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Path - Haematology > Anaemias and Haemolytic Anaemias > Flashcards

Anaemias and Haemolytic Anaemias Flashcards

1
Q

What is the life span of a RBC?

A

120 days

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2
Q

What is haemolysis?

A

Shortened RBC survival

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3
Q

What is haemolytic anaemia?

A

Anaemia (low RBC) due to shorter RBC survival

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4
Q

How do you classify haemolytic anaemias based on where they die?

A

Intravascular (within circulation)

Extravascular (removal/destruction by reticuloendothelial system)

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5
Q

Give examples of causes of intravascular anaemia

A
  • Malaria (most common)
  • G6PD deficiency
  • Mismatched blood transfusion
  • Cold antibody haemolytic syndromes
  • Drugs (DAPSONE)
  • Microangiopathic haemolytic anaemia (HUS, TTP)
  • Paroxysmal nocturnal haemoglobinuria
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6
Q

Give examples of causes of extravascular anaemia

A

Autoimmune
Alloimmune
Hereditary spherocytosis

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7
Q

What type of intravascular anaemia offers protection against malaria?

A

G6PD deficiency

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8
Q

What is the aetiology of Paroxysmal nocturnal haemoglobinuria?

A

Acquired genetic defect in GPI anchor

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9
Q

How can you divide hereditary haemolytic anaemias?

A
  • MEMBRANE (cytoskeletal proteins, cation permeability)
  • Red cell metabolism (glycolysis)
  • Haemoglobin (thalassaemia, sickle cell, unstable Hb variant)
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10
Q

What are consequences of haemolytic anaemia?

A

Anaemia
Erythroid hyperplasia (increased RBC production, increased reticulocytes)
Increased folate demand
Susceptibility to parvovirus (aplastic crisis)
Propensity to gallstones
Increased risk of iron overload
Increased risk of osteoporosis

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11
Q

What does parvovirus do to RBC?

A

It infects RBC precursors in the bone marrow, arresting their maturation (prevents iron uptake)
In someone with shortened RBC span, this causes dangerously low Hb > APLASTIC CRISIS

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12
Q

Which disease increases propensity for gallstones in haemolytic anaemia?

A

Gilbert’s

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13
Q

What is the mutation in Gilbert’s?

A

UGT1A1 TA7

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14
Q

What are clinical features of haemolytic anaemia?

A

Pallor
Jaundice
Splenomegaly
Pigmenturia (abnormal urine colour)

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15
Q

What are lab features of haemolytic anaemia?

A
  • Anaemia (low RBC)
  • High reticulocytes
  • Polychromasia
  • Increased LDH
  • Reduces/absent haptoglobin
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16
Q

Which findings imply intravascular haemolysis?

A

haemoglobinuria

haemosiderinaemia

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17
Q

Hereditary spherocytosis occurs due to what kind of disorder?

A 
VERTICAL interaction 
Band 3
Protein 4.2
Ankyrin 
Beta spectrin
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18
Q

Hereditary elliptocytosis occurs due to what kind of disorder?

A

HORIZONTAL interaction
alpha spectrin
beta spectrin
Protein 4.1

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19
Q

What tests can you do to detect hereditary spherocytosis?

A

OSMOTIC FRAGILITY TEST
RBC show increased sensitivity to lysis in hypotonic saline

DYE BINDING TEST
Shows reduced binding of the dye (eosin 5 maleiminde)

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20
Q

What does hereditary spherocytosis look like on blood film?

A

They lack the area of central pallor

This is because they have lost the biconcave shape

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21
Q

What does hereditary elliptocytosis look like on blood film?

A

Oddly shaped, but NO polychromasia

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22
Q

How is G6PD deficiency transmitted?

A

X linked recessive

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23
Q

What is the function of G6PD enzyme?

A

It reduces NADP+ to NADPH

This is to generate gluthianone, which protects RBC against oxidative stress ,

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24
Q

What occurs when G6PD is deficient?

A

RBC become vulnerable to oxidative stress > haemolysed

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25
Q

What are broad clinical effects of G6PD deficiency?

