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RIA - HAEMATOLOGY > ANAEMIA > Flashcards

ANAEMIA Flashcards

1
Q

What is Indirect Antiglobulin Coomb’s test?

A

Test looking for antibody to patient’s own serum. Used to cross match blood for suitability for transfusion.

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2
Q

How to make diagnosis of hereditary spherocytosis?

A
  • Strong family history
  • Blood film
  • Macrocytic anaemia
  • High bilirubin
  • High reticulocyte count
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3
Q

For Beta Thalassaemia Major patients; how often are the 12 hour subcutaneous desferrioxamine infusions required?

A

3 to 5 times per week

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4
Q

What does a positive indirect antiglobulin Coomb’s test mean?

A

That the tested donor red blood cells are incompatible and should not be given as a transfusion.

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5
Q

When does Beta Thalassaemia major usually present? and why?

A

Usually presents after first 6 months of life. Because during the first 6 months the neonate has HbF (fetal haemoglobin) which gradually reduces. HbF does not contain beta globin chains (instead has gamma globin chains).

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6
Q

HbH disease/Thalassaemia is most often caused by what inheritance?

A

Inherited deletion of 3 of the 4 alpha globin genes.

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7
Q

What does EMA binding test for? and when would you request it?

A

Used to detect hereditary spherocytosis. Request when baby has signs of anaemia and neonatal jaundice.

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8
Q

What inheritance pattern is Hereditary spherocytosis?

A

Autosomal dominant

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9
Q

What is Coomb’s test?

A

there are 2 types; both of which look for antibodies to red blood cells.

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10
Q

What is DAT Coomb’s test?

A

Direct antiglobulin test; less often done, detects antibody to patient’s own serum causing an AIHA.

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11
Q

How often are blood transfusions performed in Beta Thalassaemia major patients?

A

every 4 weeks of life

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12
Q

How is the Beta Thalassaemia trait diagnosed?

A

Raised level of HbA2

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13
Q

What is the main cause of death in Beta Thalassaemia Major disease?

A

Cardiac iron overload.

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14
Q

what 2 medications are given in thalassaemia to prevent iron overload?

A

DEFERIPRONE + DESFERRIOXAMINE

twice weekly

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15
Q

what is the treatment for beta thalassaemia?

A

blood transfusions every 2-4 weeks

to maintain Hb > 90 g/dL + to suppress extramedullary haematopoiesis

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16
Q

what is SIDEROBLASTIC ANAEMIA?

A

Microcytic anaemia caused by the bone marrow producing ringed sideroblasts (instead of healthy red blood cells)

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17
Q

what is the genetics behind SICKLE CELL ANAEMIA?

A

autosomal recessive genetic conditons;

resulting in synthesis of abnormal haemoglobin chain (HbS)

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18
Q

why is sickle cell anaemia more common in the Africo-caribbean population?

A

Heterozygous (carriers) have the HbSHb which offers them protection against Plasmodium Falciparum malaria and they are only affected by the anaemia when severely hypoxic.

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19
Q

In individuals with SICKLE CELL ANAEMIA; what do the HaemoglobinS do in the deoxygenated state?

A

HbS molecules polymerise causing the RBC to sickle and haemolyse –> blockage of small vessels –> infarction.

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20
Q

List 5 symptoms of SICLE CELL ANAEMIA.

A
  • headache
  • pale/yellowish skin
  • malaise
  • SoB + chest pain
  • cold hands/feet
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21
Q

what are the 4 types of sickle cell crisis?

A
  • THROMBOTIC “PAINFUL” “
  • APLASTIC
  • SEQUESTRATION
  • HAEMOLYTIC
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22
Q

what is the management of a thrombotic sick cell crisis?

A
  • Analgesia
  • Fluid rehydration
  • Antibiotics
  • Blood transfusion
  • Oxygen
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23
Q

Name 3 precipitants of a thrombotic sickle cell crisis.

A
  • Dehydration
  • Infection
  • Deoxygenation
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24
Q

What is the pathology of a sequestration sickle cell crisis?

A

HbS sickle within organs (e.g. lungs, spleen) causing a pooling of blood w/ worsening of anaemia.

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25
Q

what is ACUTE CHEST SYNDROME, and what is it caused by?

A

Caused by a sequestration sickle cell crisis, where the HbS sickle within in the lungs causing;

  • dyspnoea
  • chest pain
  • low PO2
  • pulmonary infiltrates
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26
Q

What is the commonest anaemia in hospital patients?

A

Anaemia of chronic disease

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27
Q

What is the treatment of Anaemic of chronic disease?

A

EPO

28
Q

what are the side effects of giving EPO?

