Module 2: Hematologic Conditions

Question 1

Normal Laboratory References

Answer 1

WBCs: 4.5-11

Platelets: 150-350

Hemoglobin: 13.9-16.3 (males); 12.0-15.0 (females)

Hematocrit: 41-53 (males); 36-46 (females)

Question 2

Hematologic Disorders

Answer 2

Disorders of the hematopoietic system (production and maturation of RBCs, WBCs, and platelets)

Primary hematopoiesis:

1. Hematopoietic cells are committed and developed in the bone marrow
2. Primary disorders occur during cell development

Secondary hematopoiesis:

1. Storage sites: Spleen and lymph
2. Secondary disorders

Common hematologic disorders:

1. Leukemia/lymphoma
2. Anemia — Types: Blood loss, chronic illness, nutritional (iron, B12, or folic acid deficiencies), and sickle cell
3. Thrombocytopenia

Question 3

Assessment of Patients with Hematologic Disorders

Answer 3

Risk factors & PMHX:

1. Genetic tendency
2. Bone marrow depressing meds.
3. Chemical or radiation exposure
4. Infectious disease (especially viral)
5. Nutrition

Suspicious S/S:

1. Bleeding
2. Bruising
3. Pale/cyanotic
4. Fatigue
5. Fever
6. Frequent infections

Physical examination is based upon risks and differential diagnosis

Question 4

Diagnosis of Hematologic Disorders

Answer 4

Complete Blood Count (CBC):

1. H&H, RBC count
2. RBC indices (provide information about the Hgb content and size of RBCs) — differentiates nutritional anemias
3. WBC count
4. Platelets

Extended hematologic studies:

1. Reticulocytes (immature RBCs)
2. Iron studies — detect iron deficiency anemia
3. Nutritional studies — i.e. folate level

Bone marrow biopsy (based on CBC) — it is performed if RBCs, WBCs, and platelets are all abnormal; extremely invasive (addresses leukemia)

Coagulation profile:

1. Prothrombin time (PT)
2. Partial thromboplastin time (PTT)
3. Fibrinogen level

Chemistry tests (indicate hemolysis):

1. LDH
2. Bilirubin

Question 5

Anemia

Answer 5

Inadequate RBC quantity or RBC dysfunction; classified by etiology and cell characteristics

CMs: Decreased oxygen-carrying capacity and tissue perfusion

Contributing factors:

1. Hgb: Amount of heme protein that carries oxygen (best indicator)
2. Hct: Measurement of how much of blood is made up of RBCs (affected by fluid volume)

Question 6

Reticulocytes

Answer 6

Relatively immature precursor to mature RBC

Indicative of:

1. RBC turnover (bone marrow release of immature cells)
2. Recent acute loss of blood cell mass (i.e. bleeding or hemolysis), causing early bone marrow release of reticulocytes

Elevated (>2% of RBC count) with blood loss; hemolysis usually higher

Question 7

Anemias

Answer 7

Types of anemia:
1. Aplastic anemia: bone marrow does not produce any blood cells

2. Nutritional anemia: specific cellular abnormalities directly related to lack of nutrient
3. Hemolytic anemia: inappropriate lysis of RBCs outside normal spleen/liver pathways
4. Bone marrow dysfunction: inadequate stem cells or differentiation in marrow
5. Blood loss
6. Anemia of chronic illness: metabolic depletion of reserve and inability to replenish RBCs

Question 8

Anemia: Signs and Symptoms

Answer 8

CV system (Compensatory mechanisms wrt low oxygen-carrying capability):

1. Tachycardia
2. Full, bounding pulses
3. Cardiac murmurs
4. HTN

CV system (Due to non-effective compensatory mechanisms or volume loss):

1. Weak, thready pulses
2. Hypotension
3. Cool extremities

Neurological system:

1. HA
2. Confusion
3. Cognitive impairment

Respiratory system:

1. Tachypnea
2. Dyspnea

GI/GU:

1. Oliguria
2. Constipation

Other S/S:

1. Acidosis
2. Hypothermia
3. Fatigue
4. Pallor
5. Blood loss
6. Bruising

Question 9

Iron Deficiency Anemia

Answer 9

Iron deficiency is the most prevalent nutritional deficiency in the world

Related to:

1. Chronic blood loss (i.e. Menstrual bleeding, CA, ulcerative colitis, PUD)
2. Insufficient absorption (i.e. Crohn’s disease, celiac disease, PPIs and NSAID use)

“Hallmark” CMs:

1. Pica: compulsion to eat non-food items (often resolved with iron repletion)
2. Glossitis: painful, smooth, red tongue; cracks in corners of the mouth
3. Koilonychias: spooning and thinning nails — due to blood flow abnormalities

Iron supplements are constipators (and can exacerbate anemia) — admin. with ascorbic acid and encourage fluid intake

Question 10

Nutritional Anemia: Nursing Diagnosis & Interventions

Answer 10

Nursing diagnosis of patients with nutritional anemias:

1. Inadequate tissue perfusion
2. Fatigue
3. Activity intolerance

Interventions & education of patients with nutritional anemias:

1. Decrease/minimize blood loss
2. Increase dietary iron (i.e. dark-green leafy greens, meats, iron-fortified cereal/bread)
3. Increase dietary vitamin C (promotes iron absorption)
4. Limit calcium supplements (interfere with iron absorption)
5. Dietary supplements
6. Report S/S of anemia: SOB, increased fatigue, and bleeding

Question 11

Vitamin B12 Anemia

Answer 11

More likely to cause neurological and psychiatric dysfunction (related to demyelination of nerves)

“Hallmark” CMs of vitamin B12 anemia:

1. Paresthesia: numbness and tingling in hands and feet
2. Lhermitte sign: electric shock sensation with neck flexion
3. Depression
4. Impaired taste and balance
5. Visual disturbances
6. Tinnitus

Sources of vitamin B12:

1. Meat
2. Dairy
3. Seafood
4. Eggs
5. B12 supplements (Oral and injection)

Question 12

Folic Acid Anemia

Answer 12

Folate acid insufficiency is associated with:

1. Gastric bypass surgery
2. Whipple procedure (pancreatic CA surgery)
3. Alcoholism — increased risk of pancytopenia (suppressed production of different cell types)
4. Oral contraceptives
5. Metformin
6. Chemotherapy

Neurological symptoms: Confusion or disorientation (common)

Folic acid supplements are inducers of Phenytoin/Dilantin (increased risk of seizure breakthrough)

Question 13

Nursing Care & Management of Patients with Anemia

Answer 13

Nursing care of patients with anemia:

1. Balance physical activity, exercise, and rest
2. Adequate nutritional support and supplemental admin. (i.e. iron, EPO, oxygenation)
3. Blood transfusions

Management of patients with anemia:

1. Ensure adequate safe perfusion
2. IV fluids, blood transfusions, protein/coagulation factors, and crystalloid fluids admin.
3. Remove underlying cause of exacerbation
4. Reduce oxygen demands
5. Bone marrow stimulants (EPO) admin. when Hgb <10 — Monitor for HTN and hyper-coagulability, and ensure adequate ferritin and iron precursors
6. Immunosuppressives admin. if etiology of lysis is autoimmune
7. Blood and marrow transplant (BMT)/Hematopoietic stem cell transplant (HSCT) if there is permanent stem cell damage

Question 14

Blood Transfusion: Administration

Answer 14

Types of blood transfusion:

1. Whole blood
2. Packed RBCs
3. FFP
4. Platelets

Equipment:

1. Normal Saline (always admin. with blood products)
2. Blood tubing with/without filters

Timing:

1. Patient timing
2. Transfusion timing (wide open vs. over 3 hours)
3. RN timing

Pre-Assessment:

