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Phase 2A HT Notes > Haematology > Flashcards

Haematology Flashcards

1
Q

What is the term and range for low neutrophils?

A

Neutropenia
< 2 x 10^9 / L

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2
Q

What can cause neutropenia?

A

Myeloma
Lymphoma

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3
Q

What is the term and range for high neutrophils?

A

Neutrophilia
> 7.5 x 10^9 / L

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4
Q

What can cause neutrophilia?

A

Acute bacterial infection

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5
Q

What is the term and range for low lymphocytes?

A

Lymphocytopenia
< 1.3 x 10^9 / L

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6
Q

What is the term and range for high lymphocytes?

A

Lymphocytosis
> 3.5 x 10^9 / L

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7
Q

What can cause lymphocytosis?

A

Chronic bacterial infection

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8
Q

What is the term and range for high platelets?

A

Thrombocytosis
> 400 x 10^9 / L

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9
Q

What is the term and range for low platelets?

A

Thrombocytopenia
< 150 x 10^9 / L

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10
Q

What leukocyte is elevated in parasitic infection?

A

Eosinophils

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11
Q

Why might monocytes be elevated?

A

Myelodysplastic syndromes

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12
Q

What 4 measurements are included in an iron study?

A

Serum Fe - Level of circulating blood bound to transferrin
Transferrin - The main Fe transport protein
Total iron binding capacity (TIBC) - Blood’s ability to attach to iron
Ferritin - Storage form of iron

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13
Q

Which iron study measurement is the best test for iron deficiency anaemia?

A

Ferritin

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14
Q

Why might TIBC be increased?

A

Iron deficiency

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15
Q

Why might TIBC be decreased?

A

Haemochromatosis

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16
Q

Which measurements are included in a clotting screen?

A

Prothrombin time (PT) / International Normalised Ratio (INR)
Activated Partial Thromboplastin Time (APTT)

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17
Q

How is INR calculated?

A

Patient PT / Reference PT

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18
Q

When is INR used instead of PT?

A

INR is used to monitor patients who are being treated with blood-thinning medication (such as Warfarin)

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19
Q

Prothrombin Time measures the coagulation speed through which pathway? Which factors are involved in this pathway?

A

External pathway
Factors 3 and 7

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20
Q

Activated Partial Thromboplastin Time measures the coagulation speed through which pathway? Which factors are involved in this pathway?

A

Internal pathway
12, 11, 9 and 8

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21
Q

What is normal PT?

A

10 - 13.5s

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22
Q

What is normal INR for a normal patient? What about if the patient is on Warfarin?

A

0.8 - 1.2
Warfarin: 2 - 3

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23
Q

What is normal APTT? What about if the patient is on heparin?

A

35 - 45s
Heparin: 60 - 80s

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24
Q

Why might INR be raised?

A

Anticoagulation
Liver disease
Vitamin K deficiency
Disseminated intravascular coagulation (DIC)

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25
Q

Why might APTT be raised?

A

Haemophilia A (F8)
Haemophilia B (F9)
Von Willebrand Factor disease

In these conditions, PT will be normal and APTT will be prolonged

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26
Q

What do bleeding time and thrombin time measure?

A

How long it takes for fibrinogen to be converted to fibrin

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27
Q

What is normal thrombin time?

A

12 - 14s

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28
Q

Which factors are involved in the common pathway?

A

10, 5, 2 and 1

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29
Q

Prothrombin is the name for which factor? Which pathway is it a part of?

A

2
Common pathway

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30
Q

Fibrinogen is the name for which factor? Which pathway is it a part of?

A

1
Common

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31
Q

Define anaemia

A

Haemoglobin < 120g/L (in females) or < 130g/L (in males)

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32
Q

When should blood transfusion be considered for anaemia?

A

If Hb < 70g/L
OR Hb < 80g/L with cardiac comorbidity

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33
Q

What are the 3 types of anaemia? How are they classified?

A

Microcytic: MCV < 80
Normocytic: MCV 80-95
Macrocytic: MCV > 95

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34
Q

List some causes of Microcytic anaemia. Which is the most common worldwide??

A

Fe deficiency (MOST COMMON)
Thalassemia
Chronic disease
Sideroblastic anaemia

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35
Q

What are the 2 types of Normocytic anaemia? How are they different

A

Haemolytic and non-haemolytic

Haemolytic: High rate of RBC death BUT good bone marrow response - high reticuloytes
Non-haemolytic: Failing bone marrow - low reticulocytes

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36
Q

List some causes of haemolytic anaemia

A

Sickle cell
G6DPH deficiency
Autoimmune haemolytic anaemia
Hereditary spherocytosis

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37
Q

List some causes of non-haemolytic anaemia

A

Aplastic syndrome
Chronic disease (eg. CKD)
Myelopathic process
Pregnancy

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38
Q

What are the 2 types of macrocytic anaemia? How are they different?

