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Phase 2A revision > HAEMATOLOGY > Flashcards

HAEMATOLOGY Flashcards

1
Q

what are the investigations for CLL?

A

● Normal or low Hb
● Raised WCC with very high lymphocytes
● Blood film – smudge cells may be seen in vitro

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2
Q

What is the treatment for CLL?

A
  • Watch and wait
  • Chemotherapy
  • Monoclonal antibodies, e.g. rituximab
  • Targeted therapy, e.g. bruton kinase inhibitors (ibrutinib)
  • human IV immunoglobulins
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3
Q

what is the epidemiology of ALL?

A

Mainly a childhood disease
highest prevalence between 2-4yrs
Associated with Down’s syndrome
Associated with ionising radiation

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4
Q

what are the investigations for ALL?

A

● FBC and blood film
o WCC = high
o Blast cells on film and in bone marrow

● CXR and CT scan looking for mediastinal and abdominal lymphadenopathy

● Lumbar puncture to look for CNS involvement

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5
Q

Give 3 environmental causes of leukaemia

A
  1. Radiation exposure
  2. Chemicals (benzene compounds)
  3. Drugs
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6
Q

What is lymphoma?

A

Malignant proliferation of B and T lymphocytes in the lymph nodes

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7
Q

Although predominantly in the lymph nodes, what other organs can lymphoma effect?

A
  1. Blood
  2. Bone marrow
  3. Liver
  4. Spleen
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8
Q

Give 4 risk factors for lymphoma

A
  1. Primary immunodeficiency
  2. Secondary immunodeficiency - HIV, transplant recipients
  3. Infections - EBV, H. pylori, HTLV-1
  4. Autoimmune disorders - SLE, RA
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9
Q

Describe the pathophysiology of lymphoma

A

Impaired immunosurveillance and infected B cells escape regulation and proliferate

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10
Q

What are the 2 sub-types of lymphoma

A
  1. Hodgkins lymphoma

2. Non-hodgkins lymphoma

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11
Q

What are signs and symptoms of Hodgkins lymphoma?

A
  • Fever and sweating
  • Enlarged rubbery non-tender nodes
  • Systemic ‘B’ symptoms, e.g. fever
  • Painful nodes on drinking alcohol
  • some patients (commonly young women) have disease localised to the mediastinum
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12
Q

What is needed for the diagnosis of Hodgkins lymphoma?

A

Presence of Reed-Sternberg cells (in lymph node biopsy)

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13
Q

What blood results may you seen in someone with Hodgkins lymphoma?

A 
High ESR 
FBC - anaemia (normochromic normocytic)
Reed Sternberg cells
Low Hb 
High serum lactase dehydrogenase
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14
Q

Describe the staging go Hodgkins lymphoma

A

Stage 1 = confined to a single lymph node region

Stage 2 = Involvement of two or more nodal areas on the same side of the diaphragm

Stage 3 = Involvement of nodes on both sides of the diaphragm.

Stage 4 = Spread beyond the lymph nodes e.g. liver.

Each stage is either ‘A’ - absence of ‘B’ symptoms or ‘B’ - presence of ‘B’ symptoms

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15
Q

What the treatment for Hodgkins lymphoma?

A

ABVD chemotherapy regime

marrow transplant

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16
Q

What are the possible complications of treatment for Hodgkins lymphoma?

A
  1. Secondary malignancies
  2. IHD
  3. Infertility
  4. Nausea
  5. Alopecia
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17
Q

What is non-hodgkins lymphoma?

A

Any lymphoma not involving Reed-Sternberg cells

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18
Q

what are the signs and symptoms of non-hodgkins lymphoma?

A

Fever and sweating
Enlarged rubbery non-tender nodes
Systemic ‘B’ symptoms, e.g. fever
GI and skin involvement

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19
Q

What is the treatment for non-hodgkins lymphoma?

A

Steroids
Monoclonal antibodies to CD20 -> Rituximab
CHOP regimen

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20
Q

What is anaemia?

A

A reduced RBC mass +/- reduced Hb concentration

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21
Q

What is the functions of Hb?

A

It carries and delivers oxygen to tissues

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22
Q

What organs are responsible for the removal of RBCs?

A
  1. Spleen
  2. Liver
  3. Bone marrow
  4. Blood loss
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23
Q

what are the causes of microcytic anaemia?

A
  1. Iron deficiency
  2. Chronic disease - cancer, HF, CKD
  3. Thalassaemia
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24
Q

what are the causes of normocytic anaemia?

A
  1. Acute blood loss
  2. Chronic disease
  3. Combined haematinic deficiency (iron and B12 deficiency)
  4. renal failure
  5. pregnancy
  6. hypopituitarism, hypothyroidism, hypoadrenalism
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25
Q

Give 3 causes of macrocytic anaemia

A
  1. B12/folate deficiency
  2. Alcohol excess –> liver disease
  3. Haemolytic anaemia
  4. Hypothyroidism
  5. bone marrow infiltration/failure
  6. myeloma
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26
Q

Briefly describe the pathophysiology of anaemia

A

Reduced O2 transport –> tissue hypoxia –> compensatory mechanism = increased tissue perfusion, increased O2 transfer to tissue, increase RBC production

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27
Q

What pathological changes occur due to anaemia?

A
  1. Heart/liver fat change
  2. Ischaemia
  3. Skin/nail atrophy
  4. CNS death
  5. Aggravate angina and claudication
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28
Q

What do reticulocytes show you?

A

Marker of balance between formation and removal of RBCs

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29
Q

Give 4 symptoms of anaemia

A

Non-specific

  1. Fatigue
  2. Dyspnoea
  3. Headache
  4. Faintness
  5. Palpitations
  6. Anorexia
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30
Q

Give 3 signs of anaemia

A
  1. Conjunctival pallor
  2. Hyper-dynamic circulation
  3. Tachycardia
  4. Systolic flow murmur
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31
Q

What investigations might you do in someone with anaemia?

