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Path - Haematology > Anaemias and Haemolytic Anaemias > Flashcards

Anaemias and Haemolytic Anaemias Flashcards

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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
What are broad clinical effects of G6PD deficiency?
Neonatal jaundice Acute haemolytic Chronic haemolytic anaemia
26
What are most people with G6PD like?
Asymptomatic | Until exposed to trigger, causing intravascular haemolysis
27
What are triggers for G6PD ?
Drugs (antimalarials, antibiotics, analgesics, dapsone, VitK) Infection Fava beans, soy Naphthalene
28
What shows up on blood film of G6PD deficiency ?
In acute haemolysis: - Heinz bodies - Bite cells - Contracted cells. nucleated red cells, hemighosts In steady state: - NORMAL
29
What stain do you use to detect Heinz bodies?
methylviolet
30
What shows up on blood film with pyruvate kinase deficiency?
ECHINOCYTES (RBC with short projections) - spiky
31
What shows up on blood film with pyrimidine 5 nucleotidase deficiency?
Basophilic stippling
32
What are first line investigations for haemolytic anaemias?
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
How do you manage haemolytic anaemias?
``` 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
What are specific criteria for splenectomy
``` Transfusion dependence growth delay physical limitation hypersplenism age between 3.10 ```
35
What stain shows Heinz bodies?
methylviolet
36
what are causes of microcytic anaemia
Iron deficiency Anaemia chronic disease Sideroblastic THalassaemia
37
What are causes of normocytic anaemia
``` Acute blood loss ACD BM failure Renal failure haemolysis Pregnancy ```
38
WHat are causes of macrocytic anaemia
Megaloblastic: B12/folate | Non-megaloblastic: hypothyroidism, liver failure, myelodysplastic syndrome
39
What are signs of iron def anaemia
Koilonychia, atropgic glossigtis, angular cheilosis, post-cricoid webs (Plummer-Vinson)
40
What shows up on blood film of IDA?
``` Microcytic Hypochromic Anisocytosis Poikilocytosis Pencil cells ```
41
What does anisocytosis mean
varying in size
42
What does poikilocytosis mean
varying in shape
43
Explain causes of iron def anaemia
- 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
How do you treat iron def anaemia
Treat the cause | + oral ferrous sulphate
45
What must you be aware of when giving ferrous sulphate in sepsis
Do NOT give as in sepsis iron does not absorb well and it fuels infection Blood transfusions are PREFERABLE
46
How does ACD occur
cytokines drive inhibition of RBC production
47
What are causeas of ACD
Chronic infection Vasculitis RA malignancy
48
What are iron studies like in ACD and why
``` 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
What are iron studies like in IDA and wqhy
Low iron Low ferritin High TIBC (as no transferrin is used up yet)
50
What is sideroblastic anaemia
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
What are iron studies like in sideroblastic anaemia
High iron Normal TIBC (as it is the body that is unable to merge iron properly) High ferritin
52
What else can cause elevation of ferritin
Infectection / inflammation Because ferritin is an acute phase proteinn
53
What food is B12 found in
meat and diary
54
What causes B12 deficiency
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
Explain pernicious anaemia and the most common antibodies
Autoimmune atrophic gastritis > lack of intrinsic factor in the stomach - Parietal cell antibodies 90% - Intrinsic factor antibodies 50%
56
What other test other than antibodies can you do for pernicious anaemia
SCHILLING TEST
57
How do you manage B12 deficiency
Replenish B12 stores with IM HYDROXYCOBALAMIN
58
What are clinical fts of B12 def
glossitis, angular chhelosis neuro: irritability, depression PARASTHHESIA; PERIPHERAL NEUROPATHY
59
What kinds of food is folate found in
green venetables, nuts
60
What is needed to synthesise folate from the food
gut bacteria
61
what are causes of folate deficiency
poor diet increased folate demand (pregnancy, high cell tunrover9 malabsorption alcohol
62
How do you manage folate deficiency
oral folic acid
63
if a patient has both B12 and folate deficiency, which shoudl you correct first and wh
B12 first If you correct folate first, B12 neuropathy will get wotse
64
What are lab features of ALL haemolytic anaemia
high bilirubin high urobilinogen high LDH high reticulocytes
65
What is the clinical ft of extravasc haemolytic anameia
SPLENOMEGALY as haemolysis occurs in the splee
66
What are the lab fts of intravasc haemolytic anaemia
high free plasba Hb Low haptoglobin haemoglobinuria albumin + haem in urine
67
what are awquired causes of haemolytic anaemia
IMMUNE - autoimmune (warm/cold) - Alloimmune (haemolytic transfusion reaction() NON-IMMUNE - mechanical e.g. metal valves, trauma - PNH, MAHA - infections e.g. malaria
68
What are examples of autoimmune anaemias
warm/cold AIHA
69
Explain features of warm haemolytic anaemia
37 degrees IgG (GHANA) positive coombs Spherocytes on blood film
70
explain features of cold haemolytic anaemia
<37 deegrees IgM MOUNTAINS Positive coombs Raynauds
71
How fo you manage Warm AIHA
steroids splenectomy immunosuppression
72
How do you manage cold AIHA
treat underlying condition avoid the cold Chlorambucil (chemo)
73
Explain paroxysmal cold haemoglobiniurea
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
what are causes of Warm AIHA
IDIOPATHIC mainly lymphoman CLL SLE Methyldopa
75
What are causes of Cold AIHA
IDIOPATHIC mainly lymphoma infections e.g. EBV, mycoplasma
76
Explain cause of paroxysmal nocturla haemoglobinuri
AQUIRED loss of protective surface GPI markers on RBC | This causes complement mediated lysis > chronic IV haemolysis especially at night
77
What are features of PNH
morning haemoglobinuria n | thrombosis
78
How do you diagnose PNH (what tests)
HAM tests (in vitro acid induced lysis) OR IOmmunophenotype to show altered GO
79
GHow do you manage PNH
iron/folate supplements | prophylactic vaccines and antibiotis cs

Path - Haematology (17 decks)

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