A
  • flu like symptoms
  • hypertension
  • mild rise in platelet count
29
Q

Name the medication that increases gamma-globin; thus increasing fetal haemoglobin

A

HYDROXYUREA

30
Q

Name common side effects of HYDROXYUREA;

A
  • bone marrow suppression
  • fever
  • loss of apetite
  • SoB
  • headaches
31
Q

3 Megaloblastic Macrocytic anaemias;

A
  • Vitamin B12 deficiency
  • Folate deficiency
  • Cytotoxic drugs
32
Q

Name 5 non-megaloblastic macrocytic anaemias;

A
  • pregnancy
  • liver disease
  • alcohol
  • reticulocytosis
  • hypothyroidism
33
Q

What is megaloblastic anaemia?

A

Anaemia due to inhibition of DNA synthesis during red blood cell production.

34
Q

Causes of malabsorption causing vitamin B12 deficiency anaemia?

A
  • ilieal resection
  • lack of IF in the stomach
  • chron’s disease
  • bacterial overgrowth
35
Q

what are the dietary requirements of Vitamin B12

A

1 ug / day

36
Q

name some sources of dietary Vitamin B12;

A
  • milk
  • red meat
  • salmon
  • fortified cereals
  • sardines
37
Q

Neuropsychiatric features of Vitamin B12 deficiency;

A
  • depression
  • irritability
  • psychosis
38
Q

Neurological features of Vitamin B12 deficiency;

A
  • paraesthesia

- peripheral neuropathy

39
Q

Ataxia + decreased vision; progressing to stiffness and weakness; can develop in Vitamin B12 deficiency; caused by ?

A

subacute degeneration of spinal cord

40
Q

What is PERNICIOUS ANAEMIA?

A

Autoimmune disease; antibodies to gastric parietal cells leading to lack of IF –> vit. B12 malabsorption

41
Q

2 specific tests for PERNICIOUS ANAEMIA?

A
  • Parietal cell antibodies

- IF antibodies

42
Q

Treatment to replenish Vit.B12 stores in pernicious anaemia?

A

Hydroxocobalamin 1 mg IM

- alternate days for 2 weeks

43
Q

Treatment to maintain Vit B12 stores in pernicious anaemia?

A

Hydroxocobalamin 1 mg IM

- every 3 months for life

44
Q

IMENSLUND-GRASPECK SYNDROME?

A

Rare autosomal recessive familial form of vitamin B12 deficiency –> malfunction of cuban receptor in terminal ileum

45
Q

what are the daily requirements of folate?

A

100-200 ug

46
Q

name 4 dietary sources of folate;

A
  • liver
  • leafy vegetables
  • nuts
  • yeast
47
Q

name 2 drugs that can cause folate deficiency anaemia

A

methotrexate

anticonvulsants

48
Q

when awaiting a diagnosis for megaloblastic macrocytic anaemia; treatment ?

A

HYDROXOCOBALAMIN 1mg/48hrs IM
+
FOLIC ACID 5mg/24hrs PO

49
Q

Treatment for folate deficiency anaemia?

A

Folic acid 5mg daily PO

for 4 months

50
Q

Chronic blood loss causes ____ anaemia

A

macrocytic

51
Q

Acute blood loss causes ____ anaemia

A

normocytic

52
Q

Why does acute blood loss cause normocytic anaemia?

A

Blood loss occurs –> it takes several hours for plasma to expand –> therefore rapid blood loss will only happen when the loss of blood volume is replaced either by natural plasma expansion or replacement with non-RBC containing blood products.

53
Q

Name 3 RBC membrane defects;

A
  1. HEREDITARY SPHEROCYTOSIS
  2. ELLIPTOCYTOSIS
  3. HEREDITARY STOMATOCYTOSIS
54
Q

A RBC enzyme defect;

A

G-6-PD deficiency

55
Q

2 Haemoglobin disorders;

A
  1. SICKLE CELL DISEASE

2. THALASSAEMIA

56
Q

____ ___ anaemia has a positive direct coomb’s test?

A

autoimmune haemolytic anaemia

57
Q

Increased; unconjugated bilirubin + urinary urobilinogen

is a sign of _____?

A

increased RBC destruction

58
Q

which AIHI is IgG antibody medicated?

A

WARM AIHI

59
Q

1st line treatment for warm AIHI?

A

corticosteroids

60
Q

Treatment for cold AIHI?

A
  • avoid cold

- RITUXIMAB

61
Q

due to progressive destruction of the spleen in sickle cell anaemia; patients are at an increased risk of infection with encapsulated bacteria; give examples of these;

A
  • HAEMOPHILIUS INFLUENZAE
  • STREPTOCOCCUS PNEUMONIAE
  • NEISSERIA MENINGITIS
  • SALMONELLA
62
Q

what prophylactic treatment is given to children with sickle cell disease; to prevent infection with encapsulated bacteria ?

A

PENICILLIN
+
POLYSACCHARIDE VACCINE

63
Q

15% women with heavy periods have ___

A

vWD

64
Q

what is HbH most often caused by?

A

inherited deletion of 3 of the 4 alpha globin genes

65
Q

what is SUBCUTANEOUS DESFERRIOXAMINE used for?

A

iron chelation

12 hour infusions required 3 to 5 times per week

RIA - HAEMATOLOGY (6 decks)

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