A

Megaloblastic - Affect DNA synthesis
Non Megaloblastic - Don’t affect DNA synthesis

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39
Q

List 2 causes of Megaloblastic anaemia

A

B12 deficiency
Folate deficiency

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40
Q

List 2 causes of non Megaloblastic anaemia

A

Alcohol
HypOthyroidism
NAFLD

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41
Q

List the general symptoms of anaemia

A

FATIGUE
PALLOR
CHEST PAIN
Exertional dyspnoea
Tachycardia
Hypotension
Palpitations

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42
Q

Define iron deficiency anaemia

A

A non inherited iron deficiency, impairing Hb synthesis

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43
Q

Which type of anaemia is most common worldwide?

A

Iron deficiency anaemia

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44
Q

What are the causes of iron deficiency anaemia for infants, children and adults?

A

Infants: Malnutrition, prolonged breastfeeding

Children: Malnutrition, malabsorption (eg. IBD, coeliac)

Adults: Malnutrition, malabsorption, menorrhagia, hookworm

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45
Q

What is the most common cause of iron deficiency anaemia worldwide? How does it cause anaemia?

A

Hookworm
Causes GI bleeding

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46
Q

In which age group is iron deficiency anaemia a cause for concern? Why?

A

The elderly (60+)
Iron def anaemia is rare in the elderly, and is a red flag for colon cancer

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47
Q

What action should be taken if an elderly person has iron deficiency anaemia?

A

Urgent endoscopy should be ordered

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48
Q

What are the symptoms of iron deficiency anaemia?

A

The same as general anaemia

Fatigue
Pallor
Chest pain
Exertional dyspnoea
Tachycardia
Hypotension
Palpitations

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49
Q

List 3 signs of iron deficiency anaemia

A

Kalonchya (spoon shaped nails)
Angular stomatitis (ulceration at mouth corners)
Atrophic glossitis (enlarged tongue)

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50
Q

List the investigations for iron deficiency anaemia and what they show

A

FBC = Microcytic anaemia
Blood film = Hypochromic RBCs, target cells + Howell Jolly bodies
Iron studies = Decreased serum Fe, ferritin and transferrin saturation. Increased TIBC
Endoscopy if 60+

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51
Q

Describe the appearance of target cells

A

Bullseye pattern

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52
Q

What eponymous finding is seen on a blood film in iron deficiency anaemia?

A

Howell Jolly bodies

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53
Q

What is the first line Tx for Fe deficiency anaemia? (Apart from treating any underlying cause)

A

Oral iron supplements (ferrous sulphate)

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54
Q

List some side effects of oral Fe supplements. What can be given instead?

A

GI upset, diarrhoea/constipation, black stool

Ferrous gluconate can be given if ferrous sulphate is poorly tolerated

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55
Q

Describe the pathology of iron deficiency anaemia

A

Iron is usually absorbed and circulates in blood bound to transferrin
It is then either stored as ferritin or incorporated into Hb
Less iron means reduced Hb synthesis causing Microcytic anaemia

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56
Q

How is Thalassemia inherited?

A

Autosomal recessive

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57
Q

Define Thalassemia

A

Autosomal recessive haemoglobinopathy

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58
Q

Where is Thalassemia most prevalent? Why?

A

In areas where malaria is prevalent (Mediterranean, SE Asia and Pacific)
Thalassemia is protective against Plasmodium falciparum malaria - similar to Sickle Cell

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59
Q

What are the 2 types of Thalassemia? What are the differences?

A

Alpha and beta Thalassemia

Alpha - DELETION of up to 4 of the 4 alpha globin genes on chromosome 16

Beta (more common!!) - MUTATION of one or both beta globin genes on chromosome 11

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60
Q

What are the symptoms of beta Thalassemia major (ie homozygous genotype)?

A

General anaemia symptoms
Failure to thrive
Hepatosplenomegaly
RUQ (gallstones are a common presenting feature)
Chipmunk face (enlarged forehead and cheekbones)

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61
Q

What causes “Chipmunk face” in beta Thalassemia?

A

Fewer RBC means extramedullary haematopoiesis is needed (RBC are produced at forehead and cheekbones instead so these sites are enlarged)

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62
Q

When do beta Thalassemia symptoms present? Why?

A

In the 1st year of life. Symptoms aren’t present at birth because foetal Hb (HbF) is made up of 2 alpha and 2 gamma chains (no beta), so HbF is unaffected

HbF production stops and HbA production begins over the first 2 years of life.

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63
Q

What will a FBC and blood film show in Thalassemia?

A

FBC: Hypochromic RBCs

Blood film: Microcytic anaemia, target cells, increased reticulocytes

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64
Q

What investigation is diagnostic for Thalassemia? Which other condition is this investigation diagnostic for?

A

Hb electrophoresis (shows the proportion of different types of haemoglobin)

Also diagnostic for sickle cell anaemia

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65
Q

Why are reticulocytes raised in Thalassemia?

