lecture 11 [hematologic & immunologic dysfunctions] Flashcards

1
Q

what is the definition of hemostasis?

A

the process that stops bleeding when a blood vessel is injured

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2
Q

what blood components does hemostasis require?

A
  • vascular clotting factors
  • plasma clotting factors
  • platelets
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3
Q

what is the definition of fibrinolysis?

A

clot breakdown

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4
Q

what is hemophilia?

A

a group of bleeding disorders characterized by difficulty controlling bleeding due to lack of clotting factors

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5
Q

what are the types of hemophilia?

A
  • Hemophilia A (classic)
  • Hemophilia B
  • Von Willebrand Disease
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6
Q

which clotting factor does Hemophilia A lack?

A

factor VIII

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7
Q

which clotting factor does Hemophilia B lack?

A

factor IX

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8
Q

which is the most common type of hemophilia?

A

Von Willbrand disease

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9
Q

what is Von Willebrand hemophilia?

A

the inherited lack of the von willebrand factor protein characterized by inability of platelets to aggregate

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10
Q

both Hemophilia A & B are male dominant (x-linked recessive)

A

true

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11
Q

Von Willebrand disease affects both males & females

A

true

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12
Q

how is hemophilia diagnosed?

A
  • hx of bleeding episodes
  • evidence of x-linked inheritance
  • PTT
  • factor assays
  • DNA testing
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13
Q

what are the medications & treatment for hemophilia?

(6)

A
  • synthetic vasopressin (DDAVP)
  • steroids
  • factor VIII products
  • pooled plasma
  • recombinant products
  • NSAIDs
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14
Q

what is the MOA of DDAVP (Desmopressin)?

A

increases plasma factor VIII (only for Hemophilia A)

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15
Q

what is the nursing care for hemophilia?

(4)

A
  • monitor urine, stool, & NG fluid for occult blood
  • administer Acetaminophen instead of aspirin
  • administer injections via subQ only
  • venipunctures are preferred for blood sampling
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16
Q

what is idiopathic thrombocytopenia purpura?

A

a decrease in platelet count manifested by discoloration caused by petechia beneath the skin

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17
Q

acute idiopathic thrombocytopenia purpura is most often seen after acquiring these diseases:

A
  • MMR
  • chickenpox
  • parovirus B19 (Fifth’s disease)
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18
Q

what are the clinical manifestations of ITP?

A
  • easy bruising
  • bleeding from mucous membranes
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19
Q

what is considered a low platelet count?

A

less than 20,000

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20
Q

what is the function of the spleen?

A

controls the level of blood cells (RBC, WBC, PLT, etc) by elimination

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21
Q

what is often a therapeutic procedure for ITP?

A

splenectomy

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22
Q

what are the medications for ITP?

A
  • prednisone
  • IVIG
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23
Q

what are the neoplastic disorders?

A
  • leukemia
  • lymphoma
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24
Q

what are the types of leukemia in children?

