Hematology Flashcards

1
Q

RBCs at birth

A
  • high level of erythropoietin

- elevation of # of RBCs

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2
Q

erythropoietin

A

hormone secreted by the kidneys that increases the rate of RBCs production in response to drop in O2

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3
Q

When breathing starts…

A

increased level of O2 which then starts to decrease erythropoietin

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4
Q

2-3 mths, levels of RBCs…

A

continue to drop

-increased risk of developing anemia

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5
Q

Adult levels of RBCs reached…

A

adolescence

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6
Q

Adolescent boys…

A

have higher RBCs then girls

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7
Q

Norm Adult male RBC

A

4.6-5.9 mil

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8
Q

Norm Adult female RBC

A

4.2-5.4 mil

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9
Q

Norm Children RBC

A

4.6-4.8 mil

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10
Q

WBCs at birth

A

count is the highest

-values begin to decline after 12 hours of life and continue for the 1st week of life

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11
Q

1 wk of age, WBC..

A

begin to stabilize and remain steady until 1 year of age

After that, WBC slowly decreases until the adult value are reached in adolescence

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12
Q

Pediatric Differences in Platelets

A
  • levels are lower in NB than older children and adults
  • levels of many clotting factors are also lower in infants
  • VIt K is required for synthesis of several clotting factors….prophylactic inj of vit K at birth
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13
Q

Iron Deficiency Anemia

A
  • most prevalent nutritional disorder and most common mineral disturbance in the US
  • need iron to synthesize hemoglobin
  • iron is lost in stool so need adequate intake
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14
Q

Children 12-26 mths at risk for iron def anemia…

A

due to cows milk being main staple in this age group

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15
Q

FT infants exhaust iron stores by…

A

6 months

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16
Q

PT infants exhaust iron stores by…

A

3 months

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17
Q

Adolescents and Iron def anemia?

A

rapid growth and poor eating habits

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18
Q

Symptoms of Iron Def Anemia

A
  • pallor
  • fatigue
  • irritability
  • loss of appetite
  • decreased physical activity
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19
Q

Prevention of Iron Def Anemia

A
  • family education
  • PT infants: use iron supplement at 6-8 wks, continue to age 1
  • Formula infants: use iron fortified formula and cereals
  • BF infants: after 6 mths, use iron fortified cereals and iron rich foods. some may need iron replacement
  • diet education for teenagers, esp females
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20
Q

Iron administration

A

Iron 4-6 mg/kg for 3 months

  • ferrous sulfate better absorbed
  • give in 3 divided doses between meals when gastric aids are highest
  • also give with juice (vit C, ascorbic acid)
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21
Q

Iron Side Effects

A
  • liquid iron can stain teeth, use straw or dropper towards back of mouth
  • vomiting and diarrhea may occur, then give with meals and/or dose reduced then gradually increase
  • stools will turn a tarry green color
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22
Q

Sickle Cell Dz

A
  • autosomal-recessive disorder affecting normal RBC development
  • 25% chance with both parents carriers
  • AA: 1 in 12 blks are carriers, hispanics, west africans
  • Replacement of normal hemoglobin with Hgb S
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23
Q

Sickled RBCs

A
  • rigid, inflexible, can occlude small vessels
  • increased destruction, life expectancy in 10-20 days
  • vascular occlusion=tissue ischemia and organ damage
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24
Q

Sickling causes…

A
  • hemolytic anemia

- organ damage

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25
Q

Hemolytic anemia

A
  • pallor
  • fatigue
  • jaundice
  • irritability
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26
Q

Organ damage

A
  • enlarged spleen
  • enlarged liver
  • renal insufficiency
  • gallstones
  • priapism
  • abdominal pain
  • skin ulcers
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27
Q

Sickle Cell and Spleen..