A

Thalassemia is essentially a haemolytic anaemia (despite not being normocytic)

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66
Q

What are reticulocytes?

A

Immature red blood cells

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67
Q

What X ray finding might be seen in Thalassemia?

A

Hair on end sign due to increased bone marrow activity

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68
Q

What are the 4 phenotypes of alpha Thalassemia?

A

1 deletion: Normal phenotype (carrier)
2 deletions: Alpha Thalassemia trait
3 deletions: Alpha Thalassemia minor (marked anaemia)
4 deletions: Alpha Thalassemia major (HYDROPS FETALIS aka heart failure in utero)

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69
Q

What is the 1st line treatment for Thalassemia? What needs to be given alongside and why?

A

Regular blood transfusion

Iron chelation (desferoxamine) needs to be given alongside prevent iron overload

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70
Q

Name the drug used in iron chelation therapy

A

Desferoxamine

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71
Q

List the side effects of iron chelation therapy

A

Deafness and cataracts

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72
Q

What is the definitive treatment for thalassemia? Why is it not always used?

A

Bone marrow stem cell transplant
It’s risky

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73
Q

List all the treatment options for thalassemia

A

1st line - Blood transfusion with iron chelation therapy (desferoxamine)
Splenectomy
Folate supplements (for haemolytic anaemia)
Ascorbic acid (vitamin C)
Bone marrow stem cell transplant

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74
Q

When should you wait until to perform a splenectomy? Why?

A

After 6 years old
The spleen has a role in defending against encapsulated bacteria

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75
Q

What is desferoxamine? When is it used?

A

Iron chelation therapy
Used to prevent Fe overload from blood transfusion (to treat thalassemia) or haemochromatosis

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76
Q

Define sideroblastic anaemia

A

Defective Hb synthesis within mitochondria due to ALA synthase deficiency

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77
Q

How is sideroblastic anaemia often inherited?

A

X-linked inheritance

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78
Q

Sideroblastic anaemia is caused by the deficiency of which enzyme?

A

ALA synthase

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79
Q

What investigations are done for sideroblastic anaemia? What would they show?

A

FBC + blood film -Microcytic anaemia with ringed sideroblasts and basophilic stippling
Iron studies - Increased serum Fe, transferrin and ferritin. Decreased TIBC

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80
Q

Define sickle cell anaemia

A

Haemoglobinopathy affecting beta globin chains

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81
Q

How is sickle cell anaemia inherited?

A

Autosomal recessive

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82
Q

Where is sickle cell anaemia most common? Why?

A

Africa. Being a carrier is portective against Plasmodium falciparum malaria

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83
Q

Describe the pathology of sickle cell anaemia

A

GAG mutates to GTG on the 6th codon of beta globin causing glutamic acid to be replaced by valine. This causes irreversible RBC sickling. The RBCs are more fragile and have lower surface area so are less efficient, at risk of sequestration and die quicker.

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84
Q

Which types of haemolysis are seen in sickle cell?

A

Intravascular and extravascular

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85
Q

What is the difference between intravascular and extravascular haemolysis?

A

Intravascular - in blood vessels, marked by increased haptoglobin
Extravascular - Outside vessels at the spleen

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86
Q

What are the symptoms of sickle cell?

A

General anaemia symptoms and jaundice

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87
Q

Name 3 complications of sickle cell anaemia

A

Splenic sequestration - Can cause autosplenectomy
Vaso-occlusive crises - Sickle cells polymerise and get trapped in long bone bone vessels
Acute chest crises - Pulmonary vessel occlusion causes respiratory distress (medical emergency!!)

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88
Q

Which infection is common in sickle cell? Why is it idfferent in sickle cell?

A

Oestomyelitis
Normally caused by Staph aureus, but in sickle cell patients is due to Salmonella

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89
Q

How does Salmonella appear on XLD agar?

A

Red colonies with black centres

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90
Q

Which investigation is diagnostic for sickle cell anaemia? What other condition is this also diagnostic for?

A

Hb electrophoresis showing 90% HbS
Thalassemia

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91
Q

How are newborns screened for sickle cell?

A

Guthrie heel prick test

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92
Q

What does an FBC and blood film show in sickle cell anaemia?

A

Normocytic normochromic anaemia with increased reticulocytes
Sickle red blood cells
Howell Jolly bodies

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93
Q

What emponymous finding is seen in sickle cell anaemia? Which other condition is it seen it?

A

Howell Jolly bodies
Iron deficiency anaemia

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94
Q

What is the treatment for acute complicated sickle cell atacks?

A

IV fluid
Analgesia (NSAIDs)
Oxygen
Antibiotics if infection

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95
Q

What is the long term treatment for sickle cell anaemia?

A

Avoid precipitants
Drugs (hydroxycarbaminde and folic acid supplements)
Transfusion and iron chelation
Last resort: BM stem cell transplant

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96
Q

What is the last resort treatment for sickle cell anaemia?