A
  1. FBC and Blood film
  2. Reticulocyte count
  3. B12, folate and ferritin levels
  4. U+E’s, LFT’s, TSH levels
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32
Q

What is the treatment for anaemia?

A

Treat the underlying cause

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33
Q

Give 3 causes of iron deficiency anaemia

A
  1. Blood loss
  2. Poor absorption
  3. Decreased intake in diet
  4. Increased demand - growth/pregnancy
  5. Hookworm
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34
Q

Describe the pathophysiology of iron deficiency anaemia

A

Lack of iron –> no haem production –> lack of effective RBCs

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35
Q

What would the results of investigations be for someone with iron deficiency anaemia?

A

MICROCYTIC HYPOCHROMIC ANAEMIA

Low ferritin, low reticulocyte

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36
Q

How do you treat iron deficiency anaemia?

A 
Improve diet and treat cause 
Ferrous sulphate (iron supplement)IV iron if severe
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37
Q

Describe the pathophysiology of B12 deficiency/pernicious anaemia

A

a lack of B12/intrinsic factor –> B12 not absorbed in terminal ileum –> big facile RBCs

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38
Q

what are the causes of B12 deficiency/pernicious anaemia?

A
  1. Autoimmune parietal cell destruction (pernicious)
  2. Poor diet
  3. Ileectomy
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39
Q

What are the specific signs of B12 deficiency/pernicious anaemia?

A

Inflamed tongue (glossitis), angular stomatitis, mild jaundice
Neurological - paraesthesia, psychological problems, ataxia
- dementia and visual disturbances

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40
Q

What would the results of the investigations be for someone with B12 deficiency/pernicious anaemia?

A

MACROCYTIC NORMOCHROMIC ANAEMIA with polysegmented neutrophils
Autoantibodies = intrinsic factor and coeliac antibodies(intrinsic factor antibodies are diagnostic)

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41
Q

How would you treat B12 deficiency/pernicious anaemia ?

A

Treat cause
B12 replacement - malabsorption = hydroxocobalamin IM
dietary = oral B12

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42
Q

Describe the pathophysiology of folate deficiency

A

In folate deficiency there is an impairment of DNA synthesis, resulting in delayed nuclear maturation resulting in large RBCs as well as decreased RBC production in the bone marrow

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43
Q

How do you treat folate deficiency?

A

Folic acid supplement - with B12

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44
Q

Describe the pathophysiology of haemolytic anaemia

A

RBC destroyed before 120 day lifespan –> compensatory reticulocytes from BM –> RBC destruction

  • Extravascular
  • Intravascular
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45
Q

Give 3 inherited causes of haemolytic anaemia

A
  1. RBC membrane defect
  2. Abnormal Hb - thalassaemia’s, sickle cell
  3. Enzyme defects - Glucose-6-phosphate dehydrogenase deficiency
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46
Q

Give 3 acquired causes of haemolytic anaemia

A
  1. Autoimmune
  2. Drug induced
  3. Secondary to systemic disease
  4. Malaria
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47
Q

What is the management for haemolytic anaemia

A

Dietary = folate and iron supplementation
AI cause = immunosuppression
Surgical = splenectomy

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48
Q

Name the 3 broad categories of red cell disorders

A
  1. Haemoglobinopathies
  2. Membranopathies
  3. Enzymopathies
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49
Q

What is normal adult haemoglobin made of?

A

2 alpha and 2 beta chains

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50
Q

What is foetal haemoglobin made of?

A

2 alpha and 2 gamma chains

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51
Q

What is haemoglobin S?

A

Variant haemoglobin arising because of a point mutation in the beta globin gene
Mutation leads to a single AA change, Glutamate –> Valine

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52
Q

What two categories can haemoglobinopathies be divided in to?

A
  1. Disorders of quality - abnormal molecule or variant haemoglobin (sickle cell)
  2. Disorders of quantity - reduced production (thalassaemia’s)
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53
Q

What is sickle cell disease?

A

A haemoglobin disorder of quality

HbS polymerises –> sickle shaped RBC

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54
Q

What is the advantage of being a carrier of sickle cell disease?

A

Offers protection against falciparum malaria

They are symptom free

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55
Q

Describe the inheritance pattern of sickle cell disease

A

sickle cell anaemia = Autosomal recessive Homozygous SS

sickle cell trait = heterozygous

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56
Q

If both parents are carries of the sickle trait, what is the chance of their first child having sickle cell disease?

A

25% chance of having sickle cell

50% chance of being a carrier

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57
Q

How long do sickle cells last for?

A

5-10 days

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58
Q

Give 3 acute complications of sickle cell disease

A
  1. Painful crisis
  2. Sickle cell syndrome - blockage of blood vessels within bone leading to pain
  3. Strokes in children
  4. Infections
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59
Q

Give 3 chronic complications of sickle cell disease

A
  1. Renal impairment
  2. Pulmonary hypertension
  3. Joint damage
  4. growth and development problems
  5. cardiomegaly, MI
  6. hepatomegaly
  7. retinopathy
  8. impaired placental blood flow
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60
Q

What investigations are done to confirm a diagnosis of sickle cell disease?

A
  • Blood film - Sickled erythrocytes seen
  • Blood count - Hb range of 60-80 g/L
  • Positive sickle solubility test
  • Haemoglobin electrophoresis - Confirms diagnosis
  • Aim for diagnosis at birth
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61
Q

Describe the treatment for sickle cell disease

A
  • Precipitating factors should be avoided - e.g. infection, dehydration, cold
  • Folic acid
  • Acute attacks:
    - IV fluids
    - Analgesia
    - Oxygen
  • Possible blood transfusions
  • Oral hydroxycarbamide to reduce frequency of crises
  • Stem cell transplant

long term management

  • hydroxyurea - increases HbF levels so is used as prophylaxis
  • pneumococcal vaccine
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62
Q

What is the significance of parvovirus in someone with sickle cell disease?