A

-splenic sequestration from trapping of blood in spleen

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28
Q

Spleenectomy

A
  • severely compromised immune systems leading to increased rate of infections
  • bacterial infections are #1 cause of death in young children
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29
Q

Causes of Sickle Cell Crisis

A
  • infections
  • dehydration
  • acidosis
  • physical exertion
  • stress
  • exposure to cold
  • anything that increases body’s need for oxygen or alters transport of oxygen
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30
Q

Manifestations of Sickle Cell

A
  • depends on the severity of dz
  • usually not manifested in first 5-6 mths of life
  • anemia and fatigue
  • infections and fever
  • acute pain with decreased ROM
  • jaundice
  • hematuria
  • splenomegaly
  • hepatomegaly
  • priapism
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31
Q

Complications of Sickle Cell

A
  • CVA
  • Acute Chest syndrome
  • hand-foot syndrome (dactylitis)
  • blindness
  • multiple organ failure
  • avascular necrosis
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32
Q

Management of Sickle Cell

A

-prevent complications, primary prevention

  • NB screening
  • folic acid
  • hydration
  • vaccinate
  • prophylaxis with PCN or Amoxil until age 5
  • annual transcranial doppler for 2-16 yr olds
  • assessment for G/D delays
  • parent teaching with focus on nutritional and fluid needs plus S/S of infection
  • genetic counseling
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33
Q

If doppler results indicate stroke risk…

A

regular transfusions

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34
Q

Management of Crisis

A
  • BR with BRP-rest
  • hydration-oral and IV
  • electrolyte replacement-metabolic acidosis
  • pain control
  • oxygen
  • transfusions
  • antipyretics
  • antibiotics
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35
Q

Parental Education

A
  • seek early intervention for problems
  • adequate hydration, specify amount of intake
  • avoid hot weather
  • watch for signs of reduced intake, decrease UOP, increased thirst
  • Prevent infection, immunizations esp flu, pneumo, mening, PCN
  • seek early intervention for problems such as fever
  • avoid high altitude or non-pressurized air flights
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36
Q

Hemophilia A

A

“Classic hemophilia”

  • deficiency of factor VII
  • X-linked recessive disorder
  • Amounts for 80% of cases of hemophilia
  • occurence 1 in 5000 males
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37
Q

Hemophilia A B

A

less severe form

  • christmas disease
  • caused by deficiency of factor IX
  • accounts for 15% of cases of hemophilia
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38
Q

Pathophys of Hemophilia

A
  • based on too little antihemophilic factor (AHF)
  • AHF is produced in the liver and is needed to form thromboplastin
  • Lower amounts of AHF directly relate to the severity of disease.
  • bleeding can occur anywhere including skin, muscle, joints, GI, intracranial
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39
Q

Diagnosis of Hemophilia

A
  • hx of bleeding episodes
  • can be diagnosed through amniocentesis
  • genetic testing of family members to identify carriers
  • lab findings: low factor VIII levels and prolonged partial thromboplastin time (PTT)
  • the platelet count, prothrombin time, and fibrinogen level are all normal
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40
Q

Clinical Manifestations of Hemophilia

A
  • bleeding following circumcision
  • prolonged bleeding at umbilicus
  • slow, persistent and prolonged bleeding
  • joint tenderness with swelling, warmth, and pain
  • epistaxis
  • bruising
  • hematuria
  • pallor
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41
Q

Safety/Precaution of Hemophilia

A
  • teach family early recognition of a bleeding episode
  • teach family how to manage bleeds - RICE
  • appropriate activites to prevent accidents
  • avoid IM injections and heel/finger sticks
  • med alert
  • provide support
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42
Q

Ultimate goal of medical management for hemophilia

A

prevent bleeding, particularly into joints

may lead to crippling deformities

43
Q

Milder cases of hemophilia may respond to…

A

DDVAP, desmopressin acetate

44
Q

Factor VIII concentrate replacement

A

to be started immediately after a bleeding episode for approx. 3 to 14 days depending upon injury

or short term prophylaxis for 4-8 wks

45
Q

Prophylactic therapy for hemophilia

A
  • prevents bleeding and joint destruction
  • very expensive
  • complications from long term catheter
  • given on a schedule of 3x per week
  • does not revere damage but can slow progression and improve quality of life
46
Q