A

Neonatal jaundice
Acute haemolytic
Chronic haemolytic anaemia

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26
Q

What are most people with G6PD like?

A

Asymptomatic

Until exposed to trigger, causing intravascular haemolysis

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27
Q

What are triggers for G6PD ?

A

Drugs (antimalarials, antibiotics, analgesics, dapsone, VitK)
Infection
Fava beans, soy
Naphthalene

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28
Q

What shows up on blood film of G6PD deficiency ?

A

In acute haemolysis:

  • Heinz bodies
  • Bite cells
  • Contracted cells. nucleated red cells, hemighosts

In steady state:
- NORMAL

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29
Q

What stain do you use to detect Heinz bodies?

A

methylviolet

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30
Q

What shows up on blood film with pyruvate kinase deficiency?

A

ECHINOCYTES (RBC with short projections) - spiky

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31
Q

What shows up on blood film with pyrimidine 5 nucleotidase deficiency?

A

Basophilic stippling

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32
Q

What are first line investigations for haemolytic anaemias?

A

Direct antiglobulin test > AI haemolysis
Urinary haemosiderin/Hb > IV haemolysis
Osmotic fragility test /dye binding > hereditary spherocytosis
G6PD/PK activity
Heinz Body stain
Ham’s test/Flow cytometry for GPI linked proteins >paroxysmal nocturnal haemoglobinuria

33
Q

How do you manage haemolytic anaemias?

A 
Folic acid supplement 
Avoid precipitating factors 
RBC transfusion/exchange 
Immunisation against blood borne viruses 
Monitor for chronic complications 
Cholecystectomy for gallstones 
Splenectomy if indicated
34
Q

What are specific criteria for splenectomy

A 
Transfusion dependence 
growth delay 
physical limitation
hypersplenism 
age between 3.10
35
Q

What stain shows Heinz bodies?

A

methylviolet

36
Q

what are causes of microcytic anaemia

A

Iron deficiency
Anaemia chronic disease
Sideroblastic
THalassaemia

37
Q

What are causes of normocytic anaemia

A 
Acute blood loss 
ACD 
BM failure 
Renal failure 
haemolysis 
Pregnancy
38
Q

WHat are causes of macrocytic anaemia

A

Megaloblastic: B12/folate

Non-megaloblastic: hypothyroidism, liver failure, myelodysplastic syndrome

39
Q

What are signs of iron def anaemia

A

Koilonychia, atropgic glossigtis, angular cheilosis, post-cricoid webs (Plummer-Vinson)

40
Q

What shows up on blood film of IDA?

A 
Microcytic 
Hypochromic 
Anisocytosis 
Poikilocytosis 
Pencil cells
41
Q

What does anisocytosis mean

A

varying in size

42
Q

What does poikilocytosis mean

A

varying in shape

43
Q

Explain causes of iron def anaemia

A
  • Blood loss (menorrhagia in women, GI bleed in elderly)
  • increased use (pregnancy, lactation, infants)
  • decreased intake (prematurity, poor diet)
  • decreased absorption (coeliac, post-gastric surgery)
44
Q

How do you treat iron def anaemia

A

Treat the cause

+ oral ferrous sulphate

45
Q

What must you be aware of when giving ferrous sulphate in sepsis

A

Do NOT give as in sepsis iron does not absorb well and it fuels infection

Blood transfusions are PREFERABLE

46
Q

How does ACD occur

A

cytokines drive inhibition of RBC production

47
Q

What are causeas of ACD

A

Chronic infection
Vasculitis
RA
malignancy

48
Q

What are iron studies like in ACD and why

A 
Low iron 
High ferritin (because of high stores, as the iron is all sequestered) 
Low TIBC (as all the transferrin is already used up=
49
Q

What are iron studies like in IDA and wqhy

A

Low iron
Low ferritin
High TIBC (as no transferrin is used up yet)

50
Q

What is sideroblastic anaemia

A

occurrence of ineffective erythropoesis > iron (instead of forming a proper RBC) deposits around the erythroid precursor forming a singed sideroblast

all this iron also causes haemosiderosis (iron deposition in endocrine, liver, cardiac tissue causing damage)

