyed in the
circulation
● WBC will be destroyed in the
circulation – severe cases
● Platelets will be destroyed in the
circulation – severe cases
Paroxysmal Nocturnal Hemoglobinuria
Signs and Symptoms
● Chronic hemolysis – RBC
● Increased during the night
● CO2 retain in blood – decreased PH
● Dark urine during the night
● Hemoglobin + nitric oxide
● Esophageal spasm – severe cases
● Difficulty swallowing – severe cases
● Abdominal spasm/pain – severe
cases
● Erectile dysfunction – severe cases
● Chronic hemolysis – WBC
● Frequent infection
● Chronic hemolysis – platelets
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● Thrombotic tendency in different ● DIC (Disseminated Intravascular
parts of the body (cause of death) Hemolysis)
● Liver – abdominal pain, ascites, ● TTP (Thrombocytopenic Thrombotic
hepatomegaly Purpura)
● Brain – headache, stroke ● ITP (Idiopathic Thrombocytopenic
Purpura)
PNH Labs ● HUS (Hemolytic Uremic Syndrome)
● Intravascular anemia
● Increased LDH
● Very low/no haptoglobin
● Flow cytometry
PNH Treatment
● Steroids
● Warfarin
● Blood transfusion
● Anti complement CHON
– chemotherapy
● Bone marrow transplant
Immune Mediated Hemolysis
Macroangiopathic Hemolytic Anemia ● Auto Antibody
(MAHA) ○ Warm antibody hemolytic
● Traumatic damage anemia
● Calcific aortic valve stenosis ○ Cold antibody hemolytic
● Ill fitting prosthetic cardiac valve anemia
● Congenital cardiovascular anomalies ○ Cold antibody agglutination
eg. coarctation of aorta
● March hemoglobinuria Nursing Considerations
● Athletes ● Educate the patient about the disease
 18
● Monitor for signs and symptoms of
anemia
● Blood transfusion
● Instruct about medication (steroids,
chemotherapy)
● Prepare patient for splenectomy

Immune Mediated Hemolysis

ISO Antibody
1. ABO incompatibility
2. RH incompatibility

● 26 weeks AOG
● Within 72 hours postpartum

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Lesson 4 – Bleeding Disorders
Steps to Prevent/Control Bleeding
1. Vasospasm
2. Platelet activity
3. Clotting factor activity
–– coagulation
Thrombotic Thrombocytopenic Purpura
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● Avoid blood thinners
Thrombotic Thrombocytopenic Purpura ● Avoid OTC drugs
Causes ● Monitor platelet count
● Decreased/lack of ADAMTS13 ● Avoid contact sports
enzyme ● Avoid constipation
● Inherited or acquired mutation ● Observe for signs and symptoms of
● Platelet clump together bleeding
● Decrease platelet in the circulation ● WOF signs and symptoms of relapse
● Bleeding under the skin
Disseminated Intravascular Hemolysis
Thrombotic Thrombocytopenic Purpura ● Sudden
Signs and Symptoms ● Septicemia
● Depends on area affected ● OB complications
● Rashes ● Massive tissue damage (burns, SX,
● Brain etc.)
● Heart ● ABO/RH incompatibility
● Kidney ● Liver disease
● Snake venom
Thrombotic Thrombocytopenic Purpura ● Chronic
Treatment ● Cancer
● Inherited – platelet/ffp ● Infections
● Acquired – /plasmapheresis/plasma
exchange Disseminated Intravascular Hemolysis
● Steroids Signs and Symptoms
● Immunosuppressant ● Different presenting symptoms
● IV Ig ● 3 unrelated sites – ear, nose, throat,
● Splenectomy GI, respiratory, skin
● Confusion
Thrombotic Thrombocytopenic Purpura ● Disorientation
Nursing Consideration ● Multi organ failure
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● Wide spread bleeding Detection of Hemophilia ○ <1%
● PT test ● Family history
● Fibrin degradation product – D ● Symptoms People with hemophilia bleed longer, not
dimer ○ Bruising faster.
○ Bleeding with circumcision
Disseminated Intravascular Hemolysis ○ Muscle, joint, or soft tissue Types of Bleeds
Treatment bleeding ● Joint bleeding
● Transfusion ● Hemostatic challenges ● Muscle hemorrhage
● FFP ○ Surgery ● Soft tissue - bruising
● Platelets ○ Dental work ● Life threatening bleeding
● Treat underlying disease ○ Trauma, accidents ● Others - mouth, nose, scrapes, minor
● Reperfusion surgical intervention ● Laboratory testing cuts

Hemophilia U.S. Incidence of Hemophilia Muscle Bleeding

Types of Bleeding Disorders ● Hemophilia A: 20.6 per 100,000 ● Signs and symptoms similar to joint
● Hemophilia A (factor VIII males bleeding
deficiency) ○ Severe: 50-60% ○ Can include tightness
● Hemophilia B (factor IX deficiency) ● Hemophilia B: 5.3 per 100,000 ○ Shiny skin
Von Willebrand Disease males ○ Is very painful
○ Severe: 44% ○ Usually happens in the arms
Inheritance of Hemophilia and legs
● Hemophilia A and B are X-linked Degrees of Severity of Hemophilia ○ Swelling
recessive disorders ● Normal factor VIII or IX level = ○ Significant blood loss can
● Hemophilia is typically expressed in 50-150% happen quickly
males and carried by females ● Mild hemophilia ● Someone with a bleed can feel a
● Severity level is consistent between ○ Factor VIII or IX level = muscle bleed long before anyone
family members 6-50% sees any outward symptoms
● ~30% of cases of hemophilia are ● Moderate hemophilia
new mutations ○ 1-5% Soft Tissue Bleeding
● Severe hemophilia