A

Parvovirus is common infection in children

Leads to decreased RBC production and an cause a dramatic drop in Hb in patients who already have a reduced RBC lifespan

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63
Q

Describe the inheritance pattern for membranoapthies

A

Autosomal dominant

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64
Q

Name 2 common membranopathies

A
  1. Spherocytosis

2. Elliptocytosis

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65
Q

Briefly describe the physiology of membranopathies

A

Deficiency in red cell membrane protein caused by genetic lesions

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66
Q

What are enzymopathies?

A

Enzyme deficiencies lead to shortened RBC lifespan

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67
Q

Name a common enzymopathy

A

G6PD deficiency

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68
Q

what are the clinical features of G6PD deficiency?

A 
Most are asymptomatic 
Pallor
Fatigue
Palpitations
Shortness of breath
Jaundice 
Exacerbated by ingesting fava beans
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69
Q

Name 3 things that can precipitate G6PD deficiency

A
  1. Broad beans / fava beans
  2. Infection
  3. Drugs - Primaquine, sulphonamide, quinolones
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70
Q

What is polycythaemia?

A
  • increased RBC count
  • increased haematocrit
  • Due to a JAK2 mutation
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71
Q

What hormone is responsible for regulating RBC production?

A

Erythropoietin (EPO)

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72
Q

What stimulates EPO?

A

Tissue hypoxia

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73
Q

what are the primary causes of absolute polycythaemia?

A

Polycythaemia vera - overactive bone marrow causing increase in RBC mass

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74
Q

what are the secondary causes of absolute polycythaemia?

A

hypoxia

 - Smoking
  - Lung disease
 - Cyanotic heart disease
  - High altitude 
  - obstructive sleep apnoea

Excess EPO - renal disease

alcohol

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75
Q

what are the clinical features of polycythaemia?

A 
Headaches and dizziness 
Itching
Fatigue
Tinnitus
Erythromelalgia – burning sensation in fingers and toes 
Hypertension 
Angina 
Hepatosplenomegaly (distinguishes 1o from 2o)
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76
Q

What is the treatment for polycythaemia?

A 
No cure
Treatment aim is to maintain normal blood count 
Venesection - relieves symptoms 
Low does aspirin 
Radioactive phosphorus in those over 70
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77
Q

Where are platelets produced?

A

In the bone marrow

They are fragments of megakaryocytes

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78
Q

What hormone regulated platelet production?

A

Thrombopoietin - produced mainly in the liver

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79
Q

What is the lifespan of a platelet?

A

7-10 days

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80
Q

What organ is responsible for platelet removal?

A

Spleen

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81
Q

What can cause platelet dysfunction?

A

Reduced platelet number (thrombocytopenia)Reduced platelet function

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82
Q

What can cause decreased platelet production?

A
  1. Congenital causes (e.g. malfunctioning megakaryocytes)
  2. Infiltration of bone marrow (e.g. leukaemia)
  3. Alcohol
  4. Infection (e.g. HIV/TB)
  5. Reduced TPO
  6. Aplastic anaemia
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83
Q

What can cause increased platelet destruction?

A
  1. Autoimmune (e.g. ITP)
  2. Hypersplenism
  3. Drug related (e.g. heparin induced)
  4. DIC and TTP –> increased consumption
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84
Q

What can cause reduced platelet function?

A
  1. Congenital abnormality
  2. Medications - aspirin
  3. VWF disease
  4. Uraemia
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85
Q

Give 3 symptoms of platelet dysfunction

A
  1. Mucosal bleeding - epistaxis, gum bleeding, menorrhagia
  2. Easy bruising
  3. Petechiae/purpura
  4. Traumatic haematomas
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86
Q

Give 4 causes of bleeding

A
  1. Trauma
  2. Platelet deficiency - thrombocytopenia
  3. Platelet dysfunction - aspirin induced
  4. Vascular disorders
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87
Q

Give 3 things that can cause coagulation disorders

A
  1. Vitamin K deficiency
  2. Liver disease
  3. Congenital - haemophilia
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88
Q

What is thrombotic thrombocytopenia (TTP)?

A
  • Widespread adhesion and aggregation of platelets leads to microvascular thrombosis and consumption of platelets
  • Occurs due to a reduction in ADAMTS-13 – a protease that is normally responsible for the degradation of vWF
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89
Q

what are the clinical features of thrombotic thrombocytopenia (TTP)

A
  • Flu-like symptoms
  • fever, fatigue, aches
  • Purpura
  • Epistaxis (nosebleeds)
  • Easy bruising
  • Menorrhagia (heavy periods)
  • Haemoptysis (coughing up blood)
  • Headache
  • Abdominal pain
  • GI bleeding
  • Chest pain (rare)
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90
Q

What is the treatment for thrombotic thrombocytopenia (TTP)?

A
  • Plasma exchange (removed ADAMTS13 autoantibodies)
  • Splenectomy
  • IV steroids
  • IV methylprednisolone
  • IV rituximab
  • folic acid
  • platelet transfusion = contraindicated
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91
Q

What is immune thrombocytopenia (ITP)?

A

Antibodies formed against platelet –> autoimmune platelet destruction via reticuloendothelial system

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92
Q

Give 3 causes of immune thrombocytopenia (ITP)

A 
Primary = viral infection (children)
Secondary (chronic, adults)  
  - Autoimmune conditions
  - Malignancies - CLL
  - Infections - HIV/Hep C
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93
Q

what is the clinical presentation of immune thrombocytopenia (ITP)?