Medical Management for hemophilia

A
  • NSAIDs help, but may interfere with platelet function
  • Corticosteroids
  • if bleeding, RICE and immobilize affected joint
  • proper administration of Factor VIII or DDVAP
  • ROM only after bleeding stops to prevent contractures
  • analgesics
  • avoid obesity to minimize joint stress
  • PT to promote safe, age appropriate exercises to strengthen muscles and joints
47
Q

Immune (idiopathic) thrombocytopenia purpura (ITP)

A

most frequently occurring thrombocytopenia of childhood

most freq occuring between 2-10 years old

acquired hemorrhagic disorder characterized by:

  • thrombocytopenia
  • purpura
  • normal bone marrow

-chronic if over 6 mths duration (rare)…acute in 60-80 percent of cases

48
Q

Probable autoimmune etiology

A

caused by antibodies that bind to platelet membranes, this can lead to splenic destruction of platelets

may follow viral infections such as measles, mumps, chickenpox, rubella, URI, parvovirus

49
Q

Diagnosis of Immune (Idiopathic) Thrombocytopenia Purpura (ITP)

A
  • clinical findings
  • CBC
  • platelet count less than 20k
  • bleeding time prolonged
  • stool occult blood
50
Q

Symptoms of Immune (Idiopathic) Thrombocytopenia Purpura (ITP)

A
  • rapid onset
  • easy bruising esp over jt
  • purpura
  • petechiae
  • epistaxis
  • bleeding gums
  • hematuria
  • melena
  • menorrhagia
  • hepatosplenomegaly
51
Q

thrombocytopenia

A

excessive platelet destruction

52
Q

purpura

A

petechial discoloration of the skin

53
Q

Management of Immune (Idiopathic) Thrombocytopenia Purpura (ITP)

A

-milder forms: supported with activity restrictions and monitoring

more severe:
1. methylprednisolone IV

  1. IVIG
  2. Anti-D immunoglobulin
    - platelet transfusions
    - splenectomy in cases lasting one year or longer
54
Q

Pediatric Cancer Differences

A
  • immune system less mature, does not respond well to invading substances
  • cells grow quickly in children
  • adoptosis less developed in children
  • adults: cancer often due to dietary, lifestyle, prolonged exposure
    children: continuing presence of fetal cells in small children related to some cancers
55
Q

Most common childhood cancer

A

leukemia followed by brain and CNS tumors

56
Q

Signs of Childhood Cancer

A

C: continual, unexpected weight loss

H: headaches often with vomiting at night or early morning

I: increased swelling, jt pain

L: lump or mass in abdomen, neck, chest, pelvis, or armpits

D: development of excessive bruising, bleeding, or rash

C: constant infections

A: A whitish color behind the pupil

N: nausea which persist or vomiting without nausea

C: contant tiredness or noticeable paleness

E: eye or vision changes

R: recurrent fevers unknown origin

57
Q

3 categories of childhood cancer etiology

A
  1. external stimuli causing genetic mutations
  2. immune system and gene abnormalities
  3. chromosomal abnormalities
58
Q

external stimuli causing genetic mutations

A

radiation, immunosuppressant’s for organ transplants

59
Q

immune system and gene abnormalities

A

viruses can alter immune system, allow cancer to occur

60
Q

Chromosomal abnormalities

A

reported link between Down syndrome and leukemia

61
Q

Purpose of Diagnostic Tests for cancer

A
  • identify source of cancer (primary site)
  • determine if cancer has metastasized
  • stage cancer
62
Q

Clinical Therapy for Cancer

A
  • sx
  • chemo
  • radiation
  • biotherapy
  • bone marrow/stem cell transplant
  • complimentary therapy and traditional medical approach
63
Q

Wilm’s tumor

A

commonly surgically removed

64
Q

Chemo

A
  • damages dividing cells
  • can be given IV, PO, or intrathecal
  • circulate through bloodstream and damage any dividing cells
  • drugs combined for optimum cell cycle destruction with minimum toxic effects
65
Q

DNA in a normal cell…

A

can repair itself after chemo, a neoplastic cell cannot

66
Q

Chemo Protocol

A
  • plan of tx

- goal: maximize lethal impact on cells in all stages of activity

67
Q

Nursing implications for chemo administration

A
  • must be chemo certified
  • many chemotherapy agents are vesicants
  • can cause severe cell damage if even min amts infiltrate surrounding tissues
68
Q

