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PEDS > lecture 11 [hematologic & immunologic dysfunctions] > Flashcards

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
Q

acute lymphoblastic leukemia is more common than acute myelogenous

A

true

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26
Q

what are the s/s of leukemia?

A
  • fever
  • bone/ joint pain
  • petechiae
  • anemia
  • bleeding
  • pallor
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27
Q

what is the most common form of childhood cancer?

A

leukemia

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28
Q

what is leukemia?

A

unrestricted proliferation of immature WBCs

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29
Q

what is the diagnostic procedure for leukemia?

A

bone marrow aspiration

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30
Q

what are the stages of chemotherapy?

A
  1. induction
  2. CNS prophylaxis
  3. intensification
  4. maintenance
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31
Q

what is the goal of induction?

stage of chemotherapy

A

to achieve complete remission or reduce leukemic cells in bone marrow by 5% or less

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32
Q

what is the goal of CNS prophylaxis?

stage of chemotherapy

A

prevents leukemic cells from invading CNS

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33
Q

what is the goal of intensification (consolidation)?

stage of chemotherapy

A

to eradicate residual leukemia cells

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34
Q

what is the goal of maintenance?

stage of chemotherapy

A

maintain remission

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35
Q

what are the effects of late stage leukemia?

A
  • cardiomyopathy
  • cognitive delays & deficits
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36
Q

what are the complications of myelosuppression?

A
  • infection
  • anemia
  • thrombocytopenia
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37
Q

what is considered an emergency for patients who are immunosuppressed?

A

fever

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38
Q

what are the lab tests & diagnostic procedures for immunocompromised patients?

A
  • blood cultures
  • urine cultures
  • CXR
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39
Q

what are the prognostic factors for leukemia?

A
  • initial WBC count
  • Karyotype analysis
  • type of cell involved
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40
Q

what are common side effects of chemotherapy?

A
  • nausea
  • vomiting
  • hemorrhagic cystitis
  • alopecia
  • moon face
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41
Q

what is lymphoma?

A

a group of neoplastic diseases that arise from the lymphoid & hematopoietic system

42
Q

what are the types of lymphoma?

A
  • Hodgkin lymphoma
  • Non-hodgkin lymphoma
43
Q

Hodgkin lymphoma primarily affects which age group?

A

patients aged 15-19

44
Q

Non-Hodgkin lymphoma primarily affects which age group?

A

children younger than 14 years old

45
Q

what are the diagnostic tests for lymphoma?

(2)

A
  • bone scan
  • lymph node biopsy
46
Q

what are the clinical manifestations of lymphoma?

A
  • fever
  • weight loss
  • night sweats
  • anorexia
  • enlarged lymph nodes
  • pruritus
47
Q

what is the therapeutic management for lymphoma?

A
  • radiation
  • chemotherapy
48
Q

what is the immunologic deficiency disorder in this lecture?

A

HIV/ AIDS

49
Q

HIV is usually transmitted by lymphocytes & monocytes

A

true

50
Q

in which bodily fluids is HIV found?

A
  • blood
  • semen
  • vaginal secretions
  • breast milk
51
Q

what is vertical transmission of HIV?

A

mother passes it to infant during pregnancy

52
Q

what is horizontal transmission of HIV?

A

through sexual contact or exposure to infected body fluids

53
Q

what are the diagnostic tests for HIV?

A
  • ELISA
  • Western blot test
54
Q

what is the treatment & prevention for HIV?

A
  • antiretroviral drugs
  • immunizations
55
Q

what are the treatments for hematologic & immunologic deficiencies?

A
  • blood transfusions
  • hematopoietic stem cell transplant
56
Q

what is the most severe reaction to a blood transfusion?

A

hemolytic rxn

57
Q

what are the most common rxns to a blood transfusion?

A
  • febrile
  • allergic
58
Q

what is intensive ablative therapy?

A

deplete entire bone marrow to prevent rejection of new stem cells w/ chemo or radiation

59
Q

what are the steps to hematopoietic stem cell transplant?

A
  1. intensive ablative therapy
  2. stem cells infusion by IV
  3. engrafting
60
Q

definition

engrafting

A

body accepts new bone marrow & proliferates

61
Q

definition

autologous

HSCT

A

use of the patient’s own bone marrow cells

62
Q

definition

allogenic

HSCT

A

use of matching histocompatible donor with a recipient

63
Q

what is the goal of treating WBC disorders?

