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My Phase 2a > Haematology Full > Flashcards

Haematology Full Flashcards

1
Q

What is myeloma?

A

Malignancy of plasma cells leading to progressive bone marrow failure. It is associated with production of characteristic paraprotein, bone disease, anaemia and renal failure.

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2
Q

In order to make a diagnosis of myeloma, there must be evidence of mono-clonality. What is mono-clonality?

A

Abnormal proliferation of a single clone of plasma cell leading to immunoglobulin secretion and causing organ dysfunction especially to the kidney.

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3
Q

What disease often precedes myeloma?

A

Monoclonal gammopathy of undetermined significance (MGUS).

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4
Q

What is MGUS?

A

A common disease with paraprotein present in the serum but no myeloma. Often asymptomatic. <10% plasma cells in the bone marrow.

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5
Q

In approximately 2/3 of people with myeloma, what might their urine contain?

A

Bence-jones proteins: light chains in urine

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6
Q

Give 3 symptoms of myeloma.

A
  1. Tiredness.
  2. Bone/back pain.
  3. Infections.
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7
Q

Give 4 key feature of myeloma.

A

CRAB!

  1. Calcium is elevated.
  2. Renal failure.
  3. Anaemia.
  4. Bone lesions.
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8
Q

Why is calcium elevated in myeloma?

A

There is increased bone resorption and decreased formation meaning there is more calcium in the blood.

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9
Q

Why might someone with myeloma have anaemia?

A
  • The bone marrow is infiltrated with plasma cells, causing suppression of other blood cell lines.
  • Consequences of this are anaemia, neutropenia, and thrombocytopenia –> tiredness, infections and bleeding.
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10
Q

Why might someone with myeloma have renal failure?

A
  • light chain deposition in the kidney tubules
  • calcium deposition within the kidney parenchyma
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11
Q

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

A
  1. Blood film: rouleaux formation of RBCs
  2. Bone marrow aspirate and trephine biopsy: ≥ 10% plasma cell infiltration.
  3. Electrophoresis of urine and blood serum.
  4. X-ray.
  5. CT scan.
  6. MRI scan.

B–Bence–Jones protein (requesturine electrophoresis)
L– Serum‑freeLight‑chain assay
I– SerumImmunoglobulins
P– SerumProtein electrophoresis

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12
Q

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

A

Rouleaux formation (aggregations of RBC’s).

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13
Q

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

A

Increased plasma cells.

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14
Q

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

A

Monoclonal protein band.

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15
Q

What are you looking for on an X-ray taken from someone with myeloma?

A

Bone lesions.

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16
Q

What is the treatment for MGUS and asymptomatic myeloma?

A

Watch and wait.

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17
Q

Describe the treatment for symptomatic myeloma.

A
  1. chemo
  2. stem cell transplant
  3. bisphosphonates for bone treatment
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18
Q

What is lymphoma?

A

A malignant growth of WBC’s predominantly in the lymph nodes.

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19
Q

Although predominantly in the lymph nodes, lymphoma is systemic. What other organs might it effect?

A
  1. Blood.
  2. Liver.
  3. Spleen.
  4. Bone marrow.
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20
Q

Give 4 risk factors for lymphoma.

A
  1. family hx.
  2. Secondary immunodeficiency e.g. HIV.
  3. EBV Infection.
  4. Autoimmune disorders e.g. RA.
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21
Q

Describe the pathophysiology of lymphoma.

A

There is impaired immunosurveillance and infected B cells escape regulation and proliferate. (This is just a theory).

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22
Q

Give 4 symptoms of lymphoma.

A
  1. Enlarged painless lymph nodes in arm/neck.
  2. itching.
  3. General systemic ‘B’ symptoms e.g. weight loss, night sweats, malaise.
  4. Pain in lymph nodes when drinking alcohol [HL only].
  5. SoB, cough and dyspnoea due to compression of mediastinal lymph nodes.

also recurrent infection

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23
Q

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

A
  1. Lymph node ultrasound [1st line]
  2. Lactate dehydrogenase blood test: elevated.
  3. lymph node biopsy = diagnostic: shows Reed-Sternberg cells.[gold standard]
  4. CT scan for staging
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24
Q

What are the two sub-types of lymphoma?

A
  1. Hodgkins lymphoma.

2. Non-hodgkins lymphoma.

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25
Q

What are the symptoms of Hodgkins lymphoma?

A
  1. Painless lymphadenopathy.
  2. Presence of ‘B’ symptoms e.g. night sweats, weight loss.
  3. lymph node pain triggered by alcohol
  4. pruritus
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26
Q

What is a key feature for diagnosis of Hodgkins lymphoma?

A

Presence of Reed-sternberg cells.

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27
Q

Describe the staging of Hodgkins lymphoma.

A
  1. Stage 1: confined to a single lymph node region.
  2. Stage 2: Involvement of two or more nodal areas on the same side of the diaphragm.
  3. Stage 3: involvement of nodes on both sides of the diaphragm.
  4. 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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28
Q

What are the treatment options for Hodgkins lymphoma?

