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Nursing 202 > Hematologics > Flashcards

Hematologics Flashcards

1
Q

Anemia definition:

A

reduction of either the number of RBCs, the amount of hemoglobin, or hematocrit (% of packed RBCs per deciliter of blood)

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2
Q

Definition of Sickle Cell Disease:

A

genetic disorder that results n chronic anemia, pain, disability, organ damage, increased risk for infection, and early death

Formation of abnormal hemoglobin chains - most common in Af. Americans

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3
Q

In normal hemoglobin, hemoglobin A(HbA) has

A

two alpha chains and two beta chains of amino acids

98-99% with a small % of fetal form of HbF

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4
Q

In SCD, what % is the total hemoglobin is composed of an abnormal beta chain?

A

40%

HbS - sensitive to low O2 content of RBCs

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5
Q

The clumped masses of sickled RBCs block blood flow is called what?

A

Vaso-occlusive event (VOE)

leads to hypoxia

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6
Q

The average life span of ab RBC conatining 40% or more of HbS is about how many days?

A

10-20 days – causes hemolytic (cell-destroying) anemia in patients with SCD

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7
Q

normal life span of RBCs

A

120 days

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8
Q

Patients with SCD has periodic episodes of extensive cellular sickling called what?

A

Crises –lead to hypoxemia (deficit of O2 in blood)

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9
Q

Etiology of SCD

A

Autosomal recessive pattern of inheritance

Specific mutation on chromosome 11

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10
Q

AS:

A

Sickle cell trait: occurs when one normal gene allele and one abnormal gene allele for hemoglobin are inherited and only half of the hemoglobin chains are abnormal

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11
Q

History of SCD:

A

Long sanding diagnosis
Usually has no manifestations other than presence of HbS

-Determine the patient’s energy level using a scale ranging from 0 to 10 to access degree of fatigue

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12
Q

What is the most common manifestation of SCD crisis?

A

pain

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13
Q

Physical assessments of SCD include:

A 
Cardiovascular changes
Priapism
Skin changes 
Abdominal changes
Kidney and urinary changes 
Musculoskeletal changes 
CNS changes
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14
Q

Cardiovascular changes: SCD

A

risk for high output heart failure

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15
Q

Priapism: SCD

A

prolonged penile erection that can occur to men with SCD

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16
Q

Skin changes: SCD

A

include pallor or cyanosis because of poor gas exchange from decreased perfusion and anemia
plus Jaundice results from RBC destruction and release of bilirubin

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17
Q

Abdominal changes: SCD

A

damage to the spleen and liver, occurs often early from many episodes of hypoxia and ischemia

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18
Q

Kidney and urinary changes: SCD

A

common as a result of poor perfusion and decreased tissue gas exchange

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19
Q

Musculoskeletal change: SCD

A

occur because arms and legs are often sites of blood vessel occlusion

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20
Q

CNS changes: SCD

A

During crisis, patients may have a low grade fever, if CNS has infarcts or repeated episodes of hypoxia, patients may have seizures or manifestations of a stroke
Assess for presence of “pronator drift” bilateral hand grasp strength, gait, and coordination

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21
Q

Psychosocial assessment: SCD

A

Often behavior changes are early manifestations of cerebral hypoxia from poor perfusion

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22
Q

Lab assessment: SCD

A

The diagnosis of SCD is based on the percentage of hemoglobin S on electrophoresis
AS is usually less than 40% HbS, and does not change during crisis

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23
Q

what count is usually high in SCD?

A

WBCs

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24
Q

Imaging assessment: SCD

A

Bone changes occur as a result of chronically stimulated marrow and low bone O2 levels

Skull= crew cut appearance on x-ray
x-rays of joints may show necrosis and desruction

MRIs may show soft tissue and organ changes from poor perfusion and chronic inflammation