A

BM stem cell transplant

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97
Q

List some precipitants of sickle cell attacks

A

Cold, infection, dehydration, hypoxia (acidosis)

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98
Q

What is hydroxycarbamide used for?

A

To prevent sickling of red blood cells in sickle cell anaemia

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99
Q

How is G6PDH deficiency inherited?

A

X-linked recessive inheritance

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100
Q

Define G6PDH deficiency

A

X-linked excessive enzymopathy causing halved lifespan and RBC degradation (aka haemolysis)

101
Q

What does G6PDH do?

A

Protects against oxidative damage to red blood cells

102
Q

Describe the pathology of G6PDH deficiency

A

G6PDH is involved in slutathione synthesis
Glutathione protects against reactive oxidation species such as H2O2

103
Q

What are the symptoms of G6PDH deficiency?

A

Mostly asymptomatic unless haemolysis is precipitated

104
Q

What can precipitate acute haemolysis in G6PDH deficiency?

A

Major cause: Napthelene (pesticide) in moth balls
Nitrofurantoin
Aspirin
Fava beans

105
Q

What are the symptoms of acute haemolysis in G6PDH deficiency?

A

Rapid anaemia and jaundice (intravascular haemolysis)

106
Q

What are the investigations for suspected G6PDH deficiency?

A

FBC + blood film
Serum G6PDH levels

107
Q

What does a FBC + blood film show in G6PDH deficiency?

A

Between attacks: Normal
During attacks: Normocytic normochromic anaemia with raised reticulocytes, Heinz bodies and bite cells

108
Q

What is the treatment for G6PDH deficiency?

A

Avoid precipitants
Blood transfusions when attacks get worse

109
Q

What protein is deficient in hereditary spherocytosis?

A

Spectrin

110
Q

How is hereditary spherocytosis inherited?

A

Autosomal dominant

111
Q

Where is hereditary spherocytosis more common?

A

Northern Europe and America

112
Q

How are RBCs different in hereditary spherocytosis?

A

Spherical and rigid

113
Q

Which kind of haemolysis is seen in hereditary spherocytosis

A

Extravascular

114
Q

Define hereditary spherocytosis

A

Autosomal dominant deficiency in spectrin causing increased splenic recycling (extravascular haemolysis)

115
Q

What are the symptoms of hereditary spherocytosis

A

Anaemia
Neonatal jaundice
Splenomegaly
Gall stones (in 50%)

116
Q

What would a FBC and blood film show in hereditary spherocytosis?

A

Normocytic normochromic anaemia with increased reticulocytes and spherocytes

117
Q

Which condition also shows spherocytes on a blood film? How can you differentiate these two conditions?

A

Autoimmune haemolytic anaemia

Direct Coombs test (looks for Abs attached to RBCs). AHA is positive, HS is negative

118
Q

What is the treatment for hereditary spherocytosis? When should you wait until and why?

A

Splenectomy (plus folate supplements and transfusions)
After 6 years old. Spleen plays a role in defending against encapsulated bacteria

119
Q

What is the treatment for neonatal jaundice? Why is it important to treat?

A

Phototherapy
KERNICTERUS if untreated (bilirubin accumulates in basal ganglia causing CNS dysfunction and death)

120
Q

Define kernicterus and name 2 conditions it is a complication of

A

CNS damage caused by the accumulation of unconjugated bilirubin in the brain and nerve tissues
Crigler Najjar syndrome and hereditary spherocytosis

121
Q

What are the 2 subtypes of autoimmune haemolytic anaemia? Which is ore common?

A

Warm and cold. Warm is more common

122
Q

Describe the pathology of autoimmune haemolytic anaemia

A

Anti-RBC autoantibodies bind to RBCs and cause increased intravascular and extravascular haemolysis

123
Q

What is the diagnostic investigation for autoimmune haemolytic anaemia?

A

Postiive Direct Coombs test

124
Q

What precipitates haemolysis in autoimmune haemolytic anaemia?

A

Temperature change

125
Q

Define aplastic anaemia

A

Pancytopenia caused by failing bone marrow (no longer produces haematopoietic stem cells)

126
Q

List 3 causes of aplstic anaemia

A

Mostly idiopathic acquired
Could be infection (EBV, parovirus B19)
Congenital

127
Q

What would a FBC show in aplastic anaemia?

A

Normocytic anaemia with decreased reticulocytes

128
Q

What would a bone marrow biopsy show in aplastic anaemia?

A

Hypocellularity

129
Q

What is a complication of aplastic anaemia? What is the prophylactic treatment?

A

Increased infection risk due to neutropenia

Broad spectrum ABx and bone marrow transplant

130
Q

What causes anaemia of chornic disease?

A

Reduced erythropoietin causes reduced bone marrow stimulation for erythropoiesis

131
Q

What would a FBC show in anaemia of chronic disease?