A
  • chemotherapy
  • radiotherapy.
  • rituximab
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29
Q

what are the potential side effects of Hodgkin’s lymphoma treatment

A

Chemotherapy creates a risk of leukaemia and infertility.

Radiotherapy creates a risk of cancer, damage to tissues and hypothyroidism.

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30
Q

What are the possible complications of treatment for Hodgkins lymphoma?

A
  1. Secondary malignancies.
  2. IHD.
  3. Infertility.
  4. Nausea.
  5. Alopecia.
  6. Tumour lysis syndrome
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31
Q

Describe low grade non-hodgkins lymphoma.

A

Slow growing
advanced at presentation
often incurable.
Median survival is 10 years.

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32
Q

What is the treatment for low grade non-hodgkins lymphoma?

A

If symptomless - watchful waiting.

Radiotherapy, combination chemotherapy and mAb and stem cell transplant may be used if symptomatic.

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33
Q

Describe high grade non-hodgkins lymphoma.

A
  • Aggressive.
  • Nodal presentation, patient unwell with symptoms.
  • Often curable.
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34
Q

Describe the treatment for high grade non-hodgkins lymphoma.

A
  • Early: short course chemotherapy and radiotherapy.

- Advanced: combination chemotherapy and mAb.

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35
Q

what is bone marrow failure

A

the decreased production of one or more major hematopoietic lineage -> various cytopenias

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36
Q

symptoms of bone marrow failure

A
  • Fatigue.
  • Shortness of breath.
  • Pale appearance.
  • Frequent infections.
  • Easy bruising or bleeding.
  • Bone pain
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37
Q

define pancytopenia

A

low:
- red blood cellls
- platelets
- white blood cel

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38
Q

signs of bone marrow failure on a blood test

A
  • thrombocytopenia
  • leukopenia
  • anaemia
  • neutropenia
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39
Q

What is leukaemia?

A

A malignant proliferation of haemopoietic stem cells.

can have mixed lineage or heterogenous lineage

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40
Q

Name 4 sub-types of leukaemia.

A
  1. AML - acute myeloid leukaemia.
  2. CML - chronic myeloid leukaemia.
  3. ALL - acute lymphoblastic leukaemia. [Ab]
  4. CLL - chronic lymphocytic leukaemia. [Cc]
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41
Q

What is acute myeloid leukaemia?

A

Rapid, neoplastic proliferation of myeloblast cells in the bone marrow and blood.

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42
Q

What can increase the risk of developing AML?

A
  1. Preceding myeloproliferative disorders.
  2. Prior chemotherapy.
  3. Exposure to ionising radiation.
  4. increasing age
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43
Q

what is a granulocyte

A

a type of WBC with granules, namely:
- basophils,
- eosinophils
- neutrophils

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44
Q

what is a myeloblast

A

A myeloblast is an immature white blood cell

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45
Q

Give 5 symptoms of leukaemia.

A
  1. fatigue.
  2. recurrent infection.
  3. Bleeding + bruising.
  4. Fever.
  5. Splenomegaly.
  6. Hepatomegaly
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46
Q

Why are anaemia, infection and bleeding symptoms of leukaemia?

A

Because of bone marrow failure.

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47
Q

Why are hepatomegaly and splenomoegaly symptoms of leukaemia?

A

Because of tissue infiltration.

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48
Q

What investigations might you do on someone who you suspect has leukaemia?

A
  1. Blood film.
  2. Bone marrow biopsy.
  3. Lymph node biopsy.
  4. Immunophenotyping.
  5. Cytogenetics.
  6. FBC
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49
Q

Describe the treatment for AML.

A
  1. Supportive care.
  2. Chemotherapy: curative v palliative.
  3. Bone marrow transplant.
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50
Q

What is CML?

A

Chronic myeloid leukaemia, there is uncontrolled clonal proliferation of mature myeloid cells within the bone marrow and blood: basophils, eosinophils and neutrophils [granulocytes]

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51
Q

What would the FBC from someone with CML look like?

A
  • LeukocytosisHigh WBC’s.
  • thrombocytopenia
  • anaemia
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52
Q

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

A

Philadelphia chromosome 9:22

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53
Q

What are the treatment options for CML?

A
  • Tyrosine kinase inhibitors +/- interferon alpha inibitors.
  • chemo
  • stem cell transplant
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54
Q

What is ALL?

A
  • Acute lymphoblastic leukaemia.
  • There is rapid uncontrolled proliferation of lymphocyte precursor cells.
  • usually affects B-cells over T-cells.
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55
Q

What is the treatment for ALL?

A

chemotherapy and stem cell transplant.

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56
Q

What is CLL?

A

Chronic lymphoid leukaemia. Proliferation of B lymphocytes leads to the accumulation of mature B cells that have escaped apoptosis and are incompetent

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57
Q

What is the treatment for CLL?

A
  1. watch + wait in early stages.
  2. Chemotherapy.
  3. Bone marrow transplant.
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58
Q

Name the 3 broad categories of red cell disorders.

