Hematological Disorders Flashcards

1
Q

what is the pathophysiology of iron deficiency anemia?

A

1) low iron
2) decreased Hgb production
3) decreased oxygen delivery

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2
Q

what are the risk factors for iron deficiency anemia?

A

inadequate intake
excessive cows milk
premature birth
malabsorption

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3
Q

how does cow’s milk affect iron absorption?

A

high calcium inhibits iron absorption

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4
Q

what are the signs and symptoms of iron deficiency anemia?

A

pallor
fatigue
tachycardia
pica
spoon shaped nails (koilonychia)

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5
Q

what are the management interventions for iron deficiency anemia?

A

iron-rich diet
iron supplements (ferrous sulphate)

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6
Q

what are some iron-rich foods?

A

red meat
leafy greens
beans
fortified cereal

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7
Q

what are some considerations for iron supplementation?

A

give between meals
give with vitamin C (orange juice) for better absorption
not with cow’s milk or calcium
increase fibre and fluid

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8
Q

what are some side effects of iron supplements?

A

black stool
constipation

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9
Q

what is the pathophysiology of SCD?

A

autosomal recessive disorder
1) RBCs become sickled
2) vasooclusion
3) ischemia
4) organ damage

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10
Q

what are some risk factors for SCD?

A

cold exposure
stress
hypoxia
dehydration

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11
Q

what are some complications of SCD?

A

vasooclusive crisis
- ischemia = severe pain
acute chest syndrome
- chest pain
- hypoxia
- fever
- resp distress
splenic sequestration crisis
stroke
hyperhemolysis
- rapid destruction of RBCs

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12
Q

what is splenic sequestration? what can it lead to?

A

sickled RBCs get trapped in spleen
hypovolemic shock
reduced spell function = risk of infection

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13
Q

what are the management interventions for SCD?

A

hydration
oxygen
pain relief
hydroxyurea
antibiotics
blood transfusion
vaccinations

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14
Q

what is hydroxyurea?

A

increases hemoglobin in RBCs
makes RBCs larger, round and more flexible
less likely to become sickle shaped

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15
Q

why should aspirin and NSAIDs be avoided in SCD?

A

risk of kidney damage

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16
Q

why should cold packs be avoided in SCD?

A

vasoconstriction worsens sickling

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17
Q

what is the pathophysiology of leukaemia?

A

overproduction of immature WBCs

leads to bone marrow suppression

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18
Q

what are the signs and symptoms of leukaemia?

A

anemia
thrombocytopenia
neutropenia
fever
fatigue
dark purple rash
bone pain
hepatosplenomegaly
swollen lymph nodes
bleeding/swollen gums
poor wound healing
abdominal fullness
unexplained bruising

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19
Q

how is leukaemia diagnosed?

A

bone marrow aspiration/biopsy
peripheral blood smear
lumbar puncture

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20
Q

what are the management interventions for leukaemia?

A

chemotherapy
cranial irradiation (rare)
blood transfusion

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21
Q

what is tumor lysis syndrome?

A

cancer cells break down
release substances into blood

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22
Q

what are the signs of tumour lysis syndrome?

A

hyperkalemia
hyperuricemia

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23
Q

what is the pathophysiology of febrile neutropenia?

A

neutropenia < 0.5 + fever > 38.3 degrees C
high risk of infection

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24
Q

what are the causes of febrile neutropenia?

