4.1 Hematopoiesis Review and Disorders Flashcards

1
Q

Functions of the Hematologic System

A
  • Transportation of nutrients and oxygen. Transporting of bodily waste. Transporting of secretory substances within the body
  • Regulation/maintenance of body temperature, pH, electrolytes and toxins.
  • Protection such as physical barriers and immune function
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2
Q

Body Composition

A
  • 55% plasma
  • 40% RBC
  • Small amounts of WBC, platelets, etc
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3
Q

Hematopoiesis

A
  • Creation of RBC’s
  • Begins with hematopoietic stem cells in the bone marrow
  • A committed hematopoietic cell splits into myeloid line or the lymphoid line
  • The myeloid line becomes RBC’s and some WBC’s
  • The lymphoid line becomes T and B cells.
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4
Q

Hemostasis

A

Vascular Phase - Blood vessel is damaged

Platelet Phase - Platelets are attracted, aggregate and adhere. This causes a blood clot (which eventually gets re-absorbed)

Coagulation Phase (Intrinsic and Extrinsic phase can be triggered at the same time)
- Intrinsic Phase - Activated through exposed endothelial collagen
- Extrinsic Phase - Released from endothelial cells caused by external damage. Activated via tissue factor.

Common pieces between intrinsic and extrinsic pathways
- Prothrombin is converted to thrombin which acts on fibrinogen and converts it to fibrin.
- Fibrin is what makes up a blood clot.

MANY CLOTTING FACTORS COME FROM THE LIVER
- People with liver disease may have clotting issues

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5
Q

Hematologic Health Assessment

A
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6
Q

Physical

A
  • Symptoms such as fatigue, weakness, malaise, pain
  • Nutrition
  • Medication
  • Cardiac/GI assessment
  • Blood loss
  • Easy bruising
  • Neurological assessment
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7
Q

Diagnostic

A
  • Hemoglobin/Hematocrit
  • Reticulocyte Count (immature RBCs)
  • RBC indices (size shape and quality of rbc’s)
  • WBC and differential (type and amount of WBC in blood)
  • Platelets
  • Iron
  • Vitamin B12
  • Folate
  • Haptoglobin and Erythropoietin levels
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8
Q

Diagnostics (cont)

A

MVC (Mean Corpus Volume) - 80-100 fl/cell
- Size of RBC (microcytic or macrocytic)

MCH (Mean Corpuscular Hemoglobin)
- Amount of Hgb in each RBC (by weight)

MCHC (Mean Corpuscular Hemoglobin Concentration) - 31-35 g/dL
- Concentration of Hgb in each RBC (color of cell)
- Pale = Hypochromic
- Darker = Hyperchromic

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9
Q

Bone Marrow Aspiration and Biopsy

A
  • Diagnostic evaluation of hematologic function
  • Aspiration of bone marrow for lab evaluation

Intraosseous Infusion
- Used when failure to insert IV into a patient
- Needle can be placed through the bone to provide fluids, meds, and blood.

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10
Q

Anemia

A
  • Low erythrocytes and hemoglobin
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11
Q

Hypoproliferative Anemia

A

Caused by
- Iron, B12, Folate deficiency
- Decreased erythropoietin production
- Cancer

Types of Hypoproliferative anemia
- Iron deficiency Anemia
- Anemia in Renal Disease
- Anemia of Inflammation
- Aplastic Anemia
- Megaloblastic Anemia (very large RBC’s which causes fewer production)
- Folic Acid Deficient Anemia
- Vitamin B Deficient Anemia

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12
Q

Hemolytic Anemia

A
  • Excess destruction of RBC’s

Caused by
- Altered erythropoiesis
- Hypersplenism
- Drug induced
- Autoimmune processes
- Mechanical Heart Valves

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13
Q

Manifestations of Anemia

A
  • ANEMIA IS NOT A DISEASE IT IS AN ALTERED BODILY FUNCTION
  • Fatigue, weakness, malaise
  • Pallor/Jaundice
  • Cardiac/Respiratory issues
  • Tongue/Nail Changes
  • Angular Cheilosis (breakdown of corners of mouth)
  • Pica (eating non-food items)
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14
Q

Values

A

RBC Count
Male - 4.2 - 5.4 x 10^6
Female - 3.6 - 5.0 x 10^6

Hematocrit
Male - 40-50%
Female - 37-47%

Hemoglobin
Male - 14-16.5 g/dL
Female - 12-15 g/dL

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15
Q

Sickle Cell Disease (SCD)

A
  • Characterized by sickle-cell RBC’s
  • Recessive disorder
  • Caused by point of mutation where valine (amino acid) is substituted for glutamic acid which causes abnormal hemoglobin (Hemoglobin S)
  • Typically patients are asymptomatic for their first year of life.
  • Anemia is sporadic in nature (comes and goes)
  • RBC’s elongate and take a sickle form when presence of low oxygen or dehydration

SICKLE CELL ANEMIA (more severe than SCD)
- Severe, chronic hemolytic anemia that causes erythrocytes to stiffen and elongate.