A
  1. Haemoglobinopathies.
  2. Membranopathies.
  3. Enzymopathies.
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59
Q

What is normal adult haemoglobin made of?

A

2 alpha and 2 beta chains.

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60
Q

What is foetal haemoglobin made of?

A

2 alpha and 2 gamma chains.

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61
Q

What is haemoglobin S?

A
  • Haemoglobin S is a variant of Hb arising from a point mutation in the beta globin gene.
  • The mutation causes a single amino acid change, valine -> glutamine.
  • causes sickle cell anaemia
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62
Q

What is sickle cell disease?

A

A haemoglobin disorder of quality. HbS polymerises -> sickle shaped RBC.

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63
Q

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

A

Carriage offers protection against falciparum malaria.

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64
Q

Describe the inheritance pattern of sickle cell disease.

A

Autosomal recessive.
Sickle cell disease is homozygous SS.

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65
Q

If both parents are carriers of the sickle trait. What is the chance that their first child will have sickle cell disease?

A

Their offspring have a 1/4 chance of being affected with a sickle cell disease. (50% chance of being a carrier).

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66
Q

How long do sickle cells last for?

A

5-10 days - this explains why sickle cell disease is described as haemolytic.

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67
Q

Give 4 acute complications of sickle cell disease.

A
  1. Very painful crisis.
  2. Stroke in children.
  3. Cognitive impairment.
  4. Infections.
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68
Q

Give 3 chronic complications of sickle cell disease.

A
  1. Renal impairment.
  2. Pulmonary hypertension.
  3. Joint damage.
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69
Q

Describe the treatment for sickle cell disease.

A
  1. Transfusion.
  2. Hydroxycarbamide ↑ HbF which is protective against sickling.
  3. Stem cell transplant.
  4. analgesia
  5. Lifelong phenoxymethylpenicillin as prophylaxis due to autosplenectomy. Folic acid supps
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70
Q

What is thalassaemia?

A

A haemoglobin disorder of quantity.
There is reduced synthesis of one or more globin chains -> a reduction in Hb -> anaemia.

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71
Q

If someone has beta thalassaemia do they have more alpha or beta globin chains?

A

They have very few beta chains, alpha chains are in excess.

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72
Q

What is the clinical classification of beta thalassaemia?

A
  1. Thalassaemia major.
  2. Thalassaemia intermedia.
  3. Thalassaemia carrier/heterozygote.
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73
Q

Which clinical classification of thalassaemia relies on regular transfusions?

A

Thalassaemia major.

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74
Q

Which clinical classification of thalassaemia is often asymptormatic?

A

Thalassaemia carrier/heterozygote.

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75
Q

When do people with beta thalassaemia major usually present and why?

A

These patients usually present very young due to having severe anaemia and so a failure to feed/thrive.

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76
Q

Why is it important to monitor iron levels in someone with beta thalassaemia major?

A

There is a risk of iron overload from the regular trasnfusions. Excess iron will be deposited in various organs e.g. the liver and spleen and cause fibrosis.

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77
Q

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

A

Parvovirus is a common infection in children. It leads to decreased RBC production and can cause a dramatic drop in Hb in patients who already have a reduced RBC lifespan. This can be dangerous for someone with sickle cell.

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78
Q

Describe the inheritance pattern for membranopathies.

A

Autosomal dominant.

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79
Q

Name 2 common membranopathies.

A
  1. Spherocytosis.

2. Elliptocytosis.

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80
Q

Briefly describe the physiology of membranopathies.

A

Deficiency of red cell membrane proteins caused by genetic lesions

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81
Q

What are enzymopathies?

A

Enzyme deficiencies lead to shortened RBC lifespan.

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82
Q

Name a common enzymopathy.

A

G6PD deficiency.

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83
Q

Give 3 signs of G6PD deficiency.

A

Crises characterised by:

  1. Haemolysis.
  2. Jaundice.
  3. Anaemia.
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84
Q

What is anaemia?

A

A decrease in the amount of Hb in the blood below the reference range.

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85
Q

What is the function of Hb?

A

It carries and delivers oxygen to tissues.

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86
Q

What organs are responsible for removal of RBC’s?

A
  1. Spleen.
  2. Liver.
  3. Bone marrow.
  4. Blood loss.
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87
Q

Give 3 causes of microcytic anaemia.

A
  1. Iron deficiency.
  2. Anaemia of chronic disease.
  3. Thalassaemia.
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88
Q

Give 3 causes of normocytic anaemia.

A
  1. Acute blood loss.
  2. Anaemia of chronic disease.
  3. Combined hematinic deficiency.
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89
Q

Give 3 causes of macrocytic anaemia.

A
  1. B12/folate deficiency.
  2. Alcohol excess/liver disease.
  3. Hypothyroid.
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90
Q

Where is B12 absorbed?

A

The terminal ileum.

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91
Q

Explain how pernicious anaemia leads to B12 deficiency.

A

Pernicious anaemia leads to a loss of parietal cells -> reduced intrinsic factor production -> vitamin B12 malabsorption.

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92
Q

Give 5 causes of iron deficiency.

