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Test 3 > Nonmalignant Hematological Disorders > Flashcards

Nonmalignant Hematological Disorders Flashcards

1
Q

Q: What is anemia?

A

A condition characterized by lower than normal hemoglobin and fewer than normal erythrocytes.

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2
Q

List 3 different ways to be Anemic.

A
  1. Hypoproliferative (defective production of RBCs)
  2. Hemolytic (increased destruction of RBCs)
  3. Bleeding (blood loss)
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3
Q

Q: What is the result of anemia?

A

Decreased oxygen-carrying capacity of the blood.

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4
Q

Q: What is considered severe anemia?
(give a number)

A

hemoglobin < 6 g/dL

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5
Q

Give 6 significant symptoms of Severe Anemia

A
  1. Pallor or jaundice
  2. Difficulty concentrating
  3. Angina/Heart failure/MI **
  4. Glossitis or smooth tongue
  5. Difficulty swallowing, sore mouth
  6. Bone pain
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6
Q

6 Lab Studies for Anemic Patients

A
  • H&H
  • iron studies
  • B12 levels
  • folate
  • erythropoietin levels
  • bone marrow aspiration
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7
Q

List 4 HYPOproliferative Anemias
(defective RBC production)

A
  1. Iron Deficiency Anemia
  2. Anemia in Renal Disease
  3. Aplastic Anemia
  4. Vitamin B12 or Folate Deficiency (Megaloblastic Anemia)
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8
Q

What happens in Hypoproliferative anemia

A

Bone marrow does not produce enough RBCs

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9
Q

What are the 3 causes for Hypoproliferative Anemia

A
  1. Bone marrow damage from chemicals or medications
  2. Lack of erythropoietin (EPO)
  3. Lack of nutrients – (e.g. iron, vitamin B12, folic acid)
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10
Q

What exactly is Erythropoietin (EPO)

A

hormone produced primarily by the kidneys that stimulates the production of red blood cells in the bone marrow

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11
Q

Most common ANEMIA in the world

A

Iron deficiency anemia

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12
Q

Iron deficiency anemia results from:

List 4

A
  • Not taking in enough iron in the diet
  • Increase in the body’s demand for iron- children/adolescents rapid growth
  • Decrease in absorption of iron- need enough stomach acid
  • Losing iron (blood loss)
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13
Q

S/S that will be seen on pt who is Iron deficient.

List 3

A
  1. Spoon-shaped nails
  2. Pica-abnormal cravings
  3. Restless leg syndrome (RLS) - uncontrollable urge to move the legs
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14
Q

Iron Deficiency Anemia:

A

Spoon-shaped nails

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15
Q

#1 Common cause of Iron Deficiency Anemia in ADULTS is due to

A

Bleeding
Due to:
* menstrual period
* Colon polyps/cancer
* GI bleed from ulcers

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16
Q

Other COMMON cause

A
  1. Pregnancy
  2. Following bariatric surgery /gastrectomy **
  3. Celiac disease- caused by eating gluten: causes inflammation & kills duodenum cells
  4. Inflammatory bowel disease
  5. GERD – taking PPI **
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17
Q

Q: What do decreased hemoglobin (Hgb) and hematocrit (Hct) levels indicate?

A

A: They indicate the presence of anemia but do NOT provide the cause.

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18
Q

Q: What additional testing is needed to determine the cause of anemia?

A
  • The patient will need blood work, specifically iron studies
    -This determines if a lack of iron is the cause of their anemia.
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19
Q

List 4 Iron Studies

A
  1. Serum Iron levels -Measures the amount of iron in the blood
  2. Percent transferrin saturation- Measures how many sites on the transferrin are occupied by iron
  3. Total iron binding capacity (transferrin levels)
    Measures the amount of transferrin – the body recognizes when iron levels are low and starts producing more transferrin
  4. Ferritin levels- A blood protein that contains iron– low ferritin indicates the body’s iron stores are low
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20
Q

In Iron Deficiency:

What iron studies (blood work) will be HIGH?

A

Total iron binding capacity (transferrin levels)

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21
Q

In Iron Deficiency:

What iron studies (blood work) will be LOW?

A
  1. Serum Iron levels
  2. Percent transferrin saturation
  3. Ferritin levels
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22
Q

What is the primary mode of treatment for Iron Deficiency anemia?

A

oral iron supplements

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23
Q

How long does it typically take for hemoglobin (Hgb) levels to increase when taking Iron supplements (oral)?

A

a few weeks.

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24
Q

How long does it typically take for anemia to be corrected when taking oral iron supplements?

A

few months- continue taking 6-12 months

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25
Q

Instructions when taking Oral Iron Supplements.