At risk populations of SCA
- Parents with the trait/disease
- African American

SICKLE CELL TRAIT
- Carriers of SCD (less than 50% hemoglobin in erythrocytes are HbS)

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16
Q

RBC’s

A
  • Red blood cells are shaped as discs so they can carry more oxygen, increase surface area, and compressible to fit through blood vessels of any size.
  • Sickle cells may entangle and damage endothelial lining of vessels which may lead to clot formation
17
Q

SCD Crisis

A

Vaso-Occlusive
- Occlusion of vessels leading to ischemia and infarction of tissue. Can be painful due to lactic acid buildup in tissue from ischemia
- Causes include dehydration, hypoxia, infection/illness, cold, physical exertion, acidosis, stress.

Splenic Sequestration
- RBC’s clog the spleen which causes splenomegaly. Enlarged spleen is very fragile

Aplastic Crisis
- Large numbers of RBC’s destroyed in the spleen overwhelm bone marrow response to replace RBC’s causing bone marrow to shut down. This causes severe drops in HgB. Manifestations include severe lethargy, malaise and desaturation.

18
Q

Assessment of Crisis

A

Vaso-occlusive Crisis
- Assess pain, extremities and organ function
- Occluded vessels affect kidney blood supply which alters renal function
- Assess for chest pain because this could mean occlusion of heart (ischemia)
- Assess for enlarged spleen
- Assess LOC for cerebral perfusion

Priapism - Painful erect penis due to clogging of blood vessels that cannot empty
Avascular Necrosis - Interruption of blood flow causes bone damage and joint pain (especially femoral head)

  • Look for your patient symptoms and how it affects their life. Vaso-occlusive crisis is the most common.
19
Q

Diagnostics for SCD

A
  • Blood Smear
  • Electrophoresis (looks at hemoglobin)
  • DNA analysis

CBC
- Decreased RBCs
- Increased WBC and Platelets
- Decrease in ESR (Erythrocyte Sedimentation Rate)

CBC DURING CRISIS
- Decreased RBCs
- Increased WBC and Platelets
- Decreased ESR
- DECREASED HEMOGLOBIN

20
Q

Manifestations of SCD

A

SCD is a Hemolytic Anemia

  • Every single body system is affected
  • Hematocrit is 18-30%
  • Hyperbilirubinemia (risk of jaundice and gallstones is high)

Vaso-occlusive Crisis
- Is a result of hypoxia
- Can affect abdomen, chest, bones, joints
- If unrelieved, causes organ damage and infarction
- Acute Pain
- Splenomegaly

Skin Changes
- Stasis ulcers due to impaired perfusion

21
Q

Complications of SCD

A
  • Hypoxia
  • Ischemia
  • Infection
  • Dehydration
  • Stroke
  • CVA (Cerebrovascular Accident)
  • Kidney Disease
  • HF
  • Impotence
22
Q

Prevention of Sickling of RBC’s

A
  • Adequate Oxygen
  • Hemodilution (intentionally removing RBC’s to reduce hematocrit)

Sports Activities
- Patients need to prepare themselves when engaging in sports. They should pre-oxygenate and increase fluid intake if undergoing hemodilution

INTERVENTIONS FOR CRISIS
Aplastic Crisis - Blood transfusion, splenic sequestration, volume expanders
Hemolytic Crisis - Hydration and prevention of waste buildup in the blood
Vaso-occlusive - Pain management

KEY IS TO PREVENT CRISIS BY PREVENTING DEHYDRATION AND STAGNATION OF BLOOD

23
Q

Treatments

A

Hematopoietic Stem Cell Transplant
- Can cure SCD
- Low usage due to lack of compatible donors or severe organ damage is already present in the patient.

Hydroxyurea (medication)
- Chemotherapy that increases fetal hemoglobin which decreases formation of sickle cells.
- Unknown if it can reverse organ damage
- Side effects include suppression of leukocyte formation, teratogenesis, potential for malignancy later in life.

  • Folic Acid replacement to maintain supply for erythropoiesis