A

Can by asymptomatic

  • Purpura
  • bruising and purple/red rash
  • Epistaxis (nosebleeds)
  • Menorrhagia (heavy periods)
  • Prolonged bleeding from the gums
  • Severe headache
  • Vomiting
  • Fatigue
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94
Q

What is the management for immune thrombocytopenia (ITP)?

A
  • first line = Corticosteroid (prednisolone) or IV immunoglobulin - IV IgG
  • second line = Splenectomy or immunosuppression (rituximab)
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95
Q

Give 4 causes of folate deficiency

A
  1. Dietary
  2. Malabsorption
  3. Increased requirement - pregnancy
  4. Folate antagonists - methotrexate
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96
Q

Give 3 signs of haemolytic anaemia

A
  1. Pallor
  2. Jaundice
  3. Splenomegaly
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97
Q

Give 2 specific symptoms of iron deficiency anaemia

A
  1. Koilonychia - spoon nails
  2. Brittle hair and nails
  3. Atrophic glossitis
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98
Q

What is neutrophilia?

A

Too many neutrophils

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99
Q

Give 3 causes of neutrophilia

A
  1. Infection
  2. Inflammation
  3. Cancer
  4. CML = primary cause
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100
Q

What is lymphocytosis?

A

Too many lymphocytes

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101
Q

Give 3 causes of lymphocytosis

A
  1. Viral infection
  2. Inflammation
  3. Cancer
  4. CLL = primary cause
102
Q

What is thrombocytopenia?

A

Not enough platelets

103
Q

What is thrombocytosis?

A

Too many platelets

104
Q

Give 3 causes of thrombocytosis

A
  1. Infection
  2. Inflammation
  3. Cancer
  4. Essential thrombocythaemia = primary cause
105
Q

What is neutropenia?

A

Not enough neutrophils

106
Q

What is the major risk associated with being neutropenic?

A

Susceptible to infection

107
Q

Give 3 causes of neutropenia

A
  1. Underproduction = marrow failure, marrow infiltration, marrow toxicity (e.g. drugs)
  2. Increased removal = autoimmune, Felty’s syndrome, cyclical
108
Q

Give 2 causes of thrombocytopenia

A
  1. Production failure - marrow suppression, marrow failure

2. Increased removal - immune response (ITP), consumption (DIC), splenomegaly

109
Q

What is the definition of febrile neutropenia?

A

Temperature >38 degrees in a patient with neutrophil count <1x10^9/Lit is an emergency

110
Q

Give 4 risk factors for febrile neutropenia

A
  1. If the patient had chemotherapy <6 weeks ago
  2. Any patient who has had a stem cell transplant <1 year ago
  3. Any haematological condition causing neutropenia
  4. Bone marrow infiltration
  5. those on methotrexate, carbimazole and clozapine
111
Q

Give a risk for spinal cord compression

A

Any malignancy that can cause compression - bone metastasis

commonly seen in myeloma and lymphoma

112
Q

What is hyper viscosity syndrome?

A

Increase in blood viscosity usually due to high levels of immunoglobulins

113
Q

What does rituximab target?

A

Targets CD20 on the surface of B cells

114
Q

What is the affect of sickle cell anaemia on reticulocyte count?

115
Q

What clotting factors depend on vitamin K?

A

2, 7, 9, 10

116
Q

Haemophilia A is due to deficiency of what clotting factor?

A

Factor 8 deficiency

117
Q

Haemophilia B is due to deficiency of what clotting factor?

A

Factor 9 deficiency

118
Q

How does warfarin work?

A

Antagonises vitamin K so reduction in clotting factors 2, 7, 9 and 10

119
Q

How does heparin work?

A

Indirect thrombin inhibitor

It binds to and activates antithrombin which then inhibits thrombin and factor Xa

120
Q

What 3 blood test values would be increased in someone with polycythaemia?

A
  1. Hb
  2. RCC
  3. PCV
121
Q

what are the clinical features of chronic lymphoblastic leukaemia (CLL)?

A
  • often no symptoms
  • may be anaemic
  • weight loss, sweats, anorexia
  • hepatosplenomegaly
  • enlarged, rubbery, non-tender nodes
122
Q

what is the progression of CLL like?

A
  • may transform into aggressive lymphoma
  • Richter’s syndrome
  • 1/3 never progress
  • 1/3 progress slowly
  • 1/3 progress actively
123
Q

what are the complications of CLL?

A
  • autoimmune haemolysis
  • increased infection risk
  • marrow failure
  • richter’s syndrome - transformation to aggressive lymphoma
124
Q

what is the epidemiology of hodgkins lymphoma?

A
  • male predominance

- majority of cases occur in teenagers and the elderly

125
Q

what are the 2 different groups of macrocytic anaemia?

A
  • megaloblastic = erythroblasts with delayed nuclear nmaturation
  • non-megaloblastic = normal erythroblasts
126
Q

how is vitamin B12 absorbed in the body?

A

binds to intrinsic factor (made by parietal cells) and absorbed in terminal ileum

127
Q

what is the role of vitamin B12?

A

essential for thymidine and DNA synthesis

128
Q

what is the epidemiology of pernicious anaemia and B12 deficiency?

A
  • common in elderly- women > men

- more common in people with fair hair and blue eyes

129
Q

what are the risk factors for pernicious anaemia?

A
  • female
  • elderly
  • fair hair and blue eyes
  • blood group A
  • thyroid and addison’s disease
130
Q

what are the risk factors for folate deficiency?

A
  • elderly
  • poverty
  • alcoholic
  • pregnant
  • crohn’s/coeliac
131
Q

what is the clinical presentation of folate deficiency?

A
  • may be asymptomatic
  • may present with anaemia symptoms
  • glossitis
  • no neuropathy
132
Q

what investigations should be undertaken in folate deficiency?