A
  1. Blood loss.
  2. Poor absorption.
  3. Decreased intake in diet.
  4. Hook worm!
  5. Breastfeeding, low iron in breast milk.
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93
Q

Give 3 symptoms of anaemia.

A
  1. Fatigue.
  2. Faintness.
  3. Breathlessness.
  4. Reduced exercise tolerance.
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94
Q

What investigations might you do in someone with anaemia?

A
  1. Blood tests: FBC and blood film.
  2. Biopsies.
  3. Reticulocyte count.
  4. B12 levels.
  5. Serum ferritin.
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95
Q

What is the treatment for anaemia?

A

Treat the underlying cause e.g. if iron deficient give ferrous sulphate.

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96
Q

What is polycythaemia?

A

Too many RBC’s, an increase in Hb.

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97
Q

What hormone is responsible for regulating RBC production?

A

Erythropoietin.

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98
Q

What stimulates EPO?

A

Tissue hypoxia.

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99
Q

Name a primary cause of polycythaemia.

A

Polycythaemia rubra vera - over reactive bone marrow.

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100
Q

Give 3 secondary causes of polycythaemia.

A
  1. Heavy smoking.
  2. Lung disease.
  3. Cyanotic heart disease.
  4. High altitude.
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101
Q

What is the treatment for polycythaemia?

A
  1. If a secondary cause treat the underlying cause.

2. If a primary cause, treatment aims to maintain a normal blood count and prevent complications e.g. aspirin.

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102
Q

What is neutrophilia?

A

Too many neutrophils.

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103
Q

Give 3 causes of neutrophilia.

A
  1. Infection.
  2. Inflammation.
  3. CML.
  4. Cancer.
104
Q

What is lymphocytosis?

A

Too many lymphocytes.

105
Q

Give 3 causes of lymphocytosis.

A
  1. Viral infections.
  2. Inflammation.
  3. Malignancy.
  4. CLL.
106
Q

What is thrombocytopenia?

A

Not enough platelets.

107
Q

Give 2 causes of thrombocytopenia.

A
  1. Production failure e.g. marrow suppression, marrow failure.
  2. Increased removal e.g. immune response (ITP), consumption (DIC), splenomegaly.
108
Q

What is thrombocytosis?

A

Too many platelets.

109
Q

What is neutropenia?

A

Not enough neutrophils.

110
Q

What is the major risk associated with being neutropenic?

A

Susceptible to infection.

111
Q

Give 3 causes of neutropenia.

A
  1. Marrow failure.
  2. Marrow infiltration.
  3. Marrow toxicity.
112
Q

Where are platelets produced?

A

In the bone marrow. They are fragments of megakaryocytes.

113
Q

What hormone regulates platelet production?

A

Thrombopoietin - produced mainly in the liver.

114
Q

What is the lifespan of a a platelet?

A

7 - 10 days.

115
Q

What organ is responsible for platelet removal?

A

The spleen.

116
Q

What can cause platelet dysfunction.

A
  1. Reduced platelet number (thrombocyotpenia).

2. Reduced platelet function.

117
Q

Platelet dysfunction: 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.
118
Q

Platelet dysfunction: 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.
119
Q

Platelet dysfunction: what can cause reduced platelet function?

A
  1. Congenital abnormality.
  2. Medication e.g. aspirin.
  3. VWF disease.
  4. Uraemia.
120
Q

Give 3 symptoms of platelet dysfunction.

A
  1. Mucosal bleeding.
  2. Easy bruising.
  3. Petechiae/purpura.
121
Q

Give 4 causes of bleeding.

A
  1. Trauma.
  2. Platelet deficiency e.g. thrombocytopenia.
  3. Platelet dysfunction e.g. aspirin induced.
  4. Vascular disorders.
122
Q

What is the definition of febrile neutropenia.

A

Temperature >38°C in a patient with neutrophil count <1x10^9/L.

123
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.
124
Q

What is the presentation of febrile neutropenia?

A
  1. Pyrexia, 38°C.
  2. Generally unwell.
  3. Confusion.
  4. Hypotensive.
  5. Tachycardic.
125
Q

Describe the management of febrile neutropenia.

A
  1. Thorough history and examination.
  2. Bloods.
  3. Antibiotics within 1 hour!
126
Q

Give a risk factor for spinal cord compression.

A

Any malignancy that can cause compression e.g. bone metastasis.

127
Q

Describe the presentation of spinal cord compression.

A
  1. Back pain.
  2. Weakness in legs.
  3. Inability to control bladder.
  4. Spastic paresis.
  5. Sensory level.
128
Q

Describe the management of spinal cord compression.

A
  1. Bed rest.
  2. High dose steroids.
  3. Analgesia.
  4. Urgent MRI of the whole spine.
129
Q

What is tumour lysis syndrome?

A

Break down of malignant cells -> content release -> metabolic disturbances; can cause hyperuricaemia, hyperkalaemia, hypocalcaemia.

130
Q

Give 3 risk factors for tumour lysis syndrome.