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25
Other diagnostic testing: SCD
ECGs document cardiac infarcts and tissue damage | -may show cardiomyopahy and decreased cardiac output
26
Managing pain of SCD:
Mild pain = managed at home Severe pain = require hospitalization and opioid analgesics -Drug therapies, hydration, and complementary therapies
27
Preventing sepsis: for SCD
Continually assess for infection and monitor CBC with differential WBC count daily Drug therapy Hydration O2 is given during crisis Transfusion possible to help increase HbA levels
28
Most common type of inherited hemolytic anemia is the deficiency of the enzyme:
glucose-6-phosphate dehydrogenase (G6PD)
29
Define G6PD:
inherited as an X-linked recessive disorder with more severe expression in males and mild expression in carrier females Affects 10% of Af. Americans and also may occur in Sephardic Jews, Greeks, Iranians, Chinese, Filipinos, and Indoesians
30
Cells with reduced G6PD do what
break more easily during exposure to some drugs ex. aspirin patient usually does not have manifestations until exposed to triggering agents or until severe infection -after exposure, breakage begins and lasts 7-12 days. anemia and jaundice develop
31
Men in high risk of G6PD should do what?
be tested for this problem before being given drugs that can cause the hemolytic reaction -Osmotic diuretics help prevent complications
32
Immunohemolytic anemia:
immune system products attack a person's own RBCs for unknown reasons
33
2 types of immunohemolytic anemia:
1. Warm antibody anemia: occurs with immunoglobulim G antibody excess, most active 98.6 F (37 C) may be triggered by drugs, chemicals, or other autoimmune problems 2. Cold antibody: has complement protein fixation on immunoglobulin M, most active 86 F (30 C) - occurs like Raynaud's response
34
Anemias resulting from decreased production of RBCs:
Iron deficiency anemia: serum levels less than 10ng/mL Vitamin B deficiency: glossitis - smooth beefy tongue Folic Acid deficiency: due from malabsorption ex. Crohn's Aplastic therapy: failure of bone marrow to produce RBCs
35
Disorders of excess RBCs or Iron:
Polythemia Vera | Hereditary Hemochromatosis
36
Polythemia Vera Patho:
Hyperviscous | Increase hemoglobin levels to 18 g/dL, RBC count of 6 million/mm, hematocrit of 55% or greater
37
More than 90% of patients with PV show mutation of the
JAK2 kinase gene in the affected cells | Extreme hypercellularity of peripheral blood occurs in people with PV
38
Features of PV:
- the patient's facial skin and mucous membranes have a dark, purple or cyanotic, flushed (plethoric) appearance with distended viens - intense itching caused by diluted blood vessels and poor perfusion is common - blood flow may be so slow that stasis occurs --> leads to thrombosis (clotting) within smaller vessels --> tissue hypoxia, anoxia and, later, to infartion and necrosis
39
Apheresis:
- the withdrawal of whole blood and removal of some of the patient's blood component, in this case RBCs - increasing hydration and promoting venous return help prevent clot formation - therapy for PV also includes the use of anticoagulants - Aggressive IV chemotherapy is no longer recommended due to risks of getting leukemia
40
Hereditary hemochromatosis definition:
autosomal recessive disorder in which a mutation in both alleles of the HFE gene cause increased intestinal absorption of dietary iron -excess iron is deposited in a variety of tissues and organs, including the liver, spleen, heart, joints, skin, and pancreas Treatment: phlebotomy and removal of 500 mL of blood at a time, occurring as often as twice weekly at first, is performed to reduce the overall iron load of the blood
41
Myelodysplastic syndromes (MDS):
group of disorders caused by the formation of abnormal cells in the bone marrow - decrease in all blood cell types - Conditions include Anemia (most common), neutropenia (low WBC), and thrombocytopenia (low platelets) - precancerous state but has cancer like features
42
Patients with MDS, patients are categorized into risk groups
based on the severity of panytopenia (low counts of all blood cell types), cytogenetic abnormalities, and numbers of blast cells (immature WBC) found in the bone marrow -Exact cause is not clear
43
For low risk and intermediate 1 risk MDS, the antitumor immunomodulatory agent called what? And is approved for patients whose dysplastic cells have the chromosome abnormality of a deleted what?
lenalidomide (Revlimid) | 5q
44
2 other agents approved for high risk intermediate 2-risk MDS are
Azacitidine (Vidaza) | Decitabine (Dacogen)
45
Platelet Disorders:
Autoimmune thrombocytopenic purpura (ITP) | Thrombotic thrombocytopenic purpura (TTP)
46
Thrombocytopenia:
Platelet numbers below that needed for blood clotting, may occur as a result of other conditions or treatments that suppress general bone marrow activity