A

chemotherapy
leukemia
BM disorders
infection
- bacterial
- viral
-fungal

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25
what are the signs and symptoms of febrile neutropenia?
fever (earliest) malaise chills
26
what are the management interventions for febrile neutropenia?
immediate IV antibiotics (pip/tazo) infection precautions G-CSF to stimulate WBC production
27
what is the pathophysiology of lymphoma?
DNA mutation cause uncontrollable growth of lymphocytes
28
what is the main difference between Hodgkin's lymphoma and non-hodgkin's lymphoma?
Hodgkin's: Reed-Sternberg cells Non-Hodgkin's: no Reed-Sternberg cells
29
Hodgkin's lymphoma is more common among what age group?
adolescents young adults
30
Non-Hodgkin's Lymphoma is more common among what age group?
children teens
31
What is the lymph node involvement in Hodgkin's lymphoma?
painless, firm cervical lymph nodes
32
What is the lymph node involvement in Non-Hodgkin's lymphoma?
diffuse lymph node involvement
33
what are the symptoms of Hodgkin's lymphoma?
B symptoms - fever - night sweats - weight loss
34
what are the symptoms of Non-Hodgkin's lymphoma?
more aggressive B symptoms - fever - night sweats - weight loss
35
how is Hodgkin's lymphoma diagnosed?
biopsy of lymph nodes presence of reed-sternberg cells
36
how is Non-Hodgkin's lymphoma diagnosed?
biopsy CT/MRI for staging
37
what is the treatment for Hodgkin's lymphoma?
radiation chemo (AVBD regimen)
38
what is the treatment for non-Hodgkin's lymphoma?
aggressive chemo
39
what are some signs of hematological and immune dysfunction?
high WBCs - infection - leukemia low hemoglobin - anemia low hematocrit - anemia bleeding and bruising - low platelets frequent infections pale fatigue
40
what is the most common childhood hematological disorder?
anemia
41
what is anemia?
decreased RBCs or hemoglobin decreased oxygen carrying capacity
42
what are the causes of anemia?
manifestations of other disorders - blood loss - infection - autoimmune disorders - leukemia - lymphoma
43
why are premature infants at risk for anemia?
don't get as much iron rich blood from mom
44
what causes micro-sized RBCs?
iron deficiency
45
what are the signs and symptoms of anemia?
pallor jaundice swollen liver and spleen
46
what are the treatments for anemia?
treat underlying cause - hemorrhage = transfusion - iron deficient = diet and supplements IV fluids - replace volume oxygen -decrease O2 demand bed rest - decrease oxygen demand hand hygiene decrease risk of falls
47
when should infants start breastfeeding?
4 months
48
when should premature infants start breastfeeding?
2 weeks
49
when should cow's milk be avoided until?
12 months
50
what is the limit for cow's milk per day?
600 mL
51
why are adolescents at risk for iron deficiency anemia?
rapid growth poor eating habits
52
why are indigenous at risk for iron deficiency anemia?
food insecurity poverty
53
what is the diet and supplementation requirement for iron deficiency anemia?
3 mg/kg body weight
54
what are the causes of SCD?
obstruction/destruction of RBCs vascular inflammation
55
why is hydration important for SCD?
dehydration causes RBCs to develop sickle shape ad occlude vessels sickle cells increase urination
56
how is SCD pain managed?
morphine PCA pumps
57
what type of blood transfusion is used for SCD?
exchange sickled RBCs removed ad replaced with normal RBCs decreases blood viscosity
58
why do we give antibiotics for SCD?
spleen isn't functioning
59
what kind of antibiotics dose is given for SCD?
low PREVENTATIVE dose to decrease risk
60
what is the leading cause of death in children?
neoplastic disorders
61
what is the most common childhood cancer?
leukemia
62
leukemia is more frequent in __________________
males > 1 year
63
what is the peak age of leukaemia onset?
2-5 years
64
leukemia has good ___________
survivability
65
what are the 3 types of leukaemia?
1) acute lymphoid leukaemia (ALL) 2) acute myeloid leukemia (AML) 3) stem cell/blast cell leukaemia
66
how does a peripheral blood smear test for leukaemia?
immature leukocytes low blood cell counts
67
how does bone marrow aspiration/biopsy test for leukaemia?
from iliac crest check for blast cells
68
how does lumbar puncture test for leukaemia?
check CNS involvement - test CSF for blast cells
69
what is important for best leukaemia prognosis?
start chemo right away
70
what factors affect the leukaemia prognosis?
initial WBC count age cell type involved cytogenetics (chromosomes) immunophenotype response to induction therapy gender relapse
71
WBC count of _____________ improves prognosis for leukemia
> 50,000
72
what age increases risk of worse prognosis for leukaemia?
< 10 years - ALL is ok - ANL is high risk > 10 years - ANL is ok - ALL is high risk
73
what is the best prognosis for leukaemia?
B cell ALL
74
what response to induction therapy is a good sign for leukaemia prognosis?