A
  • blood count and film = megaloblastic, RBCs macrocytic
  • low serum and red cell folate low
  • serum bilirubin may be raised
133
Q

why do sickle cell anaemia symptoms typically take 3-6 months to develop?

A

synthesis of HbF is normal so disease doesn’t manifest until HbF decreases to adult levels at about 6 months old

134
Q

why can patients with sickle cell anaemia appear well despite being anaemic?

A

HbS release oxygen to tissues more readily than normal RBCs - will still feel well

135
Q

what is the management for acute attacks in sickle cell anaemia?

A

IV fluids
analgesia
oxygen
antibiotics

136
Q

what investigations should be undertaken for G6PD?

A

blood film - irregularly contracted cells, bite cells, reticulocytosis
G6PD enzyme levels low

137
Q

what is the treatment for G6PD?

A
  • blood transfusion

- stop exacerbating drugs

138
Q

what is the epidemiology of non-hodgkins lymphoma?

A

rare before 40

strong link between EBV and Burkitt’s lymphoma

139
Q

which chemotherapy regimen is used for treating non-hodgkin’s lymphoma?

140
Q

what investigations should be undertaken for polycythaemia?

A
  • blood count = raised WBC and platelets
  • raised Hb
  • JAK2 mutation on genetic screen
  • bone marrow biopsy - proliferation of granulocytes and megakaryocytes
  • low serum EPO
141
Q

what are the complications of polycythaemia?

A

thrombosis

haemorrhage

142
Q

which investigations should be undertaken for immune thrombocytopenia purpura (ITP)?

A
  • bone marrow examination = thrombocytopenia with normal/increased megakaryocytes
  • platelet autoantibodies
143
Q

what are the causes of thrombotic thrombocytopenic purpura (TTP)?

A 
idiopathic
autoimmune e.g. SLE
cancer
pregnancy
drug related
144
Q

what is the clinical presentation of haemophilia A?

A
  • mild = bleed after trauma
  • moderate = bleeding after venepuncture and trauma
  • severe = neonatal bleeding, spontaneous bleed, GI bleeds, haematuria
145
Q

what is the treatment for haemophilia A?

A
  • factor VIII
  • fresh frozen plasma containing factor VIII for acute bleeds
  • desmopressin
146
Q

what type of inheritance is haemophilia?

A

x linked - male predominance

147
Q

what is the clinical presentation of haemophilia B?

A
  • bruising
  • nosebleeds
  • pallor
  • heavy bleeding from minor injury
  • bleeding from tooth loss in childhood
  • joint pain and stiffness
  • headache, vomiting
  • GI bleed
148
Q

what is the treatment for haemophilia B?

A

treatment must be started before diagnosis is confirmed

  • recombinant factor IX
  • vaccination against hep A and B
149
Q

what is von Willebrand’s disease?

A
  • vWF deficiency

- blood cannot clot- also called pseudohaemophilia- more common in females

150
Q

what is the clinical presentation of von Willebrnad’s disease?

A
  • Bleeding from mucosa
  • Epistaxis (nose bleeds)
  • Menorrhagia (heavy periods)
  • Spontaneous bleeding
  • Blood clots during childbirth (rare)
  • Spontaneous death (rare)
151
Q

what is the management for von Willebrand’s disease?

A
  • Education on bleeding risks
  • Stop any antiplatelet drugs and NSAIDs
  • Tranexamic acid - used for minor bleeds
  • Combined oral contraceptive to control menorrhagia in women
  • Desmopressin -> increases vWF levels
  • Platelet transfusions can be helpful in some
  • Family screening should be offered
152
Q

what are the causes of aplastic anaemia?

A
  • inherited
  • idiopathic
  • benzene
  • chemotherapeutic drugs
  • antibiotics
  • infections - EBV, HIV and TB
153
Q

what is the pathophysiology of aplastic anaemia?

A

due to a reduction in the number of pluripotent stem cells, together with a fault in those remaining or an immune reaction against them so they are unable to repopulate the bone marrow

154
Q

what is the clinical presentation of aplastic anaemia?

A
  • anaemia
  • increased susceptibility to infection
  • bleeding- bleeding gums, bruising and blood blisters in mouth
155
Q

what investigations are undertaken for aplastic anaemia?

A
  • blood cell count
    • pancytopenia and low reticulocyte count
      bone marrow examination
    • hypocellular with increased fat spaces
156
Q

what is the treatment for aplastic anaemia?

A
  • treat cause
  • antibiotics for neutropenia
  • RBC transfusion and platelets
  • bone marrow transplant
  • immunosuppressive therapy
    - ATG and ciclosporin if under 40
157
Q

what is haemolytic anaemia?

A
  • premature breakdown of RBCs before normal lifespan
158
Q

what are the causes of haemolytic anaemia?

A
  • RBC membrane defects
  • spherocytosis
  • enzyme defects
  • G6PD
  • alpha and beta thalassaemia
  • sickle cell anaemia
  • autoimmune haemolytic anaemia
159
Q

what is the epidemiology of myeloma?

A
  • older adults 75+

- more common in afro-caribbean’s

160
Q

what are the investigations for non-hodgkins lymphoma?

A

FBC - anaemia, high ESR
Raised lactose dehydrogenase
Lymph node biopsy
Bone marrow biopsy for classification

161
Q

what is the epidemiology of hodkins lymphoma?

A
  • Bimodal – peaks twice in life
  • Young adults and elderly
  • Male > female
  • Associated with EBV, SLE, siblings
162
Q

what is the epidemiology of non-hodgkins lymphoma?

A

Adults 40+
Associated with EBV and Burkitt’s lymphoma
Family history increases risk

163
Q

what are the different types of non-hodgkins lymphoma?

A

low grade = follicular lymphoma
high grade = diffused large B cell lymphoma
very high grade = burkitt’s lymphoma

164
Q

what is the epidemiology of AML?