A

Normocytic normochromic anaemia with reduced reticulocytes

132
Q

Define myelopathic process and state what kind of anaemia it causes

A

Bone marrow is replaced with something else eg. malignancy
Normocytic non-haemolytic anaemia

133
Q

Pernicious anaemia is the deficiency of what vitamin?

A

B12

134
Q

What are some risk factors for pernicious anaemia?

A

Older patient
Vegan diet

135
Q

List some causes of B12 deficiency

A

Pernicious anaemia
Malnutrition
Pregnancy
Crohn’s (affects iluem)
Gastrectomy

136
Q

Define pernicious anaemia

A

B12 deficiency caused by autoantibody mediated immune destruction of parietal cells in the fundus and body of the stomach

137
Q

Describe the pathophysiology of pernicious anaemia

A

Anti parietal antibodies destroy parietal cells in the stomach. Less intrinsic factor is produced so fewer B12-IF complexes are formed and less B12 is absorbed.

138
Q

What are symptoms of pernicious anaemia?

A

General anaemia
Lemon yellow skin
Angular stomatitis
Glossitis
Neurological symptoms (eg. symmetrical parasthesia, muscle weakness, altered mental status)

139
Q

Why does low B12 cause neurological symptoms?

A

Low B12 causes demyelination

140
Q

What is a haematinimic and give examples? What are the symptoms of haematinimic deficiency?

A

Nutrients needed for haematopoiesis

B12, folate, iron

Angular stomatitis and glossitis

141
Q

What would a FBC and blood film show in pernicious anaemia?

A

Macrocytic anaemia
Megaloblasts (hypersegmented nucleated neutrophils with 6+ lobes)

142
Q

What is a megaloblast? Which conditions are they seen in?

A

Hypersegmented nucleated neutrophils with 6+ lobes. Less mature DNA has more lobes, since DNA becomes more tightly wound into histones as it matures. (Bigger therefore MEGA)

B12 deficiency and folate deficiency

143
Q

What is the treatment for Thalassemia?

A

1st line - Regular blood transfusions + Iron chelation (to prevent iron overload from the transfusions) in the form of desferoxamine

Splenectomy (after 6 years old)

Folate supplements

Ascorbic acid (vitamin C)

Definitive treatment is bone marrow stem cell transplant

144
Q

List the investigations for B12 deficiency and what they show

A

FBC + Blood film: Macrocytic anaemia, megaloblasts
Low serum B12
Anti IF antibodies (very specific to pernicious anaemia so DIAGNOSTIC but not present in all patients)
Anti parietal antibodies

145
Q

What is the treatment for pernicious anaemia?

A

Dietary advice (salmon, eggs)
B12 supplements (oral hydroxycoalbumin)

146
Q

What is hydroxycoalbumin ?

A

Oral B12 supplement used to treat pernicious anaemia

147
Q

What are 2 complications of pernicious anaemia?

A

Neurological disorders
CVD

148
Q

List the causes of folate deficiency anaemia?

A

Trimethoprim (antibiotic) and methotrexate (DMARD)!!
Malnutrition
Malabsorption
Pregnancy
Alcohol

149
Q

Why do trimethoprim and methotrexate cause folate deficiency?

A

They are dihydrofolate reductase inhibitors

150
Q

Where is folate found in the diet?

A

Animal protein and leafy greens

151
Q

What are symptoms of folate deficiency?

A

General anaemia
Angular stomatitis
Glossitis

152
Q

What symptoms are found in B12 deficiency but NOT folate deficiency?

A

Lemon yellow skin
Neurological symptoms

153
Q

List the investigations for folate deficiency and what they show

A

FBC + blood film: Macrocytic anaemia and megaloblasts (DDx - B12 def)
Low serum folate
May have concomitant B12 deficiency

154
Q

What is the treatment for folate deficiency?

A

Dietary advice (leafy greens, brown rice)
Folate supplements (not before B12 supplements!!)

155
Q

If a patient has folate deficiency with concomitant B12 deficiency, which should be supplemented first? Why?

A

B12 should be replaced first
Giving folate first further depletes B12 by activating the complex DNA synthesis pathway (risk of neurological symptoms)

156
Q

Which patients should be given prophylactic folate supplements? How much?

A

Pregnant patients
400mg for first 12 weeks

157
Q

What is a complication of folate deficiency?

A

CVD

158
Q

What does dihydrofolate reductase do?

A

Catalyses the reduction of dihydrofolate to tetrahydrofolate. THF is needed for the action of folate-dependent enzymes

159
Q

List 3 causes of non Megaloblastic macrocytic anaemia

A

Alcohol - Toxic to RBCs and depletes folate (+ vit B1)

Hypothyroidism - Multifactorial, interferes with erythropoietin

Liver disease - Decompensated cirrhosis (hep B associated, +/- HCC) is strongly linked to macrocytic anaemia (short term predictor or mortality)

160
Q

What are the 4 type of leukaemia? Which cells does each type affect?