1. Lung sounds
2. Skin
3. Vitals

Checking blood (2 nurses):

1. Consent for blood
2. Patient ID
3. Blood type of patient
4. Blood type of product
5. Blood ID number
6. Expiration date
7. Special preparations (neutrophils/irradiated)

Question 15

Blood Transfusion: Signs and Symptoms of Reactions

Answer 15

ALLERGIC RXN:

1. Facial flushing
2. Hives/rash
3. Anxiety
4. Wheezing
5. SOB
6. Hypotension (histamines causes BVs to widen)

FEBRILE RXN:

1. Fever
2. Chills
3. Anxiety
4. HA
5. Tachycardia
6. Tachypnea

HEMOLYTIC RXN:

1. Chest pain
2. Hemoglobulinuria
3. Lower back pain
4. Fever
5. Chills
6. Tachycardia
7. Hypotension
8. Tachypnea

Question 16

Blood Transfusion: Management

Answer 16

Preventing transfusion reactions:

1. Monitoring
2. Med. admin. prior to transfusion — i.e. Tylenol for fever; Benadryl for allergic reactions

Reaction management:

1. Discontinue product and preserve tubing
2. Normal saline admin.
3. New type and crossmatch
4. Coombs test — detects antibodies (identifies hemolytic reaction)
5. Urine

Fluid overload management:

1. Check lung sounds
2. BP
3. Weight gain
4. Exercise intolerance
5. Lasix admin.

Question 17

Sickle Cell Disease

Answer 17

An autosomal recessive (inherited) disease characterized by sickling of RBCs in the presence of hypoxemia due to abnormal Hgb S DNA structure

Sickled cells are longer and more stiff with a very shortened life span (20 days instead of normal 120 days)

Sickled cells easily hemolyze and destroy organs

Sickling of cells is intermittently triggered (there are few symptoms when not in crisis); there is some reversal of symptoms with correction of hypoxemia

Question 18

Sickle Cell Disease: Diagnosis

Answer 18

Sickle cell diagnosis:
1. CBC

2. RBC indices
3. Coagulation profile
4. Chemistry tests
5. Bilirubin
6. Haptoglobin test (binds with heme)
7. Transferrin saturation (binds with iron)
8. Ferritin test (early iron precursor)
9. Hgb electrophoresis: specific diagnosis that shows the presence of abnormal Hgb

Question 19

Sickle Cell Crisis: Signs and Symptoms

Answer 19

Hypoxemia is triggered by:

1. Dehydration
2. Cold temps.
3. Infection
4. Low atmospheric oxygen

Types of sickling crisis:
1. Vaso occlusion (most common) is triggered by hypoxemia; it causes tissue hypoxia and pain — S/S: Cool, cyanotic extremities, splenic infarction, stroke, and MI

2. Aplastic
3. Sequestration: excessive amount of blood becomes trapped in the spleen, causing hypovolemic shock
4. Hemolytic

Question 20

Sickle Cell Disease: Joint Complications

Answer 20

Dactylitis (“Sausage digit” or Hand Foot Syndrome): severe pain that affects the bones of the hands and feet; it is often the first symptom of sickle cell disease between the time of birth and 4 yrs. of age

Swelling is caused by blocked blood circulation; S/S: Extreme pain, tenderness, swelling

Question 21

Sickle Cell Disease: Management

Answer 21

Primary treatment of sickle cell disease:

1. Identify and avoid triggers
2. Prevent hypoxemia
3. Promote healthy lifestyle, nutrition, and sleep
4. Limit sickling episodes

Management of sickle cell disease:

1. Control hypoxemia (oxygen therapy admin.)
2. Control thromboses (fluid admin.)
3. Aggressively manage crisis-induced pain (rapid opioid admin.)
4. Compensate for past hemolysis events
5. Incentive spirometry — prevents pneumonia and atelectasis (due to inability to take deep breath)
6. Supportive care — addresses challenges of exercise, limiting sickling, and chronic illness issues
7. Minimize long-term organ dysfunction