A

● Progresses rapidly with death in 2 months if untreated
● Commonest acute leukaemia in adults
● Associations with radiation and Down’s

165
Q

what are the possible complications of AML?

A

infections such as sepsis

166
Q

what is the epidemiology of CML?

A
  • almost exclusively a disease of adults
  • occurs between 40-60
  • slight male predominance
167
Q

what is the epidemiology of CLL?

A
  • most common leukaemia- occurs later in life
  • mutations, trisomy’s and deletions influence risk
  • Pneumonia may be a triggering event
  • Progression – may stay stable for years or regress
  • Death often due to complication of infection
  • May transform into aggressive lymphoma – Richter’s syndrome
  • 1/3 will never progress, 1/3 progress slowly, 1/3 progress actively
168
Q

what is the pathophysiology of polycythaemia?

A

● A clonal stem cell disorder resulting in malignant proliferation of a clone derived from 1 pluripotent marrow stem cell
● Erythropoietin progenitor offspring do not need erythropoietin to avoid apoptosis
● Results in excess proliferation of RBCs, WBCs and platelets – cause raised haematocrit = hyper viscosity and thrombosis

169
Q

what is the epidemiology of von Willebrand’s disease?

A

Most common hereditary coagulopathy
Can be congenital or acquired
More common in females Poorer prognosis in blood type O

170
Q

what are the different types of von Willebrand’s disease?

A
  • 3 types: Type 1, 2, 3, platelet type
  • Type 1 is the most common (60-80%)
  • Platelet type is the most rare (70 cases)
171
Q

what are the investigations for von Willebrand’s disease?

A
  • FBC
  • Fibrinogen level
  • Platelet count - normal
  • Clotting screen- Plasma vWF decreased
  • Factor VIII levels - can be decreased as vWF is not present to protect it
172
Q

what is the epidemiology of haemophilia A?

A
  • majority are inherited
  • x-linked
  • male predominance
  • 5 x more common than haemophilia B
173
Q

what are the investigations for haemophilia A?

A
  • FBC - low haematocrit, low Hb
  • Normal prothrombin and bleeding time
  • Prolonged activated partial thromboplastin time
  • Heavily reduced factor VIII
  • CT head - look for haemorrhages
174
Q

what is the epidemiology of haemophilia B?

A
  • Caused by Factor IX deficiency
  • Male predominance (X-linked)
  • Mild disease has factor IX levels of 6-30%
  • Moderate disease has levels of 1-5%
  • Severe disease has levels of <1% - this accounts for 50% of cases
175
Q

what are the investigations for haemophilia B?

A
  • Normal prothrombin time
  • Prolonged activated partial thromboplastin time
  • Low factor IX levels
  • Endoscopy if GI bleed suspected
176
Q

what are the clinical features of heterozygous sickle cell anaemia (trait)?

A

Symptom free

Protection against falciparum malaria

177
Q

what are the clinical features of homozygous sickle cell anaemia?

A

Acute pain in hands and feet
Long bone pain, e.g. femur, spine, ribs
Cognitive defects in children
Pulmonary hypertension and chronic lung disease (most common cause of death)
Splenic sequestration (cells get trapped)

178
Q

what are the risk factors for sickle cell anaemia?

A

african

family history

179
Q

what is the pathophysiology of sickle cell anaemia?

A
  • Results from a single base emulation of adenine to thymine
    – valine substituted for glutamic acid at the 6th codon of the beta globin chain
  • HbS is insoluble and polymerises when oxygenated
  • Flexibility of cells is decreased – become rigid and sickled
  • Initially reversible but then go on to lose membrane flexibility and become irreversibly sickled – these cells are dehydrated and dense
  • Sickling results in a shorted RBC survival resulting in haemolysis and impaired passage of cells through the microcirculation leading to obstruction of small vessels and tissue infarction
180
Q

what is G6PD deficiency?

A

G6PD is protective against oxidative damage

G6PD deficiency results in reduced red blood cells lifespan

181
Q

what is the epidemiology of G6PD deficiency?

A

More common in Africa, the Middle East and SE Asia

More common in males

182
Q

what is the clinical presentation of microcytic anaemia?

A

in addition to anaemia symptoms:
● Brittle hair and nails
● Spoon shaped nails – koilonychia
● Atrophy of papillae of tongue – atrophic glossitis
● Angular stomatitis – ulceration of corners of the mouth

183
Q

what is the epidemiology of malaria?

A 
P. falciparum is the most common
P. malariae is the least common 
Most common in Africa 
Risks include: infants, pregnancy, old age
notifiable condition
184
Q

what are the different species of malaria?

A

4 species: P. falciparum, P. vivax, P. ovale P. malariae
P. falciparum is the most common
P. malariae is the least common

185
Q

what is malaria?

A

Parasitic infection, Plasmodium, spread by female anopheles mosquito

186
Q

what are the clinical features of malaria?

A

Non-specific symptoms

  • Fever, chills
  • Headache
  • Cough
  • Splenomegaly

Severe disease in P. falciparum

  • Shortness of breath
  • Fits and hypovolaemia
  • AKI and nephrotic syndrome
187
Q

when do clinical features of malaria present?

A

Symptoms occur from 6 days post infection

P. vivax and P. ovale commonly present 6 months post infection - can relapse as it can lay dormant in the liver for years.

188
Q

what are the investigations for malaria?

A
  • Travel history
  • Thick and thin blood smears
    - Negative film can be seen
    - If negative, 2 more films should be sent over the next 48 hours
  • Rapid diagnostic tests
    • Detect parasitic antigens
  • PCR- FBC, LFTs, U&Es, blood gases, blood culture
  • CXR, lumbar puncture
189
Q

what is the treatment for malaria?