A
  1. High tumour burden.
  2. Pre-existing renal failure.
  3. Increasing age.
131
Q

Describe the treatment of tumour lysis syndrome.

A
  1. Aggressive hydration.
  2. Monitor electrolytes.
  3. Drugs to reduce uric acid production e.g. allopurinol.
132
Q

What is hyperviscosity syndrome?

A

Increase in blood viscosity usually due to high levels of immunoglobulins.

133
Q

Give 2 consequences of hyperviscosity syndrome.

A
  1. Vascular stasis.

2. Hypoperfusion.

134
Q

Describe the presentation of hyperviscosity syndrome.

A
  1. Mucosal bleeding.
  2. Visual change.
  3. Neurological disturbances.
  4. Breathlessness.
  5. Fatigue.
135
Q

What investigations might you do in someone who you suspect has hyperviscosity syndrome?

A
  1. FBC and blood film; look for rouleaux formation.
  2. U&E.
  3. Immunoglobulins.
136
Q

What is the treatment for hyperviscosity syndrome?

A
  1. Keep hydrated!
  2. Avoid blood transfusion.
  3. Treat the underlying cause.
137
Q

Give 5 signs/symptoms of hypercalcaemia.

A
  1. Confusion.
  2. Bone pain.
  3. Constipation.
  4. Nausea.
  5. Abdominal pain.
138
Q

What might you see in the ECG taken from someone with hypercalcaemia.

A

Shortened QT interval.

Hypercalcaemia = risk of MI.

139
Q

What is the treatment for hypercalcaemia?

A

IV hydration and bisphosphonates.

140
Q

What does rituximab target?

A

Targets CD20 on the surface of B.

141
Q

What is the characteristic genetic abnormality in chronic myeloid leukaemia?

A

t(9; 22) - philadelphia chromosome.

142
Q

How is myeloma bone disease usually assessed?

A

X-ray.

143
Q

What is the commonest cause of microcytic anaemia?

A

Iron deficiency.

144
Q

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

A

Reticulocyte count is raised.

145
Q

Why is reticulocyte count raised in sickle cell anaemia?

A

Sickle cell disease is haemolytic, there is increased degradation of RBC’s. Production therefore increases in order to keep up with degradation and so reticulocyte count is raised.

146
Q

What clotting factors depend on vitamin K?

A

2, 7, 9 and 10.

147
Q

Haemophilia A is due to deficiency of what clotting factor?

A

Factor 8 deficiency.

148
Q

Haemophilia B is due to deficiency of what clotting factor?

A

Factor 9 deficiency.

149
Q

Give 4 symptoms of ALL.

A
  1. Bone pain.
  2. Recurrent infections (neutropenia).
  3. Pale and tired (anaemia).
  4. Bruising (low platelets).
150
Q

Is ALL more common in adults or children?

A

ALL is mainly a childhood disease.

151
Q

What kind of anaemia could methotrexate cause?

A

Macrocytic due to folate deficiency.

152
Q

Give 4 causes of folate deficiency.

A
  1. Dietary.
  2. Malabsorption.
  3. Increased requirement e.g. in pregnancy.
  4. Folate antagonists e.g. methotrexate.
153
Q

Give 3 signs of haemolytic anaemia.

A
  1. Pallor.
  2. Jaundice.
  3. Splenomegaly.
154
Q

Give 4 causes of haemolytic anaemia.

A
  1. GP6D deficiency.
  2. Sickle cell anaemia.
  3. Spherocytosis/elliptocytosis (membranopathies).
  4. Autoimmune haemolytic anaemia.
155
Q

Give 3 things that can cause coagulation disorders.

A
  1. Vitamin K deficiency.
  2. Liver disease.
  3. Congenital e.g. haemophilia.
156
Q

How does warfarin work?

A

It antagonises vitamin K and so you get a reduction in clotting factors 2, 7, 9 and 10.

157
Q

How does heparin work?

A

It activates antithrombin which then inhibits thrombin and factor Xa.

158
Q

What is disseminated intravascular coagulation (DIC)?

A

Pathological activation of the coagulation cascade -> fibrin in vessel walls. There is platelet (thrombocytopenia) and coagulation factor consumption.

159
Q

Give 3 causes of disseminated intravascular coagulation (DIC).

A
  1. Sepsis.
  2. Major trauma.
  3. Malignancy.
160
Q

What is the affect on TT, PTT and APTT in someone with disseminated intravascular coagulation (DIC)?

A

All increased.

161
Q

What is the affect on fibrinogen in someone with disseminated intravascular coagulation (DIC)?

A

Decreased.

162
Q

Give 5 risk factors for DVT.

A
  1. Increasing age.
  2. Obesity.
  3. Pregnancy.
  4. OCP (hyper-coagulability).
  5. Major surgery.
  6. Immobility.
  7. Past DVT.
163
Q

Give 3 symptoms of DVT.

A

Unilateral warm, tender, painful, swollen leg.

164
Q

What forms the differential diagnosis for a DVT?

A

Cellulitis.

165
Q

What investigations might you do in someone to see if they have a DVT?