Common SE of Chemo

A
  • bone marrow suppression….infection, bleeding
  • N/V…antiemetic before chemo
  • anorexia and wt loss
  • stomatitis–oral care
  • neuro problems
  • alopecia
  • cushingoid changes…moon face
  • mood changes
69
Q

Radiation

A
  • energy destroys DNA molecule and cell
  • goal is to irradiate tumor but not health tissue
  • may be used in combo with sx and drugs
  • curative or palliative
70
Q

Biotherapy

A

use parts of the boy already programmed to destroy cells and applies them to cancer cells

most are new or still under investigation

  • antibodies specific to cancer
  • vaccines that help body fight cancer
  • interferon
  • TNF –tumor necrosis factor
  • gene therapy
71
Q

Bone Marrow/Stem Cell Transplant

A

goal: kill cancer with chemo or radiation then resupply the body with stem cells
- tx of choice for some cancers that relapse
- sources of stem cells: childs bone marrow, compatible donor, cord blood

72
Q

Cell Cycle Specific Agents

A
  • antimetabolites
  • alkaloids
  • misc. phase activity
73
Q

Cell Cycle Nonspecific Agents

A
  • alkylating agents
  • antibiotics
  • nitrosureas
  • hormones
74
Q

Colony-stimulating factors

A
  • epoetin alfa
  • neupogen
  • neumega
75
Q

Nursing care for cancer

A
  • ensure optimal nutritional intake
  • admin meds
  • manage SE
  • ensure hydration
  • prevent and tx infections
  • manage pain
  • psychosocial support
  • discharge planning
76
Q

Leukemia

A

most common form of childhood cancer under 14 years old

abnormal proliferation of WBCs

related to chromosomal defects and exposure to radiation

77
Q

two main types of leukemia

A
  1. acute lymphoblastic leukemia (more common)

2. acute myelogenous leukemia

78
Q

Pathophys of Leukemia

A
  • stem cells in bone marrow produce large number of WBCs
  • WBCs are immature and do not fx normally
  • proliferate rapidly by cloning instead of mitosis and replace normal WBCs
  • risk for infection increases
  • RBC and platelets are replaced by malignant WBCs
79
Q

Results of Leukemia…

A
  • anemia
  • thrombocytopenia
  • immunosuppression
  • abnormal bleeding
80
Q

Clinical Manifestations

A
  • fever
  • anemia
  • Hepatospleenomegaly
  • Lymphadenopathy
  • CNS involvement, HA, V, papilledema
  • immunosuppression…fever, infection, poor wound healing
  • decreased platelets…bruising, petechiae, purpura, hematuria, epistaxis, tarry stool, frank bleeding
  • wt loss and anorexia
81
Q

Chemo Four Phases for Leukemia

A
  1. induction: max cell death, remission after 3-4 wks
  2. consolidation: CNS prophylaxis, prevent spread to brain, given with radiation
  3. delayed intensification: additional drugs to target surviing leukemic cells
  4. maintenance: may continue for 2-3 yrs
82
Q

Prognosis for Leukemia

A
  • generally better than in the past
  • favorable factors…2 to 10 onset, initial HgB less than 10, rapid response to chemo, WBCs less than 50,000 ***most important indicator
  • 10 percent relapse within 1 year after tx
83
Q

Nursing Care for Leukemia

A
  • prevent infection
  • skin and mouth care
  • attention to renal fx
  • central line care
  • RBC, platelet admin
  • chemo admin
  • monitor IV site for extravasation
  • psychosocial
84
Q

Hodgkin’s Disease

A
  • disorder of lymphoid system
  • arises from single lymph nose or group of nodes
  • leak occurrence in adolescent boys
  • possible genetic link
  • infectious agent or environmental hazard link
  • main symptom is nontender, firm lymphadenopathy (**supraclavicular cervical or mediastinal)
85
Q

Clinical Manifestations of Hodking’s Dz

A

main symptom: non-tender, firm lymph nodes

  • usual location: supraclavicular or cervical nodes
  • others seen with aggressive: fever, night sweats, wt loss, mediastinal mass can cause resp. problems or superior vena cava syndrome
86
Q