want to be in remission by the end of the reduction period (1 month)
75
what gender has better leukaemia prognosis?
girls
76
what would indicate dismal (poor) prognosis for leukaemia?
relapse after transplant
77
what is acute lymphoid leukaemia (ALL)?
develops from immature lymphoid stem cells B cells or T cells more common in children good prognosis in children
78
what is acute myeloid leukaemia?
develops from lymphoid stem cells more common in adults highly variable prognosis
79
what are some considerations for chemotherapy?
monitor for tumour lysis syndrome prevent infection monitor for oral mucositis
80
when is radiation used for leukaemia?
only if CNS involvement
81
what are the indications for a blood transfusion for leukaemia?
haemoglobin < 70 or symptomatic platelets < 10,000
82
why are blood transfusions often used to treat leukaemia?
chemotherapy is not selective and destroys all cell types blood transfusion helps to replenish with normal cells
83
what are the 4 phases of leukaemia therapy?
1) induction 2) CNS prophylaxis 3) intensification 4) maintenance
84
induction phase of leukaemia therapy
4-6 weeks to destroy leukaemia cells and induce remission
85
CNS prophylaxis for leukaemia therapy
throughout all phases of treatment leukaemia cells can hide I brain and spinal cord intrathecal chemo or radiation (if severe)
86
intensification phase of leukaemia therapy
4-8 months prevent regrowth and resistant clones higher doses of chemo
87
maintenance phase of leukaemia therapy
2-3 years destroy remaining undetectable leukemia cells low dose chemo (oral with some IV)
88
what are neutrophils?
type of white blood cell (leukocyte)
89
hospitalization protocol for febrile neutropenia
minimum of 48 hours regardless if no fever for 48h = discharged after 48h, must go 24h without fever
90
what is the protocol for antibiotics for febrile neutropenia?
put on pip/tazo immediately if blood culture positive = 10-14 days of antibiotics if fever continues beyond 14 days = not bacterial, probably fungal
91
when is a child most at risk with leukaemia?
when they first start treatment 7-10 days after (very low WBCs) "nadir phase"
92
Symptoms of anemia
Fatigue Pale Dizzy Heart murmur Tachycardia Pica
93
Causes of anemia
Iron deficiency Blood loss Premature
94
Anemia prevention
Delayed cord clamping Iron fortified cereal Supplements Diet Avoid breast milk
95
When should iron fortified cereal be introduced?
6 months
96
Macrocytic anemia
Large, immature RBCs Iron, B12 and folate deficiency
97
Normocytic anemia
Normal RBCs Blood loss Infection Meds
98
Microcytic anemia
Small RBCs Iron deficiency Medication
99
Reticulocytes
Immature RBCs
100
High reticulocytes
Hemolytic anemia
101
Low reticulocytes
BM dysfunction
102
Anemia treatment
Blood transfusion Nutrition IV fluids O2 therapy Bed rest
103
Recommended limit for cow’s milk
600 mL/day
104
Iron supplement dose
3 mg/kg per day
105
Aplastic anemia
Decreased blood cell production in bone marrow
106
Pancytopenia
All blood cells low
107
Leukopenia
Low WBCs
108
Thrombocytopenia
Low platelets
109
Hypoplastic anemia
Decreased EPO Decreased RBCs Normal WBCs and platelets
110
How is aplastic anemia diagnosed?
CBC Bone marrow aspirate Biopsy
111
How is aplastic anemia treated?
BM transplant Immunosuppression
112
Leukaemia
Cancer of WBCs
113
Lymphoblasts
Immature WBCs
114
How do malignant lymphoblasts affect the immune system?
Decrease function and production of normal cells
115
How does acute leukaemia present on blood work?
Low WBCs Actually more WBCs but less functional
116
Why do we do a lumbar puncture for leukaemia?
To determine CNS involvement
117
Is ALL better in younger or older?
Younger
118
Is AML better in younger or older?
Older
119
When does leukaemia treatment start?
Right after diagnosis
120
How long is a patient cytotoxic after chemo?
7 days
121
What is the nadir phase?
Neutropenia response to chemo Most neutropenic 7-10 days after chemo Most at risk of infection
122
Management for febrile neutropenia
ER immediately Admitted Blood and urine cultures Start IV antibiotics (pip/tazo) Minimum 48 hrs in hospital Must have negative cultures and afebrile for 24 hrs
123
What happens if a febrile neutropenia patient has positive cultures?
Keep on IV antibiotics for 10-14 days
124
What is the work up for a febrile neutropenia patient who is still having fevers?
Cultures q24hrs Start looking for a fungal infection
125
What are the cytotoxic precautions?
Nitrile gloves Double flush toilets and close lid Cytotoxic for 7 days
126
What is lymphoma?
Malignant tumour of lymph nodes and lymph tissue
127
Hodgkin’s Lymphoma
Cancer of lymph tissue Lymph nodes Liver Spleen Bone marrow Reed stenberg cells
128
Non Hodgkins Lymphoma
Cancer of lymph tissue No reed-stenberg cells B or T cells
129
How is lymphoma treated?
Chemo Radiation (sometimes) Bone marrow aspirate or biopsy
130
What drug is often used to treat B cell Non-Hodgkin’s Lymphoma?
Rituximab