51
Q

What are iron studies like in sideroblastic anaemia

A

High iron
Normal TIBC (as it is the body that is unable to merge iron properly)
High ferritin

52
Q

What else can cause elevation of ferritin

A

Infectection / inflammation

Because ferritin is an acute phase proteinn

53
Q

What food is B12 found in

A

meat and diary

54
Q

What causes B12 deficiency

A

Insuff dietary uptake (vegans)
Malabsorption
- in stomach: lack of intrinsic factor to transpoort it to the terminal ileum > pernicious anaemia
- in terminal ileum : Chron’s, ileal resectio

55
Q

Explain pernicious anaemia and the most common antibodies

A

Autoimmune atrophic gastritis > lack of intrinsic factor in the stomach

  • Parietal cell antibodies 90%
  • Intrinsic factor antibodies 50%
56
Q

What other test other than antibodies can you do for pernicious anaemia

A

SCHILLING TEST

57
Q

How do you manage B12 deficiency

A

Replenish B12 stores with IM HYDROXYCOBALAMIN

58
Q

What are clinical fts of B12 def

A

glossitis, angular chhelosis
neuro: irritability, depression
PARASTHHESIA; PERIPHERAL NEUROPATHY

59
Q

What kinds of food is folate found in

A

green venetables, nuts

60
Q

What is needed to synthesise folate from the food

A

gut bacteria

61
Q

what are causes of folate deficiency

A

poor diet
increased folate demand (pregnancy, high cell tunrover9
malabsorption
alcohol

62
Q

How do you manage folate deficiency

A

oral folic acid

63
Q

if a patient has both B12 and folate deficiency, which shoudl you correct first and wh

A

B12 first

If you correct folate first, B12 neuropathy will get wotse

64
Q

What are lab features of ALL haemolytic anaemia

A

high bilirubin
high urobilinogen
high LDH
high reticulocytes

65
Q

What is the clinical ft of extravasc haemolytic anameia

A

SPLENOMEGALY

as haemolysis occurs in the splee

66
Q

What are the lab fts of intravasc haemolytic anaemia

A

high free plasba Hb
Low haptoglobin
haemoglobinuria
albumin + haem in urine

67
Q

what are awquired causes of haemolytic anaemia

A

IMMUNE

  • autoimmune (warm/cold)
  • Alloimmune (haemolytic transfusion reaction()

NON-IMMUNE

  • mechanical e.g. metal valves, trauma
  • PNH, MAHA
  • infections e.g. malaria
68
Q

What are examples of autoimmune anaemias

A

warm/cold AIHA

69
Q

Explain features of warm haemolytic anaemia

A

37 degrees
IgG (GHANA)
positive coombs
Spherocytes on blood film

70
Q

explain features of cold haemolytic anaemia

A

<37 deegrees
IgM MOUNTAINS
Positive coombs
Raynauds

71
Q

How fo you manage Warm AIHA

A

steroids
splenectomy
immunosuppression

72
Q

How do you manage cold AIHA

A

treat underlying condition
avoid the cold
Chlorambucil (chemo)

73
Q

Explain paroxysmal cold haemoglobiniurea

A

haemoglobin in urine caused by a viral infection
this leads to formation of DOnath-Landsteiner antibodies > stick to RBC in cold > complemented mediated haemolysis on rewarming

74
Q

what are causes of Warm AIHA

A

IDIOPATHIC mainly

lymphoman
CLL
SLE
Methyldopa

75
Q

What are causes of Cold AIHA

A

IDIOPATHIC mainly

lymphoma
infections e.g. EBV, mycoplasma

76
Q

Explain cause of paroxysmal nocturla haemoglobinuri

A

AQUIRED loss of protective surface GPI markers on RBC

This causes complement mediated lysis > chronic IV haemolysis especially at night

77
Q

What are features of PNH

A

morning haemoglobinuria n

thrombosis

78
Q

How do you diagnose PNH (what tests)

A

HAM tests (in vitro acid induced lysis)

OR

IOmmunophenotype to show altered GO

79
Q

GHow do you manage PNH

A

iron/folate supplements

prophylactic vaccines and antibiotis cs

Path - Haematology (17 decks)

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