47
Patho of ITP:
the number of circulating platelets is greatly reduced in ITP, even though platelet production is normal -make an antibody against the surface of their own platelets, making destruction by macrophages easier
48
Assessment of ITP:
manifestations are first seen in the skin and mucous membranes; large ecchymoses (bruises) or a petechial rash on the arms, legs, upper chest, and neck; mucosal bleeding occurs easily
49
Interventions for ITP:
Risk for poor clotting and increased bleeding - Drug therapy - Platelet transfusions - Maintaining a safe environment - Surgical management - Vaccinations against pneumococcal recommended
50
TTP definition:
- the patient has inappropriate clotting, yet the blood fails to clot when trauma occurs - appears to be a small autoimmune reaction in small blood vessel cells that starts platelet aggregation and clotting there - tissues become ischemic, leading to kidney failure, myocardial infarction, and stroke -Drug therapy such as aspirin
51
Clotting factor disorders:
Hemophilia | Heparin Induced Thrombocytopenia
52
Hemophilia: definition and 2 forms
hereditary bleeding disorder 1. Hemophilia A: classic, deficiency of factor of VIII and accounts for 80% of hemophilia cases 2. Hemophilia B: Christmas disease, deficiency of factor IX
53
Assessment of Hemophilia:
- Excessive bleeding - Joint and muscle hemorrhages - A tendency to bruise easily - Prolonged and potentially fatal hemorrhage after surgery
54
Heparim induced thrombocytopenia (HIT) definition and risk factors:
Serious immunity- mediated clotting disorder with an unexplained drop in platelet count after heparin treatment Risk factors: 1. Duration of heparin use longer than 1 week 2.Exposure to unfractioned heparin 3. Postsurgical thromboprophylaxis
55
Treatment for HIT:
anitcoagulation therapy | Drug management such as argatroban (Argatroban), lepirudin (Refludan), and bivalirudin (Angiomax)
56
Transfusion therapy includes:
Pretransfusion responsibilities: use normal saline Transfusion responsibilities: Remain with the patient for the first 15-30min Types of Transfusions
57
Types of Transfusions:
RBC transfusions Platelet transfusions Plasma Granulocyte (WBC)
58
Acute Transfusions Reactions:
``` Febrile transfusion reactions Hemolytic transfusion reactions Allergic transfusions Bacterial transfusion reactions Circulatory overload Transfusion associated graft-versus-host disease (TA-GVHD) Transfusion related acute lung injury (TRALI) Acute pain transfusion reaction ```
59
Febrile transfusion reactions:
occur most often in the patient with anti-WBC, antibodies, which can develop after multiple transfusions, WBC transfusions, and platelet transfusions
60
Hemolytic transfusion reactions are caused by
``` blood type or Rh incompatibility manifestations include: apprehension headache chest pain low back pain tachycardia tachypnea hypotension hemoglobinuria a sense of impeding doom ```
61
Allergic transfusions are most often seen in patients with
other allergies
62
Bacterial transfusion reactions
occur from infusion of contaminated blood products, especially contaminated with gram-negative organism
63
Circulatory overload:
can occur when a blood product is infused too quickly, especially in older adults
64
Transfusion associated graft-versus-host disease (TA-GVHD):
rare but life threatening problem that occurs more often in an immunosuppresed patient
65
Transfusion related acute lung injury (TRALI):
life threatening event that occurs most often when donor blood contains antibodies against the recipient's neutrophil antigens, HLA, or both
66
Acute pain transfusion reaction (APTR):
recently identified but rare event that can occur during or shortly after transfusion of any blood product -severe chest pain, back pain, joint pain, hypertension, and redness of the head and neck
67
Interventions for transfusion reactions
Stopping transfusion and removing blood tubing -Do not flush contents of the blood transfusion tubing, which would allow more of the reaction causing blood to enter the patient
68
Autologous blood transfusions:
may be stored for 40 days | for patients with rare blood types, blood may be frozen for up to 10 years
69
The patient with Thrombocytopenia:
Avoid IM injections or venipunctures: use the smallest gauge needle if neccessary Apply firm pressure to the needle stick site for 10 min or until site no longer oozes blood Apply ice Test presence of occult blood Check IV sites every 2 hrs of bleeding Avoid trauma to rectal tissues Measure abdominal girth daily Use an electric shaver Avoid mouth trauma Not to blow the nose or insert objects into the nose Avoid contact sports Wear shoes with firm soles with firm soles when ambulating

Nursing 202 (20 decks)

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