Hematological Disorders Flashcards
what is the pathophysiology of iron deficiency anemia?
1) low iron
2) decreased Hgb production
3) decreased oxygen delivery
what are the risk factors for iron deficiency anemia?
inadequate intake
excessive cows milk
premature birth
malabsorption
how does cow’s milk affect iron absorption?
high calcium inhibits iron absorption
what are the signs and symptoms of iron deficiency anemia?
pallor
fatigue
tachycardia
pica
spoon shaped nails (koilonychia)
what are the management interventions for iron deficiency anemia?
iron-rich diet
iron supplements (ferrous sulphate)
what are some iron-rich foods?
red meat
leafy greens
beans
fortified cereal
what are some considerations for iron supplementation?
give between meals
give with vitamin C (orange juice) for better absorption
not with cow’s milk or calcium
increase fibre and fluid
what are some side effects of iron supplements?
black stool
constipation
what is the pathophysiology of SCD?
autosomal recessive disorder
1) RBCs become sickled
2) vasooclusion
3) ischemia
4) organ damage
what are some risk factors for SCD?
cold exposure
stress
hypoxia
dehydration
what are some complications of SCD?
vasooclusive crisis
- ischemia = severe pain
acute chest syndrome
- chest pain
- hypoxia
- fever
- resp distress
splenic sequestration crisis
stroke
hyperhemolysis
- rapid destruction of RBCs
what is splenic sequestration? what can it lead to?
sickled RBCs get trapped in spleen
hypovolemic shock
reduced spell function = risk of infection
what are the management interventions for SCD?
hydration
oxygen
pain relief
hydroxyurea
antibiotics
blood transfusion
vaccinations
what is hydroxyurea?
increases hemoglobin in RBCs
makes RBCs larger, round and more flexible
less likely to become sickle shaped
why should aspirin and NSAIDs be avoided in SCD?
risk of kidney damage
why should cold packs be avoided in SCD?
vasoconstriction worsens sickling
what is the pathophysiology of leukaemia?
overproduction of immature WBCs
leads to bone marrow suppression
what are the signs and symptoms of leukaemia?
anemia
thrombocytopenia
neutropenia
fever
fatigue
dark purple rash
bone pain
hepatosplenomegaly
swollen lymph nodes
bleeding/swollen gums
poor wound healing
abdominal fullness
unexplained bruising
how is leukaemia diagnosed?
bone marrow aspiration/biopsy
peripheral blood smear
lumbar puncture
what are the management interventions for leukaemia?
chemotherapy
cranial irradiation (rare)
blood transfusion
what is tumor lysis syndrome?
cancer cells break down
release substances into blood
what are the signs of tumour lysis syndrome?
hyperkalemia
hyperuricemia
what is the pathophysiology of febrile neutropenia?
neutropenia < 0.5 + fever > 38.3 degrees C
high risk of infection
what are the causes of febrile neutropenia?
chemotherapy
leukemia
BM disorders
infection
- bacterial
- viral
-fungal
what are the signs and symptoms of febrile neutropenia?
fever (earliest)
malaise
chills
what are the management interventions for febrile neutropenia?
immediate IV antibiotics (pip/tazo)
infection precautions
G-CSF to stimulate WBC production
what is the pathophysiology of lymphoma?
DNA mutation cause uncontrollable growth of lymphocytes
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
Hodgkin’s lymphoma is more common among what age group?
adolescents
young adults
Non-Hodgkin’s Lymphoma is more common among what age group?
children
teens
What is the lymph node involvement in Hodgkin’s lymphoma?
painless, firm cervical lymph nodes
What is the lymph node involvement in Non-Hodgkin’s lymphoma?
diffuse lymph node involvement
what are the symptoms of Hodgkin’s lymphoma?
B symptoms
- fever
- night sweats
- weight loss
what are the symptoms of Non-Hodgkin’s lymphoma?
more aggressive B symptoms
- fever
- night sweats
- weight loss
how is Hodgkin’s lymphoma diagnosed?
biopsy of lymph nodes
presence of reed-sternberg cells
how is Non-Hodgkin’s lymphoma diagnosed?
biopsy
CT/MRI for staging
what is the treatment for Hodgkin’s lymphoma?
radiation
chemo (AVBD regimen)
what is the treatment for non-Hodgkin’s lymphoma?
aggressive chemo
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
what is the most common childhood hematological disorder?
anemia
what is anemia?
decreased RBCs or hemoglobin
decreased oxygen carrying capacity
what are the causes of anemia?
manifestations of other disorders
- blood loss
- infection
- autoimmune disorders
- leukemia
- lymphoma
why are premature infants at risk for anemia?
don’t get as much iron rich blood from mom
what causes micro-sized RBCs?
iron deficiency
what are the signs and symptoms of anemia?
pallor
jaundice
swollen liver and spleen
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
when should infants start breastfeeding?
4 months
when should premature infants start breastfeeding?
2 weeks
when should cow’s milk be avoided until?
12 months
what is the limit for cow’s milk per day?
600 mL
why are adolescents at risk for iron deficiency anemia?
rapid growth
poor eating habits
why are indigenous at risk for iron deficiency anemia?
food insecurity
poverty
what is the diet and supplementation requirement for iron deficiency anemia?