A
  • Chloroquine for non-falciparum malaria
  • Oral quinine sulphate for falciparum
  • Add IV quinine dihydrochloride for severe disease
    Do not treat those with G6PD deficiency
    Need specialist help
190
Q

what are the risk factors for iron deficiency anaemia?

A

● Undeveloped countries
● High vegetable diet
● Premature infants
● Introduction of mixed feeding delayed

191
Q

what is anaemia of chronic disease?

A

Secondary to chronic disease

Occurs in chronic infections such as TB, Crohn’s, RA, SLE, malignancy

192
Q

what is the pathophysiology of anaemia of chronic disease?

A

● Decreased release of iron from bone marrow to developing erythroblasts
● Inadequate erythropoietin response to anaemia – decreased RBC survival

193
Q

what do the investigations for anaemia of chronic disease show?

A

● Low serum iron and iron-binding capacity
● Increased or normal ferritin due to inflammatory response
● RBCs normo/micro-cytic and hypochromic (pale)
● Serum soluble transferrin normal

194
Q

what is the management for anaemia of chronic disease?

A

● Treat underlying chronic condition

● Erythropoietin is effective in raising the Hb level and is used in cases of renal disease and inflammatory disease

195
Q

what are the side effects of oral iron treatment?

A

nausea, abdominal discomfort, diarrhoea/constipation, black stool

196
Q

what are the side effects of erythropoietin treatment?

A

flu-like symptoms,
hypertension,
raised platelets,
thromboembolism

197
Q

how long do folate stores last for?

198
Q

what is aplastic anaemia?

A
  • Rare stem cell disorder
  • Defined as pancocytopenia (this is a reduction in all the major cell lines; red cells, white cells and platelets) with hypocellularity (aplasia) of the bone marrow (marrow stops making cells)
  • It is usually an acquired condition but may be inherited
199
Q

what are the main causes of aplastic anaemia?

A
  • inherited - Fanconi’s anaemia
  • idiopathic- benzene, toulene, glue sniffing
  • chemotherapeutic drugs
  • antibiotics
  • infections - EBV, HIV, TB
200
Q

what is the pathophysiology of aplastic anaemia?

A

Due to a reduction in the number of pluripotent stem cells, together with afault in those remaining or an immune reaction against them so that they areunable to repopulate the bone marrow

201
Q

what are the clinical features of aplastic anaemia?

A
  • anaemia
  • increased susceptibility to infection
  • bleeding
  • bleeding gums
202
Q

what are the results for investigations for aplastic anaemia?

A
  • Blood count:
    • Pancocytopenia with low reticulocyte count
  • Bone marrow examination:
    • Hypocellular marrow with increased fat spaces
203
Q

what is the treatment of aplastic anaemia?

A
  • treat cause
  • RBC transfusion
  • bone marrow transplant
  • immunosuppressive therapy
    - ATG and ciclosporin
204
Q

what is the long term management for sickle cell anaemia?

A
  • hydroxyurea - increases the HbF levels and is used in the prophylactic management of sickle cell anaemia to prevent painful episodes
  • pneumococcal vaccine
205
Q

how is heparin treatment reversed?

A
  • proteamine
206
Q

what is the mechanism of action for aspirin?

A
  • irreversibly inhibits COX

- inhibits thromboxane formation and therefore platelet aggregation is inhibited

207
Q

what is the mechanism of action for clopidogrel?

A

inhibits ADP induced platelet aggregation

208
Q

what is the most common cell origin for lymphoma (both hodgkins and non-hodgkins)?

209
Q

what is ondansetron?

A

anti-emetic drug

210
Q

what is the mechanism of action for ondansetron?

A

5HT3 antagonist

211
Q

what is the mechanism of action for DOAC?

A

direct action on factor II or X

212
Q

What is myeloma?

A

Cancer of differentiated B lymphocytes (plasma cells)

Multiple myeloma is a palliative disease, so everyone will relapse.

213
Q

Describe the pathophysiology of myeloma

A

Accumulation of malignant plasma cells in bone –> progressive bone marrow failure
Production of one excess immunoglobulin (monoclonal paraprotein)

214
Q

What monoclonal paraprotein is usually produced in myeloma?

A 
IgG = 55%
IgA = 20%
215
Q

Give 3 symptoms of myeloma

A
  1. Tiredness
  2. Bone/back pain
  3. Infections
216
Q

Give 4 signs of myeloma

A 
old CRAB 
old =  over 75
1. Calcium is elevated 
2. Renal failure
3. Anaemia 
4. Bone lesions
217
Q

Why is calcium elevated in myeloma?

A

Increased bone resorption and decreased bone formation so more calcium in the blood

218
Q

Why might someone with myeloma have anaemia?

A

BM infiltrated with plasma cells = anaemia, neutropenia (infections) and thrombocytopenia (bleeding)

219
Q

Why might someone with myeloma have renal failure?

A

Due to light chain deposition

220
Q

What investigations might you do in someone who you suspect has myeloma?

A 
FBC - anaemia, raised ESR
Blood film - rouleaux 
Serum and urine electrophoresis - Bence Jones protein in urine 
X-rays - pepper pot skull, fractures
CT - bone lesions, cord compression
221
Q

What would you expect to see on the blood film taken from someone with myeloma?

A

Rouleaux formation (aggregation of RBCs)

222
Q

What are you looking for on a bone marrow biopsy taken from someone with myeloma?

A

Increase plasma cells

223
Q

What are you looking for on electrophoresis in a patient with myeloma?

A

Monoclonal protein band

224
Q

What would you expect to see on the x-ray taken from someone with myeloma?

A

Lytic ‘punched out’ lesions –> pepper pot skull, vertebral collapse
Fractures
Osteoporosis

225
Q

What might the urine of someone with myeloma contain?

A

Bence-jones protein - immunoglobulin light chains with kappa or lamda lineage

226
Q

What does a diagnosis of myeloma require?