A

repair or replace damaged WBCs while stopping the proliferation of immature or damaged WBCs

64
Q

what is the normal range for Hgb?

A

11.5 - 15.5g/dL

65
Q

what is the normal range for Hct?

A

35 - 45%

66
Q

what is a reticulocyte?

A

an immature RBC

67
Q

in which condition are reticulocytes elevated?

A

chronic hemolytic anemia

68
Q

purpose

neutrophils

A

primary defense of bacterial infection

69
Q

purpose

eosinophils

A

defends against parasites, allergies, & neoplasms

70
Q

purpose

basophils

A

increases blood flow to injured tissues

71
Q

purpose

lymphocytes

(antibodies)

A

fights off antigens & delays hypersensitivity

72
Q

purpose

monocytes

A

large phagocytic cells involved in the early stages of inflammatory rxns

73
Q

definition

Mean Corpuscular Volume

A
  • calculates svg size & volume of RBCs
  • determines the type of anemia
74
Q

definition

Mean Corpuscular Hemoglobin

A

calculates the avg amount of Hgb in (1) RBC

75
Q

what is the normal range for platelets?

A

150-400

76
Q

what is the main function of PLT?

A

helps clot blood

77
Q

what are the types of anemias?

A
  • iron deficiency
  • sickle cell
  • B-Thalassemia(Cooley)
  • aplastic
78
Q

what are the various reasons for anemia?

A
  • decreased RBC production
  • increased loss of RBC (from bleeding)
  • increased hemolysis
79
Q

what are common clinical manifestations of anemia?

A
  • fatigue
  • pallor
  • decrease in Hgb
80
Q

what are the main reasons for decreased RBC production?

A
  • nutritional deficiency
  • bone marrow failure
81
Q

what are the reasons for increased hemolysis?

A
  • membrane deficiencies (spherocytosis)
  • Thalassemia
  • chemotherapy
  • radiation
82
Q

what are the causes for iron deficiency anemia?

A
  • decreased supply of iron
  • malabsorption of iron
  • change in Hgb synthesis
83
Q

milk decreases absorption of iron

A

true

84
Q

what does the nurse tell parents when giving their child iron supplements?

A
  • 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
Q

what is the normal type of Hgb?

A

Hgb A

86
Q

patients with sickle cell anemia have what type of Hgb?

A

Hgb S

predominantly; it replaces Hgb A

87
Q

which diagnostic procedure is used for sickle cell anemia?

A
  • hemoglobin electrophoresis
  • sickledex finger stick
88
Q

sickle cell anemia is an autosomal recessive disorder

A

true

89
Q

what are the nursing actions for a sickle cell crisis?

A
  • adequate oxygenation
  • hydration
  • transfusion
  • ABX
  • analgesics
  • FEB
90
Q

what are the s/s of vasooculsive crisis in sickle cell anemia?

A
  • pain
  • priapism
  • acute chest syndrome
  • stroke
91
Q

what are the s/s of sequestration crisis in sickle cell anemia?

A
  • spleenic sequestration
  • hepatic sequestration
92
Q

definition

sequestration

A

trapping of cells and debris in the spleen, causing a decrease in Hgb

93
Q

what is the prevention for sepsis in sickle cell anemia?

A

prophylactic Penicillin

until 5 years old

94
Q

what are the clinical manifestations of Cooley anemia?

B-Thalassemia

A
  • small stature
  • splenomegaly
  • hepatomegaly
  • decreased H&H
  • abnormal blood smear
95
Q

how does the patient present with untreated Cooley anemia?

A
  • enlarged head due to prominent parietal & frontal bossing
  • prominent malar eminences
  • enlarged maxilla
  • freckled & bronzed complexion
96
Q

what is the therapeutic management for cooley anemia?

A
  • chronic blood transfusions
  • iron chelation essential
  • splenectomy
97
Q

what are the guidelines & parameters of chronic blood transfusion for Cooley anemia?

A
  • required every 3-5 weeks
  • maintain Hgb > 9.5
98
Q

what is aplastic anemia?

A

a bone marrow failure condition in which formed elements of the blood are simultaneously depressed

99
Q

what are the conditions associated with aplastic anemia?

A
  • anemia
  • leukopenia
  • thrombocytopenia
100
Q

what does hypoplastic anemia entail?

A

only the RBCs are decreased

101
Q

how is aplastic anemia managed?

A
  • Cyclosporin (immunosuppressive therapy)
  • steroids
  • HSCT

PEDS (11 decks)

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