Nonmalignant Hematological Disorders Flashcards
(99 cards)
1
Q
Q: What is anemia?
A
A condition characterized by lower than normal hemoglobin and fewer than normal erythrocytes.
2
Q
List 3 different ways to be Anemic.
A
- Hypoproliferative (defective production of RBCs)
- Hemolytic (increased destruction of RBCs)
- Bleeding (blood loss)
3
Q
Q: What is the result of anemia?
A
Decreased oxygen-carrying capacity of the blood.
4
Q
Q: What is considered severe anemia?
(give a number)
A
hemoglobin < 6 g/dL
5
Q
Give 6 significant symptoms of Severe Anemia
A
- Pallor or jaundice
- Difficulty concentrating
- Angina/Heart failure/MI **
- Glossitis or smooth tongue
- Difficulty swallowing, sore mouth
- Bone pain
6
Q
6 Lab Studies for Anemic Patients
A
- H&H
- iron studies
- B12 levels
- folate
- erythropoietin levels
- bone marrow aspiration
7
Q
List 4 HYPOproliferative Anemias
(defective RBC production)
A
- Iron Deficiency Anemia
- Anemia in Renal Disease
- Aplastic Anemia
- Vitamin B12 or Folate Deficiency (Megaloblastic Anemia)
8
Q
What happens in Hypoproliferative anemia
A
Bone marrow does not produce enough RBCs
9
Q
What are the 3 causes for Hypoproliferative Anemia
A
- Bone marrow damage from chemicals or medications
- Lack of erythropoietin (EPO)
- Lack of nutrients – (e.g. iron, vitamin B12, folic acid)
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
11
Q
Most common ANEMIA in the world
A
Iron deficiency anemia
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)
13
Q
S/S that will be seen on pt who is Iron deficient.
List 3
A
- Spoon-shaped nails
- Pica-abnormal cravings
- Restless leg syndrome (RLS) - uncontrollable urge to move the legs
14
Q
Iron Deficiency Anemia:
A
Spoon-shaped nails
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
16
Q
Other COMMON cause
A
- Pregnancy
- Following bariatric surgery /gastrectomy **
- Celiac disease- caused by eating gluten: causes inflammation & kills duodenum cells
- Inflammatory bowel disease
- GERD – taking PPI **
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.
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.
19
Q
List 4 Iron Studies
A
- Serum Iron levels -Measures the amount of iron in the blood
- Percent transferrin saturation- Measures how many sites on the transferrin are occupied by iron
-
Total iron binding capacity (transferrin levels)
Measures the amount of transferrin – the body recognizes when iron levels are low and starts producing more transferrin - Ferritin levels- A blood protein that contains iron– low ferritin indicates the body’s iron stores are low
20
Q
In Iron Deficiency:
What iron studies (blood work) will be HIGH?
A
Total iron binding capacity (transferrin levels)
21
Q
In Iron Deficiency:
What iron studies (blood work) will be LOW?
A
- Serum Iron levels
- Percent transferrin saturation
- Ferritin levels
22
Q
What is the primary mode of treatment for Iron Deficiency anemia?
A
oral iron supplements
23
Q
How long does it typically take for hemoglobin (Hgb) levels to increase when taking Iron supplements (oral)?
A
a few weeks.
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
25
**Instructions** when taking **Oral Iron Supplements**.
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
26
2 main common side effects of **Iron supplements**
1. **Stools often appear black**, may have constipation, cramping, nausea
2. **Liquid iron** may stain teeth
27
When might a patient need to take iron supplements with food, and what is the impact on absorption?
* If they experience GI discomfort.
* This can lead to **decreased absorption**, resulting in a **longer time to replenish iron stores.**
28
Q: How can **sustained-release** iron supplements affect GI side effects?
May **help decrease GI side effects** compared to standard formulations, **providing a gentler option for patients.**
29
Q: What precautions should be taken when using liquid iron supplements to prevent tooth staining?
* drink it with a straw
* rinse the mouth afterward to minimize staining
(liquid iron is undiluted=stronger)
30
Q: What is the primary approach to managing iron deficiency anemia?
treat the underlying cause
31
Q: What are the two types of iron replacement therapy?
oral and parenteral (injected).
32
Q: What are the 2 routes of administration for parenteral iron supplements?
1. intramuscular (IM) injection
2. intravenously (IV)- *risk of an allergic reaction, and the patient should be monitored accordingly.*
33
Q: What precautions should be taken when administering intramuscular (IM) iron solutions?