A
  1. D-dimer in those patients with a low clinical probability.
  2. US compression.
166
Q

What is the name of the score used to determine someones probability of having a DVT?

A

The Wells score.

167
Q

The Wells score determines someones clinical probability of having a DVT. Give 3 factors the score takes into account.

A
  1. Active cancer.
  2. Recently bedridden or major surgery.
  3. Tenderness along deep venous system.
  4. Swollen leg/calf.
  5. Unilateral pitting oedema.
168
Q

Describe the management for a DVT.

A

Aim of management is to prevent a PE!

- Anticoagulants e.g. warfarin/heparin.

169
Q

Explain why philadelphia chromosome causes CML.

A

Philadelphia chromosome leads to a fusion gene that has tyrosine kinase activity and enhanced phosphorylating activity -> altered cell growth.

170
Q

Where would you normally take a bone marrow biopsy from?

A

Posterior iliac crest.

171
Q

What is the most important medical treatment for DVT prophylaxis?

A

LMWH.

172
Q

What is the affect of iron deficiency anaemia on iron binding capacity?

A

Iron binding capacity will be raised.

173
Q

Why might measuring serum ferritin be inaccurate for looking at iron levels?

A

Ferritin is an acute phase protein and so its concentration will increase in response to inflammation.

174
Q

Describe the treatment for iron deficiency anaemia.

A

Ferrous sulphate tablets.

175
Q

What is aplastic anaemia?

A

When bone marrow stem cells are damaged -> pancytopenia.

176
Q

Give 3 symptoms of iron-deficiency anaemia.

A
  1. Koilonychia.
  2. Brittle hair and nails.
  3. Atrophic glossitis.
  4. Tiredness, reduced exercise tolerance.
  5. SOB.
177
Q

What is the difference between Raynaud’s disease and Raynaud’s phenomenon?

A

Raynaud’s disease is idiopathic.

Raynaud’s phenomenon can be due to SLE, scleroderma, RA, drugs e.g. beta blockers.

178
Q

Describe the pathophysiological mechanism behind Raynaud’s disease.

A

Peripheral digital ischaemia due to intermittent spasm in arteries that supply the fingers/toes. Precipitated by cold/stress.

179
Q

Describe the colour changes that are seen in Raynaud’s.

A
  • Pale - due to vasoconstriction.
  • Cyanotic - due to deoxygenation.
  • Red - due to hyperaemia.
180
Q

Describe the treatment for Raynaud’s disease.

A
  1. Physical protection.
  2. Vasodilators.
  3. Nifedipine (CCB).
  4. Stop smoking.
181
Q

State two features with regards to red blood cell appearance that would make you think a patient had anaemia due to iron deficiency.

A
  1. Hypochromia (pale).

2. Microcytosis.

182
Q

Suggest 3 ways in which multiple myeloma can lead to AKI.

A
  1. Deposition of light chain.
  2. Hypercalcaemia.
  3. Hyperuricaemia.
183
Q

In extreme cases, patients with myeloma can present with blurred vision, gangrene and bleeding. What is the pathology behind this?

A

Paraproteins form aggregates in the blood and change the viscosity.

184
Q

Why are patients with myeloma susceptible to recurrent infections?

A

There is a reduction in polyclonal immunoglobulin levels.

185
Q

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

A

Normochromic normocytic.

186
Q

What chemotherapy regime is used in patients with myeloma?

A

VAD or CTD.

187
Q

What combination chemotherapy regime is commonly used in patients with non hodgkin’s lymphoma?

A

RCHOP.

188
Q

Give 3 environmental causes of leukaemia.

A
  1. Radiation exposure.
  2. Chemicals e.g. benzene compounds.
  3. Drugs.
189
Q

Give 3 signs of anaemia.

A
  1. Pale skin and mucous membranes.
  2. Tachycardia.
  3. Bounding pulse.
190
Q

Describe the appearance of RBC’s seen in iron deficiency anaemia.

A

Hypochromic microcytic.

191
Q

What might you see on a blood count taken from someone with beta thalassaemia major?

A
  1. Raised reticulocyte count.

2. Microcytic anaemia.

192
Q

What can precipitate sickling in sickle cell anaemia?

A

Trauma, cold, stress, exercise.

193
Q

Why does sickle cell anaemia not present until after 6 months of age?

A

HbF is not affected by sickle cell anaemia as it is made up of 2 alpha and 2 gamma chains.

194
Q

What drug can be used to prevent painful crises in people with sickle cell anaemia?

A

Hydroxycarbamide.

195
Q

Give 3 clinical features of a patient with a membranopathy.

A
  1. Jaundice.
  2. Anaemia.
  3. Splenomegaly.
196
Q

Why does a deficiency in glucose-6-phosphate dehydrogenase lead to shortened red cell lifespan?

A

G6PD protects cells against oxidative damage.

197
Q

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

A
  1. Hb.
  2. RCC.
  3. PCV.
198
Q

Give 3 symptoms of polycythaemia.