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● Primarily characterized by bruising
and hematomas (raised bruises)
● Many with hemophilia have bruises
all over their bodies
● Treatment is generally not needed,
ice can help with comfort
Life-Threatening Bleeding
● head/intracranial
○ Nausea, vomiting, headache,
drowsiness, confusion, visual
changes, loss of
consciousness
● Neck and throat
○ Pain, swelling, difficulty
breathing/swallowing
● abdominal/GI
○ Pain, tenderness, swelling,
blood in the stools
● Iliopsoas Muscle
○ Back pain, thigh
tingling/numbness, decreased
hip range of motion
Other Bleeding Episodes
● Mouth bleeding
○ Looks like more than it is, as
it is mixed with saliva
○ Child may vomit
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○ Feces may be black (from
swallowed blood)
● Nose bleeding
○ Sit up, pinch bridge of nose,
cool pack on back of neck
○ If longer than 20 minutes, a
trip to ER may be required
● Scrapes and/or minor cuts
○ Wash, pressure, dressing
○ Call parents if bleeding
persists
Complications of Bleeding
● Flexion contractures
● Join arthritis/arthropathy
● Chronic pain
● Muscle atrophy
● Neurologic impairment
Treatment of Hemophilia
● Replacement of missing clotting
protein
○ On demand
○ Prophylaxis
● DDAVP
● Antifibrinolytic Agents
○ Amicar
● Supportive measures
○ Ice pack
○ Immobilization
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○ Rest
Infusions of Factor Concentrates
● Verify product with physician order
● Reconstitute factor per package
insert
● Infusion rate per package insert or
pharmacy instructions
● Document lot number, expiration
date, time of infusion, and exact dose
given in units
Nursing Considerations
● Factor replacement to be given on
time
● Lab monitoring
● Increase metabolic states will
increase factor requirements
● Factor coverage for invasive
procedures
● Document - infusions, response to
treatment
● Avoid NSAIDS
Von Willebrand Disease
● Erick von Willebrand described a
large family with a severe bleeding
disorder from the Aland Islands in
1926

● Difference in bleeding from classic
hemophilia:
○ Lack of join bleeding
○ Presence of mucosal bleeding
● Proband died of bleeding during 4th
menstrual period
Genetics of VWD
● Usually inherited, though
spontaneous mutations do occur
● Inheritance is usually autosomal
dominant, although subtypes may
have varying inheritance patterns
● Equally passed to males and females
● Inheritance may occur from either
parent
● Gene defect on chromosome 12
Von Willebrand Factor
● A large multimeric protein that
○ Circulates in the blood
○ Stabilizes FVIII
○ Provides the initial link
between the platelet and the
ruptured blood vessel
Types of VWD
● Type 1 (~80%)
○ Lower than normal levels of
VWF
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○ Symptoms usually mild
● Type 2 (~20%)
○ Defect in structure of VWF,
so doesn’t work properly
○ Different subtypes: 2A, 2B,
2N, 2M
○ Symptoms usually moderate
● Type 3 (rare)
○ Very little or no VWF
○ Symptoms are more severe,
including bleeding into
muscles and joints,
sometimes without injury
Testing for VWD
Diagnostic criteria
1. Personal history of excessive
mucocutaneous bleeding
2. Laboratory tests of hemostasis
consistent with VWD
3. Family history of excessive bleeding
Common Bleeding Symptoms
● Easy bruising
● Prolonged bleeding from lacerations
● Epistaxis
● Bleeding from gums
● Menorrhagia
● Post-dental procedural bleeding
● Post-surgical bleeding
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● Excessive postpartum bleeding
● Muscle hematomas (type 3 VWD)
● Hemarthrosis (type 3 VWD)
Testing for VWD
● VWD cannot be diagnosed with
routine blood tests - testing is
complex
● Involves measuring a person’s level
and activity of VWF and FVIII
Treatment for Bleeding Episodes
● Non-medical (RICE)
○ Rest
○ Ice
○ Compression
○ Elevation
● DDAVP
● Oral antifibrinolytics
● Factor replacement with VWF/FVIII
concentrates
Women with VWD
● Women tend to have more symptoms
than men because of menstruation
and childbirth
○ Heavier and/longer menstrual
flow
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○ Girls may have especially
heavy bleeding when they
begin to menstruate
○ Check for anemia regularly
○ Women entering menopause
are at increased risk of
unpredictable and heavy
bleeding
● Pregnancy and delivery
○ A woman with VWD should
see an obstetrician as soon as
she suspects she is pregnant
○ The obstetrician should work
with a bleeding disorders
treatment center to provide
the best care during the
pregnancy and childbirth
○ During pregnancy, VWF
and FVIII levels increase →
protection from bleeding
during delivery
○ After delivery, levels
decrease and bleeding may
occur

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