A
  1. Take on empty stomach (1 hr before or 2 hrs after meal) - best absorbed in ACIDIC ENVIRONMENT
  2. Take with orange juice or other source of vitamin C
    -Increase foods rich in vitamin C to enhance absorption
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26
Q

2 main common side effects of Iron supplements

A
  1. Stools often appear black, may have constipation, cramping, nausea
  2. Liquid iron may stain teeth
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27
Q

When might a patient need to take iron supplements with food, and what is the impact on absorption?

A
  • If they experience GI discomfort.
  • This can lead to decreased absorption, resulting in a longer time to replenish iron stores.
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28
Q

Q: How can sustained-release iron supplements affect GI side effects?

A

May help decrease GI side effects compared to standard formulations, providing a gentler option for patients.

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29
Q

Q: What precautions should be taken when using liquid iron supplements to prevent tooth staining?

A
  • drink it with a straw
  • rinse the mouth afterward to minimize staining

(liquid iron is undiluted=stronger)

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30
Q

Q: What is the primary approach to managing iron deficiency anemia?

A

treat the underlying cause

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31
Q

Q: What are the two types of iron replacement therapy?

A

oral and parenteral (injected).

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32
Q

Q: What are the 2 routes of administration for parenteral iron supplements?

A
  1. intramuscular (IM) injection
  2. intravenously (IV)- risk of an allergic reaction, and the patient should be monitored accordingly.
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33
Q

Q: What precautions should be taken when administering intramuscular (IM) iron solutions?

A
  • may stain the skin
  • therefore, separate needles should be used for withdrawing the solution and for injecting the medication.
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34
Q

2nd type of Hypoproliferate anemia:

Occurs due to reduced production of erythropoietin hormone (EPO)

A

Anemia in Renal disease

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35
Q

Whats the main job of erythropoietin hormone (EPO)

A

is the hormone that signals the bone marrow to produce RBCs

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36
Q

Renal Disease Anemia is more sever in what type of patients.

A

patients with BOTH chronic kidney disease (CKD) and diabetes

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37
Q

3 MAIN troubling symptoms of Renal Disease Anemia

A
  • Increased cardiac output
  • Reduced oxygen utilization
  • Decreased libido
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38
Q

3RD type of Hypoproliferate anemia:

Rare, life-threatening disease – caused by a decrease or damage to bone marrow stem cells = bone marrow failure

A

Aplastic anemia
“not forming”

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39
Q

How exactly does bone marrow fail in Aplastic anemia

A

Body’s T-cells attack bone marrow – bone marrow is replaced with FAT

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40
Q

What is pancytopenia

A

ALL blood cells are decreased
- Aplastic anemia should be called pancytopenia

41
Q

Q: list 4 S/S of aplastic anemia

A

All the usual signs of anemia PLUS
* bleeding **
* infection **
* cardiac arrhythmias
* heart failure

42
Q

7 Treatments for Aplastic Anemia

A
  1. Immediate cessation of any medications/chemicals that may have triggered aplastic anemia.
  2. Stem Cell Transplant
  3. Immunosuppressive therapy- prevent T-cells (lymphocytes) from destroying stem cells
  4. Eltrombopag (Promacta) **
  5. Eryhtropoietin (EPO)
  6. Transfusion PRBCs
  7. Treat infections

know

43
Q

Aplastic Anemia:

Immunosuppresive therapy will include a triple medication treatement.

What 3 meds are used

A
  1. Cyclosporoine
  2. Antithymocyte Globulin (ATG): suppresses T-lymphocytes
  3. Corticosteroids

know

44
Q

4th type of Hypoproliferate anemia:

  • Characterized by very large RBCs (MCV very high)
  • RBCs inner contents NOT completely developed – causes the bone marrow to produce fewer cells and cells may die earlier than 120-day life expectancy
A

B12 or Folic Acid Deficiency
(Megaloblastic anemias)

45
Q

Q: What are the 2 most common causes of Megaloblastic anemia?

A
  1. folic acid (folate) deficiency
  2. or Vitamin B12 (cobalamin) deficiency
46
Q

Q: How does a deficiency of intrinsic factor contribute to megaloblastic anemia?

A

deficiency of intrinsic factor leads to decreased absorption of vitamin B12
- this is called pernicious anemia- a type of megaloblastic anemia

47
Q

Lab test for Intrinsic Factor

A

Intrinsic antibody test

48
Q

What does a POSITIVE Intrinsic Antibody test indicate?

A

indicates antibodies are present and are interfering with the binding of B12, preventing absorption

49
Q

Q: How long do symptoms of megaloblastic anemia typically develop?