A

Acute myeloblastic leukaemia - Immature myeloblasts
Chronic myeloblastic leukaemia - Mature myelocytes (eg. Neutrophil, eosinophil, basophil)
Acute lymphocytic leukaemia - Immature lymphocytes
Chronic lymphocytic leukaemia - Mature lymphocytes (eg. T cells, B cells)

161
Q

List some differences between chronic and acute leukaemias

A

Acute tends to affect younger patients, chronic tends to affect older patients

Acute is generally symptomatic, chronic is generally asymptomatic

Cytopenias are more likely in acute than chronic (bone marrow has less time to adapt)
Cytopenias can still appear in chronic leukaemia when significantly progressed

162
Q

Define leukaemia

A

Neoplastic production of the WBC line (myeloblasts and lymphoblasts) in bone marrow, causing reduced production of other haematopoietic cells leading to pancytopenia

163
Q

Which kind of cancer causes pancytopenia? What are the complications?

A

Leukaemia

Neutropenia - Increased infection risk
Anaemia - Decreased Hb
Thrombocytopenia - Increased bleeding risk

164
Q

What are the general symptoms of leukaemia?

A

Bone pain
Bleeding (thrombocytopenia)
Infection (neutropenia)
Anaemia symptoms
TATT

165
Q

Which cells proliferate in acute myeloblastic leukaemia?

A

Immature myeloblasts

166
Q

Which mutation causes AML?

A

t(15:17)

167
Q

Which types of leukaemia are associated with Down’s and radiation?

A

AML and ALL

ALL is 30x higher with Down’s syndrome

168
Q

What is the prognosis for AML?

A

Rapidly progresses if not treated ASAP
3 year survival is only 20%

169
Q

What are the symptoms of AML?

A

General leukaemia symptoms
Gum infiltration
Hepatosplenomegaly

170
Q

List the investigations for AML and what they show

A

FBC + blood film: Pancytopenia. Myeloperoxidase +ve and Auer roads
BM biopsy: >=20% myeloid blasts

171
Q

What are auer rods?

A

Myeloperoxidase cytoplasmic aggregates in neutrophils

172
Q

What is the 1st line treatment for AML?

A

Chemo and ATRA (all trans-retinoid acid)

ATRA is used in subtype of AML called acute promyelocytic leukaemia

173
Q

What is the last resort treatment for AML?

A

Bone marrow transplant

174
Q

List the treatments for AML

A

1st line - Chemo and ATRA
Prophylactic antibiotics (neutropenia)
Transfusion for anaemia
Allopurinol if doing chemo
Last resort - BM transplant

175
Q

Why should allopurinol be given when doing chemo for AML?

A

To prevent tumour lysis syndrome

Chemo releases uric acid from cells, which can accumulate in kidneys

176
Q

What is a common complication of acute myeloblastic leukaemia? Which subtype of AML is known to cause this?

A

Disseminated intravascular coagulation

Acute promyelocytic leukemia

177
Q

What cell proliferates in chronic myeloid leukaemia?

A

Mature myelocytes (neutrophils, eosinophils, basophils)

178
Q

What mutation causes CML?

A

t(9:22) on the Philadelphia chromosome
BCR-ABL gene fusion causes tyrosine kinase to be irreversibly switched on, causing increased cell proliferation

179
Q

What are the symptoms of CML?

A

General leukaemia
Massive hepatosplenomegaly

180
Q

List 2 conditions that can cause massive hepatosplenomegaly

A

Chronic myeloid leukaemia
Malaria

181
Q

List the investigations for CML and what they show

A

FBC + blood count: Pancytopenia BUT granulocytosis (too many neutrophils, basophils and eosinophils). Blood blast cell % (shows severity)

BM biopsy: Raised granulocytes

Gold standard: Philadelphia chromosome genetic test

182
Q

How is CML severity determined?

A

Blood blast cell % on FBC

< 10: Chronic (best)
10 - 19: Accelerated
>20: Blast crisis (worst)

183
Q

What is the gold standard investigation for CML?

A

Philadelphia chromosome genetic test

184
Q

What is the treatment for CML?

A

Chemo and Imantinib
Consider allopurinol (to prevent tumour lysis syndrome)

185
Q

What is imantinib?

A

Tyrosine kinase inhibitor used to treat CML

186
Q

What can CML progress to if untreated or diagnosed late? Why is this bad?

A

Acute myeloblastic leukaemia
Poorer prognosis
Indicated by >20% blast cells on BM biopsy

187
Q

What is the prognosis for CML?

A

Decent (if no progression)

188
Q

What cell proliferates in acute lymphoblastic leukaemia?

A

Immature lymphoblasts - mostly B cell lineage

189
Q

What mutation causes ALL?

A

t(12:21)

190
Q

What is the prognosis for ALL?

A

Good

191
Q

What is the most common childhood malignancy?