A
  1. Itching.
  2. Headache.
  3. Dizziness.
  4. Visual disturbance.
199
Q

What can cause ITP?

A
  1. Viral infection.

2. Malignancy.

200
Q

What antibodies are often found in ITP?

A

IgG.

201
Q

Are PTT and APTT increased or decreased in DIC?

A

Increased.

202
Q

Are fibrinogen and platelets increased or decreased in DIC?

A

Decreased.

203
Q

Name 2 things that are increased and 2 that are decreased in DIC.

A

Increased: PTT and APTT.

Decreased: fibrinogen and platelets.

204
Q

How would you treat haemophilia A?

A

IV infusion of factor 8.

205
Q

causes of microcytic anaemia

A
  • iron deficiency
  • thalassemias - alpha and beta
206
Q

causes of normocytic anaemias

A
  • acute bleeding
  • chronic disease
  • renal disease
  • mixed picture = mix of big and small RBCs → normal mean size.
207
Q

causes of Macrocytic anaemias

A
  • folate deficiency
  • B12 deficiency
  • haemolysis
  • bone marrow disorders
208
Q

what are hypo chromic cells

A

pale RBCs due to reduced amounts of Hb - iron deficiency

209
Q

what factors lead to decreased RBC production

A
210
Q

what factors lead to increased increased RBC loss

A
  • Acute bleeding
  • Haemolysis
211
Q

define anaemia

A
  • haemoglobin below lower limit of normal
    • (sex adjusted Female normal 110-147g/l, male 131 – 166g/l)
212
Q

what other factors would you check in a pt with low Hb

A
  • platelet count
  • WBC level
213
Q

define MCV

A
  • Mean Corpuscular [cell] Volume
  • average cell size of RBCs = 80-98fl
214
Q

define MCH

A
  • Mean Cell Hb
  • amount of Hb in each cell
    • hypochromic, normochromic
215
Q

T/F

reticulocyte count is part of a routine FBC

A

False

has to be requested specially

216
Q

what is a reticulocyte count

A
  • count of the number of young RBCs
  • measures the rate of RBC production
217
Q

causes of macrocytosis

A

NOTE: not necessarily always caused by anaemias.

  • Folate deficiency
  • B12 deficiency
  • Reticulocytosis
  • Raised immunoglobulins
  • Hypothyroidism
  • Alcohol
  • Bone marrow failure, especially myelodysplastic neoplasms
  • Drugs, e.g. methotrexate,
218
Q

when is reticulocyte count a useful investigation

A
  • in pts with symptoms of anaemia with a normocytic MCV
    • increased of decreased reticulocyte count can help narrow down the cause of anaemia
219
Q

what are the 2 main classes of normocytic anaemia

AND

what causes them

A
  • normocytic anaemia with increased reticulocyte count - therefore there is bone marrow response
    • acute bleed
    • haemolytic anaemias e.g. sickle cell. G6pd deficiency.
  • normocytic anaemia with decreased reticulocyte count - thus there is bone marrow failure
    • aplastic anaemia - bone marrow disorder
    • chronic diseases - esp. CKD
220
Q

recommended iron/ day intake

amount of iron absorbed daily

function of iron

A
  • 15mg/ day
  • 1mg absorbed/day
  • used for Hb synthesis
221
Q

causes of iron deficiency

A
  • blood loss - GI, menstrual
  • pregnancy - a quarter of iron is transferred to foetus
  • impaired absorption - gastrectomy, coeliac disease
  • dietary deficiency - elderly and vegans
  • hookworm - blood loss @ site of attachment
222
Q

where is iron absorbed

A

SI: duodenum + jejunum

223
Q

T/F ferritin is a good diagnostic tool for iron deficiency anaemia

A

True BUT:

  • Ferritin is used to store iron in the RBCs, so the amount of ferritin in the blood gives an indication of how much iron in the blood.
  • BUT ferritin is an acute phase protein so could go up in an infection even if the pt is anaemic
    • so normal or high ferritin doesn’t rule out Fe-deficient anaemia
224
Q

what is transferrin and how is it used

A
  • A protein that binds iron.
  • transferrin saturation is used as a diagnostic tool: reduced saturation = Fe anaemia
    • transferrin synthesis is increased in Fe deficiency → reduced saturation as less is occupied by iron.
225
Q

T/F

serum iron is a useful investigative tool for Fe anaemia

A

FALSE

serum iron is useless as there are day to day fluctuations and circadian variation