A
  • Symptoms often develop over MONTHS, allowing the body to compensate for the gradual decrease in red blood cells.
  • As a result, symptoms may NOT appear until the anemia is severe
50
Q

S/S for Megaloblastic anemia

A

All the usual signs of anemia PLUS
* Numbness or tingling in hands and feet (peripheral neuropathy) **

know

51
Q

Folic Acid Deficiency occurs with

A
  1. Alcohol abuse - alcohol ingestion increases folic acid requirements
  2. Pregnancy
52
Q

Vitamin B12 Deficiency occurs with

A
  1. Strict VEGAN diet- due to no meat or dairy
  2. Impaired absorption from GI tract - most common
  3. Absence of intrinsic factor (Pernicious Anemia)
53
Q

Megaloblastic Anemia requires special assessment of

A

Neurologic- close attention to gait and stability with ambulation

know

54
Q

How is vitamin B12 administered in the treatment of pernicious anemia?

2 options

A

B12 injections or nasal spray

55
Q

2 types of Hemolytic Anemias
(caused by erythrocyte destruction)

A
  1. Thalassemia
  2. Sickle Cell Disease
56
Q

1st type under Hemolytic Anemias:

Genetic disorder caused by mutated genes that are responsible for making hemoglobin

A

Thalasssemia- Intrinsic Cause

57
Q

Thalassemia:

2 globin chains in hemoglobin are essential for the formation of hemoglobin

What are they

A
  1. Alpha α-Thalassemia
  2. Beta β-Thalassemia
58
Q

Q: What happens in Thalassemia anemia regarding alpha and beta globin chains?

A
  • either the alpha or beta globin chains are not being produced adequately.
  • This deficiency leads to an imbalance in hemoglobin production
59
Q

Q: In which populations is thalassemia commonly seen?

A

Meditterranean sea population

60
Q

There are 2 forms of Thalassemia

A
  1. Thalassemia Minor: milder form, typically has one mutated gene affecting either the alpha or beta globin chain
  2. Thalassemia Major: severe form; resulting from two mutated genes (one from each parent)
61
Q

2 main S/S for Thalassemia Minor

A
  • Asymptomatic
  • Microcytic, hypochromic anemia- small RBCs, low Hgb
62
Q

5 S/S of Thalassemia Major

A
  • Physical/mental growth retardation
  • Jaundice
  • Splenomegaly, hepatomegaly, cardiomyopathy
  • Increased iron levels **
  • Bone deformities - especially in face and skull
    -Bone marrow expands causing bones to widen
63
Q

Tx for Thalassemia Minor

A

No treatment- can usually live a normal life
- body adapts to reduction of normal Hgb

64
Q

4 Treatments for Thalassemia Major

A
  1. Blood transfusions: frequent blood transfusions to keep Hgb app. 10 g/dL
  2. Chelating agents - subcutaneous infusion of deferoxamine
  3. Splenectomy- removal of spleen
  4. Hematopoietic stem cell transplantation (HSCT)

-

65
Q

Review:

What are Chelating agents

A

bind and remove excess metals or minerals from the body, often used to treat metal poisoning or iron overload.

66
Q

Why is Splenectomy an option for Thalassemia Major

A
  • to stop the spleen from trapping too many red blood cells (RBCs)
  • the spleen can get bigger and hold onto a lot of abnormal RBCs, making anemia worse.
  • Removing the spleen can help improve blood counts and lessen anemia
67
Q

The ONLY cure for Thalassemia Major

A

Hematopoietic stem cell transplantation (HSCT)

68
Q

2nd type under Hemolytic Anemias:

  • Genetic blood disorder, inherit two copies of a mutated gene (one from each parent)
  • RBCs become rigid and shaped like a crescent or sickle, instead of being round and flexible
A

Sickle cell disease

69
Q

There are 2 types of Sickle Cell Disease

A
  1. Sickle Cell Anemia
  2. . Sickle Cell Trait
70
Q

The #1 S/S of Sickle Cell Disease

A

PAIN!!!- may need large doses of continuous opioids

KNOW

71
Q

Complications of Sickle Cell Dz have to do mostly with

A

blood clots.

  • Pulmonary infarctions
  • Retinal vessel obstruction
  • Renal failure
  • PE or stroke
  • Osteoporosis/osteosclerosis
72
Q

Care for Sickle Cell Dz includes

A
  • PAIN RELIEF **
  • Education
  • O2- no smoking
  • Vigilant assessment of respiratory system
73
Q

The ONLY med with some clinical benefit for Sickle Cell Disease

A

Hydroxyurea
* reduce the number of painful crises caused by disease and reduces the need for blood transfusions

know

74
Q

Another treatment choice for Sickle Cell Dz

A

Hematopoietic stem cell transplantation (HSCT)

75
Q

4 types of Hemostatic Anemia
(impaired blood clotting (hemostasis))

A
  • Thrombocytopenia
  • Disseminated intravascular coagulation (DIC)
  • Hemophilia
  • von Willebrand Disease
76
Q