Diagnosis of Hodkin’s

A

lymph node biopsy

-Reed-Sternberg cells

87
Q

Therapy for Leukemia

A
  • tests done to determine severity…blood, scans of nodes and lymp system, bone marrow biopsy if advanced suspected
  • chemo four drug combo
  • radiation
  • bone marrow transplant
  • 5 year survival rate 80-90 percent
88
Q

Non-Hodgkin’s Lymphoma

A
  • malignant tumor of the lymphoreticular system - internal framework of the lymph system
  • three subtypes
  • third most common form of cancer in kids
  • peak incidence is 7-11 boys over girls
89
Q

Clinical Manifestations

A
  • fever, wt loss
  • enlarged, nodular lymph nodes
  • mediastinum mass
90
Q

Therapy for Non-Hodkin’s

A
  • chemo
  • raditation not commonly used
  • sx, tissue biopsy and tx complications caused by tumor
91
Q

Osteosarcoma

A

most common bone tumor of childhood

malignant bone tumor

occurs primarily in adolescent boys

located in the metaphysis of the distal femur, proximal tibia or proximal humerus

may be associated with radiation exposure or gene abnormality

92
Q

Osteosarcoma symptoms

A

pain
swelling
limp

93
Q

Diagnosis of Osteosarcoma

A

CT
MRI
blood tests
tumor biopsy to confirm diagnosis

94
Q

Tx for Osteosarcoma

A
  • sx and chemo
  • amputation sx
  • limb-salvage surgery–insert internal prosthesis
  • aggressive chemo: started before sx to shrink tumor also after sx to prevent spread
  • metastasis is common
  • physical rehab
95
Q

Wilms’ Tumor (nephroblastoma)

A
  • intrarenal tumor (kidney)
  • common abdominal tumor
  • most common age: 41-47 mths of age
  • associated with congenital anomalies
  • tumor suppressor gene may be missing
  • tumor grows rapidly–doubles its size in 11-13 days
  • chemo is 90 percent survival
96
Q

Clinical manifestations of Nephroblastoma

A
  • usually asymptomatic, mother may find firm mass during bath
  • located to one side of the midline of the abdomen
  • bilateral 5-10 per. cases
  • HTN may occur due to renin release
  • hematuria
97
Q

Dx and Tx of Nephroblastoma

A
  • abdominal US, CT, CBC, renal fx tests…BUN, Creatinie
  • Sx to remove affected kidney, look for metastasis
  • chemo with or without raditation
  • no palpation of tumor, may spread
98
Q

Neuroblastoma

A
  • smooth, hard, nontender mass
  • originates in SNS
  • usually diagnosed less than 5 years age
  • best prognosis less than 1 year of age
  • survival 90 per. stage 1 or 2, decrease 22 per for stage 4
99
Q

common location for neuroblastoma

A

abdomen, thoracic, a`drenal, cervical

100
Q

Etiology and Dx tests for neuroblastoma

A
  • cause unknown
  • environmental factors, prenatal drug exposure
  • canada, increase in folate in flour may have led to decrease incidence in neuroblastomas
  • dx: biopsy of tumor for initial dx, bone marrow biopsy to evaluate metastasis,

***dx is uaully made after metastasisis occurred

101
Q

Clinical Manifestations of Neuroblastoma

A
  • location of mass determines symptoms
  • labs: increased catecholamines in urine and blood (VMA and HVA) used for dx

-CBC with diff–thrombocytopenia, anemia, WBCs may be elevated or decreased

102
Q

Clinical Therapy and Tx for Neuroblastoma

A
  • stage of tumor determines tx
  • surgical removal of mass
  • follow with chemo, multiple drugs used
  • stem cell transplantation for advanced dz
  • radiation often used
  • best response to tx under age 1
103
Q

Nursing Management of Neuroblastoma

A

Assess presenting site of tumor–No palpation!!!!

Assess vital signs,

bowel and bladder function

Monitor weight, height

Observe gait

Postoperative care

Administer medications

Child and family teaching

Psychosocial assessment