3 mg/kg body weight
what are the causes of SCD?
obstruction/destruction of RBCs
vascular inflammation
why is hydration important for SCD?
dehydration causes RBCs to develop sickle shape ad occlude vessels
sickle cells increase urination
how is SCD pain managed?
morphine
PCA pumps
what type of blood transfusion is used for SCD?
exchange
sickled RBCs removed ad replaced with normal RBCs
decreases blood viscosity
why do we give antibiotics for SCD?
spleen isn’t functioning
what kind of antibiotics dose is given for SCD?
low PREVENTATIVE dose to decrease risk
what is the leading cause of death in children?
neoplastic disorders
what is the most common childhood cancer?
leukemia
leukemia is more frequent in __________________
males > 1 year
what is the peak age of leukaemia onset?
2-5 years
leukemia has good ___________
survivability
what are the 3 types of leukaemia?
1) acute lymphoid leukaemia (ALL)
2) acute myeloid leukemia (AML)
3) stem cell/blast cell leukaemia
how does a peripheral blood smear test for leukaemia?
immature leukocytes
low blood cell counts
how does bone marrow aspiration/biopsy test for leukaemia?
from iliac crest
check for blast cells
how does lumbar puncture test for leukaemia?
check CNS involvement
- test CSF for blast cells
what is important for best leukaemia prognosis?
start chemo right away
what factors affect the leukaemia prognosis?
initial WBC count
age
cell type involved
cytogenetics (chromosomes)
immunophenotype
response to induction therapy
gender
relapse
WBC count of _____________ improves prognosis for leukemia
> 50,000
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
what is the best prognosis for leukaemia?
B cell ALL
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)
what gender has better leukaemia prognosis?
girls
what would indicate dismal (poor) prognosis for leukaemia?
relapse after transplant
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
what is acute myeloid leukaemia?
develops from lymphoid stem cells
more common in adults
highly variable prognosis
what are some considerations for chemotherapy?
monitor for tumour lysis syndrome
prevent infection
monitor for oral mucositis
when is radiation used for leukaemia?
only if CNS involvement
what are the indications for a blood transfusion for leukaemia?
haemoglobin < 70 or symptomatic
platelets < 10,000
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
what are the 4 phases of leukaemia therapy?
1) induction
2) CNS prophylaxis
3) intensification
4) maintenance
induction phase of leukaemia therapy
4-6 weeks
to destroy leukaemia cells and induce remission
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)
intensification phase of leukaemia therapy
4-8 months
prevent regrowth and resistant clones
higher doses of chemo
maintenance phase of leukaemia therapy
2-3 years
destroy remaining undetectable leukemia cells
low dose chemo (oral with some IV)
what are neutrophils?
type of white blood cell (leukocyte)
hospitalization protocol for febrile neutropenia
minimum of 48 hours regardless
if no fever for 48h = discharged
after 48h, must go 24h without fever
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
when is a child most at risk with leukaemia?
when they first start treatment
7-10 days after (very low WBCs)
“nadir phase”
Symptoms of anemia
Fatigue
Pale
Dizzy
Heart murmur
Tachycardia
Pica
Causes of anemia
Iron deficiency
Blood loss
Premature
Anemia prevention
Delayed cord clamping
Iron fortified cereal
Supplements
Diet
Avoid breast milk
When should iron fortified cereal be introduced?
6 months
Macrocytic anemia
Large, immature RBCs
Iron, B12 and folate deficiency
Normocytic anemia
Normal RBCs
Blood loss
Infection
Meds
Microcytic anemia
Small RBCs
Iron deficiency
Medication
Reticulocytes
Immature RBCs
High reticulocytes
Hemolytic anemia
Low reticulocytes
BM dysfunction
Anemia treatment
Blood transfusion
Nutrition
IV fluids
O2 therapy
Bed rest
Recommended limit for cow’s milk
600 mL/day
Iron supplement dose
3 mg/kg per day
Aplastic anemia
Decreased blood cell production in bone marrow
Pancytopenia
All blood cells low
Leukopenia
Low WBCs
Thrombocytopenia
Low platelets
Hypoplastic anemia
Decreased EPO
Decreased RBCs
Normal WBCs and platelets
How is aplastic anemia diagnosed?
CBC
Bone marrow aspirate
Biopsy
How is aplastic anemia treated?
BM transplant
Immunosuppression
Leukaemia
Cancer of WBCs
Lymphoblasts
Immature WBCs
How do malignant lymphoblasts affect the immune system?
Decrease function and production of normal cells
How does acute leukaemia present on blood work?
Low WBCs
Actually more WBCs but less functional
Why do we do a lumbar puncture for leukaemia?
To determine CNS involvement
Is ALL better in younger or older?
Younger
Is AML better in younger or older?
Older
When does leukaemia treatment start?
Right after diagnosis
How long is a patient cytotoxic after chemo?
7 days
What is the nadir phase?
Neutropenia response to chemo
Most neutropenic 7-10 days after chemo
Most at risk of infection
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
What happens if a febrile neutropenia patient has positive cultures?
Keep on IV antibiotics for 10-14 days
What is the work up for a febrile neutropenia patient who is still having fevers?
Cultures q24hrs
Start looking for a fungal infection
What are the cytotoxic precautions?
Nitrile gloves
Double flush toilets and close lid
Cytotoxic for 7 days
What is lymphoma?
Malignant tumour of lymph nodes and lymph tissue
Hodgkin’s Lymphoma
Cancer of lymph tissue
Lymph nodes
Liver
Spleen
Bone marrow
Reed stenberg cells
Non Hodgkins Lymphoma
Cancer of lymph tissue
No reed-stenberg cells
B or T cells
How is lymphoma treated?
Chemo
Radiation (sometimes)
Bone marrow aspirate or biopsy
What drug is often used to treat B cell Non-Hodgkin’s Lymphoma?
Rituximab