A

ALL
75% cases over 6 yrs old

192
Q

What are the symptoms of ALL?

A

General leukaemia (except 6yr old with Down’s syndrome)
Hepatosplenomegaly

193
Q

List the investigations for ALL and what they show

A

FBC: Pancytopenia
Blood film: Increased lymphoblasts
Bone marrow biopsy: Over 20% lymphoblasts (DIAGNOSTIC)
Immunefluorescence: Terminal deoxynucleotidyl transferase positive lymphoblasts (GOLD STANDARD)

194
Q

What is the treatment for ALL?

A

Chemo
Consider allopurinol

195
Q

What cell proliferates in chronic lymphocytic leukemia?

A

Lymphocytes (mostly B cells)

196
Q

Which leukaemia is the most common overall? Which is the most common in childhood?

A

CLL is most common overall
ALL is most common in children

197
Q

Which age group is CLL most common in?

A

Later life (70+)

198
Q

What is the prognosis for CLL?

A

Ok - 75% 5 year survival

199
Q

What are the symptoms for CLL?

A

General anaemia symptoms
Lymphadenopathy (non-tender)
Hepatosplenomegaly

200
Q

List the investigations for CLL and what they show

A

FBC: Pancytopenia BUT lymphocytosis
Blood film: SMUDGE CELLS
Immunoglobulins: Hypogammaglobulinemia

201
Q

Why does CLL cause hypogammaglobulinemia?

A

B cells proliferate BUT they don’t differentiate into plasma cells (which produce IgG)

202
Q

Smudge cells on a blood film are indicative of which condition?

A

Chronic lymphocytic leukaemia

203
Q

What is the treatment for CLL?

A

Chemo
Palliative care (if old)
Consider allopurinol
IV IgG for hypogammaglobulinemia

204
Q

What is a complication of CLL?

A

RICHTER TRANSFORMATION
B cells massively accumulate in lymph nodes causing massive lymphadenopathy and transformation from CLL to aggressive lymphoma

205
Q

Richter transformation is a complication of which condition?

A

Chronic lymphocytic leukaemia

206
Q

What are the 2 types of lymphoma? How can they be told apart on a blood film?

A

Hodgkin’s and non-Hodgkin’s

Hodgkin’s has REED STERNBERG cells (owl-eye nuclei)

207
Q

What are the general symptoms of lymphoma?

A

B SYMPTOMS

Fever
Night sweats
Unintentional weight loss
(Lymphadenopathy)

208
Q

What is the distribution of age of Hodgkin’s lymphoma patients?

A

Bimodal: Teens and elderly

209
Q

What virus is Hodgkin’s lymphoma associated with?

A

Epstein Barre Virus (aka “glandular fever”)

210
Q

What are the symptoms of Hodgkin’s lymphoma?

A

B symptoms + painless rubbery lymphadenopathy (especially after drinking alcohol)

211
Q

List the investigations for Hodgkin’s lymphoma and what they show

A

Lymph node biopsy: Reed Sternberg cells (DIAGNOSTIC)
Raised lactate dehydrogenase
Low Hb
Raised ESR
CT/MRI chest/abdo/pelvis for staging (Ann Arbour staging)

212
Q

How is lymphoma staged?

A

Ann Arbour staging

1) Single lymph node
2) >= 2 lymph nodes on same diaphragm side
3) Lymph nodes on both diaphragm sides
4) Extranodal organ spread

A = No “B” symptoms
B = B symptoms present

213
Q

What is the treatment for Hodgkin’s lymphoma?

A

ABVD chemotherapy - Adriamycin (doxorubicin), Bleomycin, Vinblastine, Dacarbazine

214
Q

What are the side effects of ABVD chemo?

A

Alopecia, N+V, Myelosuppression and bone marrow failure, infection

215
Q

What are patients who have had recent / high dose chemo (or on carbimazide) at risk of?

A

Febrile neutropenia

216
Q

What are the symptoms of febrile neutropenia?

A

Fever
Tachycardia
Sweats
Rigors
Tachypnoea

217
Q

What is the treatment for febrile neutropenia?

A

Immediate broad spectrum antibiotics (eg. Amoxicillin, fluoroquinolone)

218
Q

What subtype of Hodgkin’s lymphoma does popcorn cells on lymph node biopsy indicate?

A

Nodular lymphocyte predominant Hodgkin’s

219
Q

What are the subtypes of non-Hodgkin’s lymphoma? Which is most common?

A

Follicular - low grade
Diffuse large B cell - high grade (MOST COMMON)
Burkitt’s - Very high grade

220
Q

Which subtype of non-Hodgkin’s lymphoma is linked to EBV?

A

Burkitt’s lymphoma. Often causes massive jaw lymphadenopathy in children

221
Q

What are the symptoms of non Hodgkin’s lymphoma?