A
  • acute lymphoblastic leukemia
  • acute myelogenous leukemia
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25
*acute lymphoblastic* ***leukemia*** is more common than *acute myelogenous*
true
26
what are the s/s of ***leukemia***?
* fever * bone/ joint pain * petechiae * anemia * bleeding * pallor
27
what is the most common form of *childhood cancer*?
leukemia
28
what is ***leukemia***?
unrestricted proliferation of *immature* WBCs
29
what is the diagnostic procedure for ***leukemia***?
bone marrow aspiration
30
what are the stages of ***chemotherapy***?
1. induction 2. CNS prophylaxis 3. intensification 4. maintenance
31
what is the goal of ***induction***? | stage of chemotherapy
to achieve complete remission ***or*** reduce leukemic cells in bone marrow by 5% or less
32
what is the goal of ***CNS prophylaxis***? | stage of chemotherapy
prevents leukemic cells from invading CNS
33
what is the goal of ***intensification*** *(consolidation)*? | stage of chemotherapy
to eradicate residual leukemia cells
34
what is the goal of ***maintenance***? | stage of chemotherapy
maintain remission
35
what are the effects of *late stage* ***leukemia***?
* cardiomyopathy * cognitive delays & deficits
36
what are the complications of ***myelosuppression***?
* infection * anemia * thrombocytopenia
37
what is considered an emergency for patients who are *immunosuppressed*?
fever
38
what are the lab tests & diagnostic procedures for immunocompromised patients?
* blood cultures * urine cultures * CXR
39
what are the prognostic factors for ***leukemia***?
* initial WBC count * Karyotype analysis * type of cell involved
40
what are common side effects of *chemotherapy*?
* nausea * vomiting * hemorrhagic cystitis * alopecia * moon face
41
what is ***lymphoma***?
a group of neoplastic diseases that arise from the *lymphoid* & *hematopoietic* system
42
what are the types of ***lymphoma***?
* Hodgkin lymphoma * Non-hodgkin lymphoma
43
***Hodgkin lymphoma*** primarily affects which age group?
patients aged 15-19
44
***Non-Hodgkin lymphoma*** primarily affects which age group?
children younger than 14 years old
45
what are the diagnostic tests for ***lymphoma***? | (2)
* bone scan * lymph node biopsy
46
what are the clinical manifestations of ***lymphoma***?
* fever * weight loss * night sweats * anorexia * ***enlarged lymph nodes*** * pruritus
47
what is the therapeutic management for ***lymphoma***?
* radiation * chemotherapy
48
what is the ***immunologic deficiency disorder*** in this lecture?
HIV/ AIDS
49
***HIV*** is usually transmitted by *lymphocytes* & *monocytes*
true
50
in which bodily fluids is ***HIV*** found?
* blood * semen * vaginal secretions * breast milk
51
what is *vertical transmission* of ***HIV***?
mother passes it to infant during pregnancy
52
what is *horizontal transmission* of ***HIV***?
through sexual contact or exposure to infected body fluids
53
what are the diagnostic tests for ***HIV***?
* ELISA * Western blot test
54
what is the treatment & prevention for ***HIV***?
* antiretroviral drugs * immunizations
55
what are the treatments for ***hematologic*** & ***immunologic deficiencies***?
* blood transfusions * hematopoietic stem cell transplant
56
what is the most severe reaction to a ***blood transfusion***?
hemolytic rxn
57
what are the most common rxns to a ***blood transfusion***?
* febrile * allergic
58
what is ***intensive ablative therapy***?
deplete entire bone marrow to prevent rejection of new stem cells w/ chemo or radiation
59
what are the steps to ***hematopoietic stem cell transplant***?
1. intensive ablative therapy 2. stem cells infusion by IV 3. engrafting
60
# definition engrafting
body accepts new bone marrow & proliferates
61
# definition autologous | HSCT
use of the patient's own bone marrow cells
62
# definition allogenic | HSCT
use of matching histocompatible donor with a recipient
63
what is the goal of treating ***WBC disorders***?
*repair or replace* damaged WBCs while *stopping the proliferation* of immature or damaged WBCs
64
what is the normal range for ***Hgb***?
11.5 - 15.5g/dL
65
what is the normal range for ***Hct***?
35 - 45%
66
what is a ***reticulocyte***?
an immature RBC
67
in which condition are ***reticulocytes*** elevated?
chronic hemolytic anemia
68
# purpose neutrophils
primary defense of *bacterial* infection
69
# purpose eosinophils
defends against parasites, allergies, & neoplasms
70
# purpose basophils
increases blood flow to injured tissues
71
# purpose lymphocytes | (antibodies)
fights off antigens & delays hypersensitivity
72
# purpose monocytes
large phagocytic cells involved in the early stages of inflammatory rxns
73
# definition Mean Corpuscular Volume
* calculates svg *size & volume* of RBCs * determines the type of ***anemia***
74
# definition Mean Corpuscular Hemoglobin
calculates the avg amount of Hgb in (1) RBC
75
what is the normal range for ***platelets***?
150-400
76
what is the main function of ***PLT***?
helps clot blood
77
what are the types of ***anemias***?
* iron deficiency * sickle cell * B-Thalassemia*(Cooley)* * aplastic
78
what are the various reasons for ***anemia***?
* decreased RBC production * increased loss of RBC *(from bleeding)* * increased hemolysis
79
what are common clinical manifestations of ***anemia***?
* fatigue * pallor * decrease in Hgb
80
what are the main reasons for *decreased* ***RBC production***?
* nutritional deficiency * bone marrow failure
81
what are the reasons for *increased* ***hemolysis***?
* membrane deficiencies *(spherocytosis)* * Thalassemia * chemotherapy * radiation
82
what are the causes for ***iron deficiency anemia***?
* decreased supply of iron * malabsorption of iron * change in *Hgb* synthesis
83
milk decreases absorption of iron
true
84
what does the nurse tell parents when giving their child *iron supplements*?
* give 2x a day in between meals * give with Vit C * iron turns stools tarry green * it can stain teeth * do not take with milk
85
what is the normal type of ***Hgb***?
Hgb A
86
patients with ***sickle cell anemia*** have what type of *Hgb*?
Hgb S | predominantly; it replaces Hgb A
87
which diagnostic procedure is used for ***sickle cell anemia***?
* hemoglobin electrophoresis * sickledex finger stick
88
***sickle cell anemia*** is an *autosomal recessive disorder*
true
89
what are the nursing actions for a ***sickle cell*** *crisis*?
* adequate oxygenation * hydration * transfusion * ABX * analgesics * FEB
90
what are the s/s of *vasooculsive crisis* in ***sickle cell anemia***?
* pain * priapism * acute chest syndrome * stroke
91
what are the s/s of *sequestration crisis* in ***sickle cell anemia***?
* spleenic sequestration * hepatic sequestration
92
# definition sequestration
trapping of cells and debris in the spleen, causing a decrease in Hgb
93
what is the prevention for *sepsis* in ***sickle cell anemia***?
prophylactic Penicillin | until 5 years old
94
what are the clinical manifestations of ***Cooley anemia***? | B-Thalassemia
* small stature * splenomegaly * hepatomegaly * decreased H&H * abnormal blood smear
95
how does the patient present with untreated ***Cooley anemia***?
* enlarged head due to prominent parietal & frontal bossing * prominent malar eminences * enlarged maxilla * freckled & bronzed complexion
96
what is the therapeutic management for ***cooley anemia***?
* chronic blood transfusions * iron chelation essential * splenectomy
97
what are the guidelines & parameters of chronic blood transfusion for ***Cooley anemia***?
* required every 3-5 weeks * maintain *Hgb > 9.5*
98
what is ***aplastic anemia***?
a bone marrow failure condition in which formed elements of the blood are simultaneously depressed
99
what are the conditions associated with ***aplastic anemia***?
* anemia * leukopenia * thrombocytopenia
100
what does ***hypoplastic anemia*** entail?
only the RBCs are decreased
101
how is ***aplastic anemia*** managed?
* Cyclosporin *(immunosuppressive therapy)* * steroids * HSCT