A
  1. Monoclonal protein band in serum or urine
  2. Increased plasma cells on bone marrow biopsy
  3. Hypercalcaemia/renal failure/anaemia
  4. Bone lesion on skeletal survey
227
Q

Describe the treatment for symptomatic myeloma

A 
Bone pain = analgesia 
Bisphosphonates (zolendronate)   
Blood transfusion - for anaemia
Fluids and dialysis - renal failure
Chemotherapy 
Stem cell transplant
228
Q

How is myeloma bone disease usually assessed?

229
Q

Suggest 3 ways in which multiple myeloma can lead to AKI

A
  1. Deposition of light chain
  2. Hypercalcaemia
  3. Hyperuricaemia
230
Q

What kind of anaemia is seen in patients with multiple myeloma?

A

Normochromic normocytic

231
Q

What chemotherapy regime is used in patients with myeloma?

A
  • CTD - Cyclophosphamide, thalidomide and dexamethasone

- VAD - Vincristine, Adriamycin, dexamethasone (for fitter people)

232
Q

What is leukaemia?

A

A malignant proliferation of haemopoietic stem cells (immature blood cells)

233
Q

Name 4 sub types of leukaemia

A
  1. AML - Acute Myeloid Leukaemia
  2. CML - Chronic Myeloid Leukaemia
  3. ALL - Acute Lymphoblastic Leukaemia
  4. CLL - Chronic Lymphoblastic Leukaemia
234
Q

What is acute myeloid leukaemia (AML)?

A

Neoplastic proliferation of blast cells (immature blood cells)
affects myeloid progenitor cells and myeloblasts

235
Q

What are the risk factors for AML?

A
  1. Preceding haematological disorders
  2. Prior chemotherapy
  3. Exposure to ionising radiation
  4. Down’s syndrome
236
Q

what are the clinical features of AML?

A
  • Anaemia -> breathlessness, fatigue, pallor
  • low WCC = Infection
  • low platelets = bleeding/bruising
  • Hepatosplenomegaly
  • Peripheral lymphadenopathy
  • Gum hypertrophy
  • Bone marrow failure and bone pain
237
Q

Why are anaemia, infection and bleeding symptoms of leukaemia?

A

Because of bone marrow failure

238
Q

Why are hepatomegaly and splenomegaly symptoms of leukaemia?

A

Because of tissue infiltration

239
Q

What investigations do you do on someone who you suspect has AML?

A
  • FBC - anaemia, thrombocytopaenia
  • WCC often raised (can be normal/low)
  • Blood film - leukaemic blast cells
  • Bone marrow biopsy - Auer rods
240
Q

What would you expect to see on an FBC and bone marrow biopsy in someone you suspect to have AML?

A

FBC = anaemia and thrombocytopenia and neutropenia

BM biopsy = leukaemic blast cells (with Auer rods)

241
Q

What is chronic myeloid leukaemia (CML)?

A

Uncontrolled clonal proliferation of myeloid cells (basophils, eosinophils and neutrophils)

242
Q

What chromosome is present in >80% of people with CML?

A

Philadelphia chromosome

forms a fusion gene BCR/ABL on chromosome 22 – has tyrosine kinase activity – simulate cell division

243
Q

what are the clinical features of CML?

A
  1. Symptomatic anaemia
  2. Abdominal pain = splenomegaly
  3. Weight loss, tiredness, palor
  4. Gout - due to purine breakdown
  5. Bleeding - due to platelet dysfunction
  6. fever and sweats in absence of infection
244
Q

what are the investigations for CML?

A
  • FBC - anaemia, raised myeloid cells, high WCC (eosinophilia, basophilia, neutrophilia)
  • Increased B12
  • Blood film - left shirt, basophilia
  • Bone marrow biopsy - increased cellularity (hypercellular)
  • Philadelphia chromosome seen in 80+% of cases  t(9;2) - Stimulates cell division
245
Q

What is the treatment for CML?

A
  • Chemotherapy
  • Tyrosine kinase inhibitors, e.g. Imatinib - Given orally
  • Stem cell transplant
246
Q

Why does the Philadelphia chromosome cause CML?

A

Froms fusion gene BCR/ABL on chromosome 22 –> tyrosine kinase activity –> stimulates cell division

247
Q

What is acute lymphoblastic leukaemia (ALL)?

A

Uncontrolled proliferation of immature lymphoblast cells (lymphoid progenitor cells)
affects lymphoid progenitor cells and lymphoblasts

248
Q

what are the clinical features of ALL?

A
  • Anaemia = breathlessness, fatigue, pallor
  • Low WCC = Infection
  • low platelets = bleeding and bruising
  • Hepatosplenomegaly
  • Peripheral lymphadenopathy
  • CNS involvement - headache, cranial nerve palsies
  • SVC obstruction, dilated superficial chest veins
  • Bone marrow failure and bone pain
249
Q

What is the treatment for ALL?

A
  • Blood and platelet transfusion
  • Chemotherapy
  • Steroids
  • Allopurinol to prevent tumour lysis syndrome
  • Intrathecal drugs, e.g. methotrexate
  • Acute control of infections with IV antibiotics
  • Neutropenia makes this high risk
  • Stem cell transplant
250
Q

What is Chronic lymphoblastic leukaemia (CLL)?

A

Proliferation of mature B lymphocytes leads to accumulation of mature B cells that have escaped apoptosis
Chronic malignant transformation of mature lymphoid cells

251
Q

Describe the treatment for AML

A
  • Blood and platelet transfusions
  • IV fluids
  • Allopurinol to prevent tumour lysis
  • Infection control with IV antibiotics
  • Chemotherapy
  • Steroids
  • Sibling matched allogenic bone marrow transplant

Phase 2A revision (38 decks)

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