A

Varied since many subtypes

B symptoms
Painless rubbery lymphadenopathy NOT affected by alcohol

222
Q

List the investigations for Non-Hodgkin’s lymphoma and what they show

A

Lymph node biopsy: No RS/popcorn cells (DIAGNOSTIC). Confirms NHL subtype (eg. Burkitt’s = “starry sky” biopsy)
CT/MRI chest/abdo/pelvis for staging

223
Q

What is the treatment for Non-Hodgkin’s lymphoma?

A

R-CHOP chemotherapy - Rituximab,cyclophosphamide, Hydroxydoxorubicin, Vincristine (Oncovin is brand name), Prednisolone

224
Q

How does Rituximab work?

A

Monoclonal antibody which targets CD20 on Bcell

225
Q

List some differences between Hodgkin’s and Non-Hodgkin’s lymphoma

A

NHL has subtypes (follicular, B cell, Burkitt’s), HL does not
HL presents with painless lymphadenopathy which is painful with alcohol, NHL is unaffected
HL is treated with ABVD chemo, NHL is treated with R-CHOP chemo

226
Q

Define multiple myeloma

A

Neoplastic monoclonal proliferation of a plasma cell

227
Q

Describe the pathophysiology of multiple myeloma

A

Typically excess production of one immunoglobulin (“monoclonal paraprotein”)

55% IgG, 20% IgA

228
Q

What might a typical MM patient look like?

A

70 years old, Afro-Caribbean

229
Q

What are the symptoms of MM? (There’s an acronym)

A

Old CRAB

Old - 70+
HyperCalcaemia
Renal failure
Anaemia
Bone lesion

230
Q

What causes hypercalcaemia in MM?

A

Increased osteoclast bone resorption

231
Q

What are the symptoms of Hypercalcaemia (theres a phrase)

A

Bones (weak bones)
Stones (kidney stones)
Abdo moans
Psychedelic groans

232
Q

What causes renal failure in MM?

A

Hypercalcaemia causes calcium oxalate renal stones
Immunoglobulin light chain kappa deposition is nephrotoxic - Bence Jones protein in urine!

233
Q

What causes anaemia in MM?

A

Bone marrow failure

234
Q

What causes bone lesions in MM?

A

Bone marrow failure (which is painful - new onset back pain in elderly)

235
Q

List the investigations for MM and what they show

A

FBC + blood film = Normocytic normochromic anaemia and raised ESR. ROULEAUX FORMATION (abnormal aggregation of RBCs together) - pathognomic!!

Urine dipstick = Bence jones proteins

U+E = Renal failure. Consider X-RAY KUB for kidney stones

Bone profile: Hypocalcaemia and raised ALP

Serum electrolytes = Ig paraprotein “M spike”, hypergammaglobulinemia for that specific IgG
X-RAY = Pepper pot skull (Osteolytic lesions = punch out holes)

DIAGNOSTIC: BM biopsy —> More than 10% plasma cells

236
Q

What investigation is diagnostic for Multiple myeloma?

A

BM biopsy: More than 10% plasma cells

237
Q

Name the precursor to multiple myeloma. How does it differ from MM?

A

Mammyloid gammopathy of undetermined significance

BM biopsy: Under 10% plasma cells
No/little paraprotein spike
ASYMPTOMATIC

238
Q

Why do NICE recommended a full body MRI when investigating MM?

A

To identify bone marrow infiltration

239
Q

What is the treatment for MM?

A

Chemotherapy
Bisphosphonates (eg. Alendronate) to protect bones
Dialysis (for renal failure)
Consider BM stem cell transplant

240
Q

Define polycythaemia

A

Erythrocytosis of any cause

241
Q

What is a primary cause of polycythaemia?

A

Polycythaemia Vera
JAK2V617 mutation

242
Q

What are secondary causes of polycythaemia?

A

Hypoxia
More erythropoietin (eg. Renal cell carcinoma)
Dehydration (eg. Alcohol)

243
Q

What causes polycythaemia Vera?

A

JAK2V617 mutation

244
Q

List the symptoms of polycythaemia vera?

A

ITCHING AFTER A BATH
BURNING IN FINGERS AND TOES (Erythromelalgia)
Reddish plethoric complexion (facial swelling and puffiness, purplish face like in Cushings)
Blurred vision / headache
HEPATOSPLENOMEGALY

Nonspecific features of HYPERVISCOSITY

245
Q

List the investigations for polycythaemia vera and what they show

A

FBC = High WCC, platelets and high RBCs
Increased Hb
Genetic test for JAK2V617 mutation

246
Q

What is the aim to polycythaemia vera treatment?

A

Maintain normal blood count (not curative)

247
Q

What is the 1st line treatment for polycythaemia vera?

A

Regular venesection + aspirin (anti platelet)

248
Q

What treatment for polycythaemia could be considered after the 1st line?

A

Chemotherapy - Hydroxycarbamide aka hydroxyurea (only in high risk patients)

Phase 2A HT Notes (1 decks)

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