226
Q

management of Fe deficient anaemia

A
  • stop bleeding if there is bleeding
  • iron replacement - oral is preferred
    • Hb should rise 20mg every 3-4 weeks
    • oral and IV are equivalent so no benefit to IV
    • IV requires hospitalisation + there’s risk of extravasation → permanent skin discolouration
  • once Hb and MCV levels are normal continue replacement for a further 3 months to ensure bone marrow stores are replenished
227
Q

what are the results of iron studies in Fe deficient anaemia

A
  • ↓ ferritin
  • ↓ transferrin saturation
  • ↑total iron binding capacity
  • ↓ serum iron [not reliable]
228
Q

where is B12 absorbed in the gut

A

the terminal ileum

229
Q

what is the first stem cell for blood cells that → further cells

A

multipotent haematopoietic stem cell

230
Q

what cells are produced from the multipotent haematopoietic stem cell

A
  • myeloid stem cells
  • lymphoid stemcells
231
Q

what cells are derived from myeloblast cells

A
  • monocyte → macrophage
  • eosinophil
  • neutrophil
  • basophil
232
Q

what cells are derived from common myeloid progenitor cells

A
  • myeloblast
  • erythroblast
  • megakaryocytic
  • mast cells
233
Q

what cell are platelets derived from

A

megakaryocyte

234
Q

what cells are derived from lymphoid progenitor cells

A
  • B cells
  • T cells
  • Natural killer cells
235
Q

what is a reticulocyte

AND

what is it derived from

A
  • an immature RBC
  • derived from erythroblasts
236
Q

what cells are antigen presenting cells and which is the ‘professional’

A
  • professional = dendritic cell
  • APCs
    • macrophages, B cells + dendritic cells present using MHC2
    • all epithelial cells can present using MHC1.
237
Q

what is the most common inherited bleeding disorder

A

Von Willerbrand Disease

238
Q

what are the types of von willerbrand disease AND which is the most severe

A
  • type 1,2 and 3
  • type 3 is the most severe
239
Q

what is the cause of Von Willerbrand disease

A
  • an autosomal dominant genetic disease that → deficient, absent or malfunctioning Von Willerbrand Factor.
    • VWF = a glycoprotein needed for formation of the platelet plug
240
Q

what is Von Willerbrand Factor

A

a glycoprotein needed for platelet adhesion for the formation of the platelet plug.

241
Q

how does VWD present

A
  • pts present with unusually easy, heavy or prolonged bleeding:
    • Bleeding gums with brushing
    • Nose bleeds (epistaxis)
    • Heavy menstrual bleeding (menorrhagia)
    • Heavy bleeding during surgical operations
    • severe post-natal bleeding
242
Q

how is VWD diagnosed

A
  • no single diagnostic test, a combo of:
    • abnormal bleeding
    • family hx
    • bleeding assessment tools such as PT, APTT, FBC
243
Q

how is VWD managed

A
  • not managed on a day to day basis only in preparation for an operation or in response to major bleeding.
    • Desmopressin can be used to stimulates the release of VWF
    • VWF can be infused
    • Factor VIII is often infused along with plasma-derived VWF
244
Q

what are the main causes of bleeding disorders

A
  • over anticoagulation
    • heparin, aspirin, clopidogrel, heparins, thrombolytics
  • genetic conditions
    • VWD
    • haemophilia: A +B
  • DIC: Disseminated Intravascular Coagulopathy
245
Q

what causes haemophilia

  • A
  • B
A
  • haemophilia is an X-linked recessive disorder - men only need 1 and women need 2 X Genes.
  • haemophilia A is caused by a deficiency in factor 8
  • haemophilia B is caused by a deficiency in factor 9
246
Q

who is mostly affected by haemophilia

A
  • men as it is an X-Linked recessive condition.
  • women are rarely affected as 2 X-genes are needed
247
Q

symptoms of haemophilia

A
  • excessive bleeding in response to minor trauma
  • spontaneous haemorrhage without any trauma
  • easy bruising
  • epistaxis
248
Q

investigations for diagnosis of haemophilia

A
  • APTT: prolonged
  • plasma factor 8 + 9: decreased or absent = diagnostic
249
Q

signs of haemophilia

A
  • haemarthrosis: bleeding in joints: knees, ankles + elbows especially.
  • Prolonged bleeding following heel prick: common presentation in neonates
  • Cutaneous purpura: often first presentation of acquired haemophilia
250
Q

symptoms of haemophilia

A
  • spontaneous bleeding into joints
  • excessive bleeding
  • easy bruising
  • fatigue
251
Q

management of haemophilia

A
  • IV infusion factor 8 or 9 +
  • desmopressin
    • desmopressin stimulates release of VWF
  • tranexamic acid = antifibrinolytic
    • reduces bleeding
252
Q

what is disseminated intravascular coagulation

A
  • an acquired syndrome → over-activation of the coagulation cascade → formation of intravascular thrombi + depletion of clotting factors and platelets.
253
Q

what can trigger DIC

A
  • severe infection
  • major trauma
  • malignancy
  • major vascular disorder
  • immunological reactions
254
Q

signs of DIC

A
255
Q

investigations for DIC

A
  • PT: prolonged
  • platelet count: decreased
  • fibrinogen: decreased
  • imaging for sites of thrombosis
256
Q

symptoms of bone marrow failure

A
  • Fatigue.
  • Shortness of breath.
  • Pale appearance.
  • Frequent infections.
  • Easy bruising or bleeding.
  • Bone pain
257
Q

symptoms of bone marrow failure

A
  • Fatigue.
  • Shortness of breath.
  • Pale appearance.
  • Frequent infections.
  • Easy bruising or bleeding.
  • Bone pain

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