Question 22

Sickle Cell Disease: Counseling

Answer 22

Pre-pregnancy counseling:

1. Planning and anticipation
2. Health insurance
3. Parental testing

Parental counseling:

1. Infant testing
2. Health care planning
3. Development
4. Psychologic challenges

Question 23

Normal Clotting Cascade

Answer 23

Clotting cascade pathway:
1. Clotting stimulus (i.e. Tissue injury, vessel injury, FB in bloodstream)

2. Vasoconstriction (immediate); serotonin release
3. Platelet plug: one of the early phases of establishing a clot (2-5 hours); ADP release
4. Fibrin clot formation (1-3 days); plasminogen activated
5. Fibrinolysis

Question 24

Medications that Commonly Cause Anemia and Thrombocytopenia

Answer 24

Anticoagulants:

1. Warfarin (Coumadin)
2. Heparin
3. Lovenox

Fibrinolytics: Alteplase

Platelet inhibitors: Plavix

NSAIDs

Drugs that interfere with platelet production and function:

1. Antibiotics
2. Thiazide diuretics
3. Quinidine
4. Sulfonamides

Drugs that interfere with clotting factors:

1. Amiodarone
2. Anabolic steroids
3. Warfarin (Coumadin)
4. Heparin

Drugs that decrease vitamin K:

1. Antibiotics
2. Clofibrate

Question 25

Thrombocytopenia

Answer 25

Low blood platelet count; it is not a disease, but a complication or syndrome Common etiologies: 1. Bone marrow disease 2. Chemical exposure (i.e. Insecticides and dry cleaning chemicals) 3. Meds. (i.e. Heparin) 4. Radiation exposure 5. Splenic disease 6. Hypo/hyperthermia 7. Large indwelling venous catheters 8. Hemorrhagic conditions — i.e. Disseminated intravascular coagulation (DIC): over activation of proteins that control blood clotting (can lead to massive bleeding in other places)

Question 26

Assessment of Patients with Platelet Disorders

Answer 26

Patient & FHX: 1. Unusual bleeding with injury 2. Liver disease 3. GI disease 4. Alcohol or drug use 5. Occupation 6. Viral illness 7. Meds. Physical exam: 1. Bruising 2. Jaundice 3. Soft tissue bleeding (i.e. Bruising, petechiae) 4. Mucosal bleeding (i.e. Gum bleeding, hematuria, fecal occult blood) Diagnostic studies: 1. Mild (PLT count <50,000); Moderate (<20,000); Severe (<10,000) 2. Platelet quality — bleeding time

Question 27

Thrombocytopenia: Management

Answer 27

Management of patients with thrombocytopenia: 1. Remove cause of thrombocytopenia 2. Glucocorticoid admin. — temporarily suppresses the body's immune system, slowing platelet destruction 3. Platelet transfusions (more reactions than RBC transfusions) — Fewer donors reduce risk of reactions (ideal if bone marrow transplant is later required) 4. Bone marrow growth factors (i.e. Oprelvekin, neumega) admin. 5. Anti-thrombolytic therapy (i.e. Aminocaproic acid/Amicar) — does not correct platelet abnormality (it is supportive) 6. Immunosuppressives admin. — helpful if etiology of lysis is immunologic 7. Splenectomy (extreme cases)

Question 28

Thrombocytopenia: Nursing Management

Answer 28

Nursing management of patients with thrombocytopenia: 1. Assess all excrement for visual blood 2. Visualize orifices/skin/mucosa for bleeding 3. Reduce risk of bleeding — i.e. Elevating HOB, avoiding constipation, fall precautions, applying pressure after invasive procedures, and avoiding IM injections 4. Med admin. to prevent clot breakdown 5. Platelet transfusions 6. Monitor for excessive blood loss (i.e. BP, tachycardia, pallor, diaphoretic) 7. Minimize automatic BP cuff use