* may stain the skin
* therefore, separate needles should be used for withdrawing the solution and for injecting the medication.
34
*2nd type of **Hypoproliferate** anemia*:
Occurs due to **reduced production of erythropoietin hormone (EPO)**
Anemia in Renal disease
35
Whats the main job of erythropoietin hormone (EPO)
is the hormone that **signals the bone marrow to produce RBCs**
36
**Renal Disease Anemia** is more sever in what type of patients.
patients with BOTH **chronic kidney disease (CKD)** and **diabetes**
37
3 MAIN troubling symptoms of Renal Disease Anemia
* Increased cardiac output
* Reduced oxygen utilization
* Decreased libido
38
*3RD type of **Hypoproliferate** anemia*:
Rare, life-threatening disease – caused by a **decrease or damage to bone marrow stem cells** = bone marrow failure
Aplastic anemia
"*not forming*"
39
How exactly does bone marrow fail in Aplastic anemia
Body’s T-cells attack bone marrow – bone marrow is replaced with FAT
40
What is pancytopenia
**ALL** blood cells are **decreased**
- Aplastic anemia should be called pancytopenia
41
Q: list 4 S/S of **aplastic anemia**
All the usual signs of anemia **PLUS**
* **bleeding** **
* **infection** **
* cardiac arrhythmias
* heart failure
42
7 Treatments for Aplastic Anemia
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
*Aplastic Anemia*:
Immunosuppresive therapy will include a triple medication treatement.
What 3 meds are used
1. Cyclosporoine
2. Antithymocyte Globulin (ATG): *suppresses T-lymphocytes*
3. Corticosteroids
**know**
44
*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*
B12 or Folic Acid Deficiency
(**Megaloblastic anemias**)
45
Q: What are the 2 most common causes of **Megaloblastic anemia**?
1. **folic acid (folate)** deficiency
2. or **Vitamin B12 (cobalamin)** deficiency
46
Q: How does a **deficiency of intrinsic factor** contribute to **megaloblastic anemia**?
deficiency of intrinsic factor leads to **decreased absorption of vitamin B12**
- this is called **pernicious anemia**- *a type of megaloblastic anemia*
47
Lab test for Intrinsic Factor
Intrinsic **antibody** test
48
What does a **POSITIVE Intrinsic Antibody test** indicate?
indicates **antibodies are present** and are interfering with the binding of B12, **preventing absorption**
49
Q: How long do symptoms of **megaloblastic anemia** typically develop?
* 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
S/S for Megaloblastic anemia
All the usual signs of anemia **PLUS**
* **Numbness or tingling in hands and feet** (peripheral neuropathy) **
**know**
51
Folic Acid Deficiency occurs with
1. **Alcohol abuse** - alcohol ingestion increases folic acid requirements
2. Pregnancy
52
Vitamin B12 Deficiency occurs with
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
**Megaloblastic Anemia** requires special assessment of
**Neurologic**- close attention to gait and stability with ambulation
**know**
54
How is vitamin B12 **administered** in the treatment of pernicious anemia?
2 options
B12 injections or nasal spray
55
2 types of **Hemolytic Anemias**
(*caused by erythrocyte destruction*)
1. Thalassemia
2. Sickle Cell Disease
56
*1st type under Hemolytic Anemias*:
**Genetic disorder** caused by mutated genes that are responsible for **making hemoglobin**
Thalasssemia- Intrinsic Cause
57
*Thalassemia*:
**2 globin chains in hemoglobin** are essential for the formation of hemoglobin
What are they
1. Alpha α-Thalassemia
2. Beta β-Thalassemia
58
Q: What happens in Thalassemia anemia regarding **alpha and beta globin chains**?
* **either the alpha or beta globin chains are not being produced adequately**.
* This deficiency leads to an imbalance in hemoglobin production
59
Q: In which populations is thalassemia commonly seen?
