lecture 15 - classification and treatment of haematological disorders

Question 1

what are the components of blood?

Answer 1

plasma 55%

blood cells 45%
three types
- erythrocytes/ RBCs
- leukocytes/ WBCs
thrombocytes/ platelets

Question 2

describe erythrocytes/ red blood cells composed of? what is erythropoiesis?

Answer 2

composed of haemoglobin

erythropoiesis
- = RBC production
- stimulated by hypoxia
- controlled by erythropoietin - hormone synthesised in kidney

Question 3

what is haemolytic anaemia ?

Answer 3

haemolytic
-destruction of RBCs
- Releases bilirubin into the blood stream
- normal lifespan of RBC = 120 days

Question 4

what is the haemostasis - aim of thrombocytes/ platelets and describe factors which carry this out

Answer 4

haemostasis: the stoppage of blood flow
goal is to minimise blood loss when injured

1. vascular response
   - vasoconstriction
2. platelet response
   - activated during injury
   - form clumps (agglutination)
3. plasma clotting factors
   - factors I - XIII
   - intrinsic pathway
   - extrinsic pathway

Question 5

what is anticoagulation?

Answer 5

elements that interfere with blood clotting

counter mechanism to blood clotting - keeps blood liquid able to flow
- aspirin
- clopidogrel
- warfarin
- heparin
-NOACs/ DOACs

Question 6

how is the assessment of haemotologic system (risk of patient clotting or bleeding) carried out?

Answer 6

subjective data
- important health information:
past health history
medications
surgery or other treatments

objective data:
- physical examination
skin
eyes
mouth
lymph nodes
heart and chest
abdomen
nervous system
musculoskeletal system

Question 7

what is examined in the diagnostic studies of the haemotologic system: whole blood count?

Answer 7

white blood cells
red blood cells
haematocrit
platelet count
pancytopenia

Question 8

describe anaemia - what is it? function? drives?

Answer 8

anaemia is a reduction in the number of RBCs, the quantity of haemoglobin or the volume of RBCs

because the main function of RBCs is oxygenation, anaemia results in varying degrees of hypoxia

drives ischemiac pathologies

Question 9

what are common conditions in anaemia?

Answer 9

blood loss

decreased production of erythrocytes

increased destruction of erythrocytes

Question 10

what are clinical ways that anaemia can appear in a patient?

Answer 10

pallor

fatigue, weakness

dyspnea

palpitations, tachycardia

headache, dizziness, and restlessness

slowing of thought

parenthesia

Question 11

describe ways in the management of anaemia.

Answer 11

1. direct general management toward addressing the cause of anaemia and replacing blood loss as need to sustain adequate oxygenation
2. promote optimal activity and protect from injury
3. reduce activities and stimuli that cause tachycardia and increase cardiac output
4. provide nutritional needs
5. prescribed nutritional supplements

Question 12

what are actions for a patient who is anaemia or suffered blood loss?

Answer 12

oxygen (prescribed)
blood products (prescribed)
erythropoietin (prescribed)

diet advice
iron, folic acid, B12 supplementation

Question 13

what are the causes of decreased erythrocyte production or iron deficiency anaemia ?

Answer 13

1. inadequate dietary intake
2. malabsorption
   - absorbed in duodenum
   -GI surgery
3. blood loss
   - 2mls blood contain 1mg iron
   - GI, GU losses
4. haemolysis

Question 14

what are ways that a patient can present iron deficiency anaemia?

Answer 14

most common: pallor
second most common: inflammation of the tongue (glossitis)
chelations = inflammation/ fissures of lips
weakness and fatigue

Question 15

how is iron deficiency treated?

Answer 15

collaborative care
- treatment of underlying disease/ problem
- replacing iron
- diet
- drug therapy

iron replacement
- oral iron such as ferrous fumurate, absorbed best in acidic environment, GI effects

parenteral iron
- IM or IV
less desirable than PO

Question 16

how is iron deficiency managed?

Answer 16

assess cardiovascular and respiratory status

monitor vitals signs

recognising s/s/ bleeding
- monitor stool, uine and emesis for occult blood

diet teaching - foods rich in iron

provide periods of rest

supplemental iron

discuss diagnostic studies

emphasise compliance

iron therapy for 2-3 months after the haemoglobin levels return to normal

Question 17

what are megaloblastic anaemias? and what are common forms of it?

Answer 17

characterized by large RBCs which are fragile and easily destroyed

common forms of megaloblastic anaemia are
1. cobalamin deficiency
2. folic acid deficiency

Question 18

what is cobalamin vitamin B12 deficiency?

Answer 18

cobalamin deficiency also known as pernicious anaemia

vitamin B12 is an important water-soluble vitamin

intrinsic factor is required for cobalamin absorption

Question 19

what are causes of cobalamin deficiency?

Answer 19

gastric mucosa not secreting IF

GI surgery –> loss of IF secreting gastric mucosal cells
long term use of H2 histamine receptor blockers cause atrophy or loss of gastric mucosa

nutritional deficiency

hereditary defects of cobalamin utilisation

Question 20

what are clinical manifestations of cobalamin vitamin B12 deficiency?

Answer 20

general symptoms of anaemia

sore tongue

anorexia

weakness

parenthesis of the feet and hands

altered through processes
confusion –> pseudo dementia

Question 21

what are the diagnostic studies of cobalamin deficiency?

Answer 21

RBCs appear large

abnormal shapes

structure contributes to erythrocyte destruction

Question 22

how is collaborative care used in cobalamin deficiency ?

Answer 22

parenteral administration of cobalamin

increased dietary cobalamin does not correct the anaemia - still important to emphasise adequate dietary intake

intranasal form of cyanocobalaim (nascobal) is available

high dose oral cobalamin and SL cobalamin can be used

Question 23

how is cobalamin deficiency managed?

Answer 23

Familial disposition
- Early detection and treatment can lead to reversal of symptoms

• Potential for Injury related to patient’s diminished sensations to heat and pain

Compliance with medication regime

Ongoing evaluation of GI and neuro status
Evaluate patient for gastric carcinoma frequently

Question 24

what does folic acid deficiency cause? what are the causes of folic acid deficiency?

Answer 24

folic acid deficiency also causes megaloblastic anaemia (RBCs that are large and fewer in number)

folic acid required for RBC formation and maturation

causes
poor dietary intake
malabsorption syndromes
drugs that inhibit absorption
alcohol abuse
hemodialysis

Question 25

what is folic acid deficiency treated by and what is the patients encouraged to eat?

Answer 25

treated by folate replacement therapy

encourage patients to eat foods with large amounts of folic acid
- leafy green vegetables
liver
mushrooms
oatmeal
peanut butter
red beans

Question 26

what is chronic anaemia disease and what is it caused by?

Answer 26

Underproduction of RBCs, shortening of RBC survival

Causes
Impaired renal function
Chronic, inflammatory, infectious or malignant disease
Chronic liver disease
Folic acid deficiencies
Splenomegaly
Hepatitis

Question 27

what is anaemia caused by increased erythrocyte destruction

Answer 27

haemolytic anemia
- sickle cell disease

acquired haemolytic anaemia

haemochromatosis

polycythemia