1st type of Hemostatic Anemia

condition characterized by a low platelet count in the blood (below 150,000)

A

thrombocytopenia

77
Q

2 Main S/S of Thrombocytopenia

A
  1. Asymptomatic
  2. Bleeding
78
Q

Caring for Thrombocytopenic Patients

A
  1. Prevent or control hemorrhage – SAFETY
  2. Education: blowing nose, no bending with head lower than waist, use electric razor, no invasive procedures.
79
Q

2nd type of Hemostatic Anemia

serious condition characterized by widespread activation of the clotting cascade, leading to the formation of small blood clots throughout the blood vessels and bleeding

A

Disseminated Intravascular Coagulation (DIC)

80
Q

Q: Does Disseminated Intravascular Coagulation (DIC) occur on its own?

A

No! Usually occurs as secondary response to another underlying condition, such as severe infection, malignancy, trauma, or complications during pregnancy.

81
Q

Q: How does Disseminated Intravascular Coagulation (DIC) lead to organ failure?

A
  • excessive clot formation can obstruct small blood vessels, reducing blood flow to organs.
  • This impaired circulation can result in organ damage and ultimately lead to organ failure due to insufficient oxygen and nutrients reaching the tissues.
82
Q

DIC:

Over time clotting factors are consumed and ____ then occurs

A

bleeding

83
Q

How do we treat Disseminated intravascular coagulation (DIC)

A

treat underlying cause – may reverse DIC

84
Q

What blood products may be necesary for Disseminated intravascular coagulation (DIC) while treating disorder?

A

Platelets, cryoprecipitate, Frozen Fresh Plasma

85
Q

4 Nursing priorities for Disseminated intravascular coagulation (DIC)

A
  • Monitor VS closely- including Neurological checks
  • recognize s/sx of DIC
  • patient safety
  • Bleeding precautions!!!
86
Q

What medications should be avoided for Patients with Disseminated Intravascular Coagulation (DIC)

A
  • aspirin
  • NSAIDS
  • beta-lactam antibiotics
87
Q

3rd type of Hemostatic Anemia:

  • Genetic (inherited) bleeding disorder characterized by the Ability of blood to clot is severely reduced
  • Can be severe and occur with minimal trauma
A

Hemophilia

88
Q

2 types of Hemophilia

A
  1. Hemophilia A – defective factor VIII
  2. Hemophilia B – defective factor IX
89
Q

In HEMOPHILIA, About 75% of bleeding occurs in the ___.

A

joints

90
Q

Q: How is hemophilia treated with clotting factor replacement?

3 treatments available

A

Treatment for hemophilia involves replacing the defective clotting factor through:

  1. Recombinant Clotting Factor Concentrates: These are man-made factors that do not require transfusion consent
  2. Plasma-Derived Factor VIII and von Willebrand Factor: These are derived from donated human plasma
  3. Prophylactic Administration: Factors may be given preventively before traumatic procedures to minimize the risk of bleeding.
91
Q

Q: How often do children with hemophilia typically receive prophylactic treatment, and why?

A

3 to 4 times a week to minimize the risk of joint complications

92
Q

What ANTIBODY med is given to patients with HEMOPHILIA-A?

A

Emicizumab- prevents bleeding

Memory: “Emicizumab is the Eight factor” (hemophilia-A)

93
Q

Nursing Management for Hemophilia

A
  • Extensive education on activity restrictions to reduce risk of bleeding
  • Teach how to administer factor concentrate at home at earliest sign of bleeding
  • Instruct to avoid aspirin, NSAIDS, alcohol, nettle, chamomile, alfalfa
  • Help families develop written emergency plan in case of bleeding
  • Arrange genetic counseling if needed

know

94
Q

Q: What symptoms are commonly associated with von Willebrand disease (vWD)?

A
  • Frequent nosebleeds
  • Easy bruising
  • Heavy menstrual bleeding
  • Prolonged bleeding after injuries or surgeries
94
Q

4th type of Hemolitic Anemia:

Genetic (inherited) bleeding disorder caused by a deficiency or dysfunction of von Willebrand factor (vWF), a protein essential for blood clotting

A

von willebrand disease (vWD)

95
Q

Goal of treatment for von Willebrand Disease (vWD)

A

REPLACE deficient protein at time of spontaneous bleeding or prior to invasive procedures

know

96
Q

What is the medication is often used to prevent bleeding associated with dental and surgical procedures

A

Desmopressin

97
Q

von willebrand disease (vWD):

Platelet transfusion is done if

A

there is significant bleeding

98
Q

What Blood Product can be given for von willebrand disease (vWD)

A

Cryoprecipitate (contains vWF and factor VIII) in emergency situations

Test 3 (7 decks)

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