Meditterranean sea population
60
There are 2 forms of Thalassemia
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
**2** main S/S for **Thalassemia Minor**
* Asymptomatic
* Microcytic, hypochromic anemia- *small RBCs, low Hgb*
62
**5** S/S of **Thalassemia Major**
* 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
Tx for Thalassemia **Minor**
No treatment- can usually live a normal life
- body adapts to reduction of normal Hgb
64
4 Treatments for Thalassemia **Major**
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
Review:
What are Chelating agents
bind and remove excess metals or minerals from the body, often used to treat metal poisoning or **iron overload.**
66
Why is Splenectomy an option for Thalassemia Major
* 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
The ONLY cure for Thalassemia Major
Hematopoietic stem cell transplantation (HSCT)
68
*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
Sickle cell disease
69
There are 2 types of Sickle Cell Disease
1. Sickle Cell **Anemia**
2. . Sickle Cell **Trait**
70
The **#1 S/S** of **Sickle Cell Disease**
PAIN!!!- *may need large doses of continuous opioids*
**KNOW**
71
Complications of Sickle Cell Dz have to do mostly with
**blood clots.**
* Pulmonary infarctions
* Retinal vessel obstruction
* Renal failure
* PE or stroke
* Osteoporosis/osteosclerosis
72
Care for Sickle Cell Dz includes
* PAIN RELIEF **
* Education
* O2- no smoking
* Vigilant assessment of **respiratory system**
73
The ONLY med with some clinical benefit for Sickle Cell Disease
Hydroxyurea
* reduce the number of painful crises caused by disease and reduces the need for blood transfusions
**know**
74
Another treatment choice for Sickle Cell Dz
Hematopoietic stem cell transplantation (HSCT)
75
4 types of Hemostatic Anemia
(*impaired blood clotting (hemostasis)*)
* Thrombocytopenia
* Disseminated intravascular coagulation (DIC)
* Hemophilia
* von Willebrand Disease
76
*1st type of Hemostatic Anemia*
condition characterized by a low **platelet** count in the blood (below 150,000)
thrombocytopenia
77
2 Main S/S of Thrombocytopenia
1. Asymptomatic
2. Bleeding
78
Caring for Thrombocytopenic Patients
1. **Prevent or control hemorrhage – SAFETY**
2. **Education**: *blowing nose, no bending with head lower than waist, use electric razor, no invasive procedures.*
79
*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**
Disseminated Intravascular Coagulation (DIC)
80
Q: Does **Disseminated Intravascular Coagulation (DIC)** occur on its own?
No! Usually occurs as **secondary response to another underlying condition**, such as severe infection, malignancy, trauma, or complications during pregnancy.
81
Q: How does Disseminated Intravascular Coagulation (DIC) **lead to organ failure**?
* 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
*DIC:*
Over time clotting factors are consumed and ____ then occurs
bleeding
83
How do we **treat** Disseminated intravascular coagulation (DIC)
treat underlying cause – may reverse DIC
84
What blood products may be necesary for Disseminated intravascular coagulation (DIC) while treating disorder?
Platelets, cryoprecipitate, Frozen Fresh Plasma
85
4 Nursing priorities for **Disseminated intravascular coagulation (DIC)**
* Monitor VS closely- including Neurological checks
* **recognize s/sx of DIC**
* **patient safety**
* Bleeding precautions!!!
86
What medications should be avoided for Patients with Disseminated Intravascular Coagulation (DIC)
* aspirin
* NSAIDS
* beta-lactam antibiotics
87
*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**
Hemophilia
88
2 types of Hemophilia
1. Hemophilia A – defective **factor VIII**
2. Hemophilia B – defective **factor IX**
89
In HEMOPHILIA, About 75% of bleeding occurs in the ___.
joints
90
Q: How is hemophilia treated with clotting factor replacement?
3 treatments available
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: How often do children with **hemophilia** typically receive prophylactic treatment, and why?
3 to 4 times a week to minimize the risk of joint complications
92
What ANTIBODY med is given to patients with HEMOPHILIA-A?
Emicizumab- prevents bleeding
Memory: "***E**micizumab is the **E**ight factor" (hemophilia-A)*
93
Nursing Management for Hemophilia
* 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: What symptoms are commonly associated with von Willebrand disease (vWD)?
* Frequent nosebleeds
* Easy bruising
* Heavy menstrual bleeding
* Prolonged bleeding after injuries or surgeries
94
*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
von willebrand disease (vWD)
95
Goal of treatment for **von Willebrand Disease (vWD)**
REPLACE deficient protein at time of spontaneous bleeding or prior to invasive procedures
**know**
96
What is the **medication** is often used to **prevent bleeding** associated with dental and surgical procedures
Desmopressin
97
*von willebrand disease (vWD)*:
Platelet transfusion is done if
there is significant bleeding
98
What Blood Product can be given for von willebrand disease (vWD)
**Cryoprecipitate** (contains vWF and factor VIII) in emergency situations
