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Phase 2 > Haematology > Flashcards

Haematology Flashcards

1
Q

What is lymphoma

A

Malignant proliferation of lymphocytes normally in the lymph nodes but also in the blood, spleen, liver and bone marrow

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2
Q

What are the 4 causes of lymphoma

A
  1. Primary immunodeficiency (Ataxia-teangiectasia, Wiscott-Aldrich syndrome)
  2. Secondary immunodeficiency (HIV, Transplant recipients)
  3. Infections (EBV, Helicobacter pylori, Human T-lymphocyte virus
  4. Autoimmune disorders
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3
Q

How is lymphoma diagnosed

A

Blood film and bone biopsy

Lymph node biopsy

Immunophenotyping

Cytogenetics (Karyotype, FISH, PCR)

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4
Q

How do you stage lymphoma

A

Blood tests

CT scan of chest, abdo/pelvis

Bone marrow biopsy

PET scan

Ann Arbor staging system

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5
Q

What are the two types of lymphoma

A

Hodgkins

Non-hodgkins lymphoma

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6
Q

What are the different types of Non-hodgkins lymphoma

A

Low grade = follicular lymphoma

High grade = Diffuse B cell lymphoma

Severe grade = Burkitt’s Lymphoma

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7
Q

What is Hodgkins lymphoma

A

Malignant transformation of normal B/T cells in the lymph nodes

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8
Q

Hodgkins lymphoma is most common in which age groups

A

20-29 years

>60 years

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9
Q

Hodgkins lymphoma is associated with what virus

A

EBV - impaired immunosurveillnance of infected cells

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10
Q

What are the lymphadenopathy symptoms of Hodgkins lymphoma

A

Painless, asymmetric lymph nodes that spread contiguously to adjacent lymph nodes

Cervical, inguinal and axillary lymph nodes

Alcohol makes more painful

Mediastinal lymphadenopathy causes cough, SVC obstruction and bronchial obstruction

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11
Q

What are the B cell associated symptoms of Hodgkins lymphoma

A

B cell symptoms
Fever
Night sweats
Wt loss

Other symptoms
Pruritis
Hepato/splenomegaly
Pel Ebstein Fever

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12
Q

What are the investigations for someone with suspected Hodgkins lymphoma

A
  1. Bloods (FBC, Film, ESR)
    - lactate dehydrogenase will be elevated
  2. Lymph node biopsy = REED-STEENBERG CELLS

3, Stage using CT/MRI chest, abdomen and pelvis

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13
Q

Describe the Ann Arbor system of staging lymphoma

A

Stage 1 = Single LN region
stage 2 = >2 nodal areas on same side of the diaphragm
Stage 3 = nodes spread on both sides of diaphragm
Stage 4 = Spread beyond nodes (Liver/bone marrow)

A = No constitutional B cell symptoms except itch

B = With constitutional B cell symptoms

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14
Q

What is the management for Hodgkins Lymphoma

A

Stage 1-2a = Short course chemo and radiotherapy

Stage 2a-4b = Cyclic chemo + radiotherapy

Bone marrow transplant for relapse

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15
Q

What chemotherapy drugs are used in Hodgkins lymphoma (remember ABVD)

A

Adriamycin
Bleomycin
Vinblastine
Decarbazine

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16
Q

What are the complications of Hodgkins lymphoma treatment

A 
Infertility 
Anthracylcines = Cardiomyopathy 
Bleomycin = lung damage 
Vinca Alkaloids= Peripheral neuropathy 
Second cancers 
Psychological issues
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17
Q

Define non-hodgkins lymphoma

A

Any lymphoma that doesn’t involve reed-steenberg cells

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18
Q

What age group does non-hodgkins lymphoma normally present in

A

Adults >40 years

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19
Q

Name a low grade non Hodgkins lymphoma and describe its characteristics

A 
Follicular lymphoma 
 - slow growing 
 - usually advanced at presentation 
- Incurable 
Median survival = 9-11 years
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20
Q

Name a high grade non Hodgkins lymphoma and describe its characteristics

A

Diffuse large B cell lymphoma

  • Usually nodal presentation
  • 1/3 cases have extra nodal involvement
  • patient often unwell with short history
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21
Q

Name a severe grade Hodgkins lymphoma

A

Burkitts lymphoma

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22
Q

What are the lymphadenopathy symptoms of non-hodgkins lymphoma

A

Painless, symmetric lymphadenopathy at multiple sites that spreads discontinuously

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23
Q

What are the extra nodal symptoms of non-hodgkins lymphoma

A

Skin
CNS
Oropharynx and GIT
Splenomegaly

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24
Q

What are the B symptoms of non-hodgkins lymphoma

A

Fever
Night sweats
Wt loss

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25
Q

What investigations would be conducted in someone with suspected non-hodgkins lymphoma

A

FBC, U and E and LDH
Film
- Normal or circulating lymphoma cells
Pancytopenias with NO REED STEENBERG CELLS

Staging using CT/MRI chest, abdomen and pelvis and Ann Arbor classification

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26
Q

Describe the management of high grade non-hodgkins lymphoma

A 
R-CHOP regime 
Rituximab (Monoclonal antibody)
Cyclophosphamide 
Hydro-doxorubicin
Vincristine 
Prednisolone

Bone marrow transplant for relapse

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27
Q

What does rituximab target

A

CD20 on B cells

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28
Q

What is the management for low grade non-hodgkins lymphoma

A 
Watch and wait is asymptomatic 
if symptomatic = 
Chemotherapy 
Radiotherapy 
Alkylating agents 
Monoclonal antibodies 
Bone marrow transplant
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29
Q

What is T cell engaging therapy

A

Blinatumomab

- Bispecific antibody that targets CD19 n B cells and CD3 on T cells

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30
Q

Describe the pathophysiology of lymphoma associated with EBV

A

There is impaired immunosurveillance and infected B cells escape regulation and proliferate

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31
Q

What is myeloma

A

Malignant disease of bone marrow plasma cells leading to progressive bone marrow infiltration and failure

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32
Q

Describe the epidemeology of myeloma

A

Peak age = 60-70yrs

Afro-caribbean

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33
Q

What is the pathogenesis of myeloma

A

Clonal proliferation of plasma cells –> Monoclonal Ig production (usually IgG or IgA)

Clones produce light chains which are excreted in the kidney (Bence jones protein)

Clones produce IL-6 which inhibits osteoblasts and activates osteoclasts

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34
Q

What are the symptoms of myeloma (Remember CRAB)

A
  1. Calcium elevated (Increased osteoclast activity leading to hypercalcaemia)
  2. Renal impairment (Caused by light chain deposition and increased calcium
  3. Anaemia (Decreased bone marrow RBC production leading to normochromic normocytic anaemia)
  4. Bone lesions (Increased osteoclast activity leading to lytic lesions = bone pain esp back

Recurrent infections due to neutropenia and immunoparesis

Hypercalcaemia and bone pain = pepper pot skull

Bone marrow infiltration = anaemia, thrombocytopenia and neutropenia

Amyloidosis

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35
Q

What investigations would be conducted in someone with suspected myeloma

A

FBC = normocytic normochromic anaemia

Film = rouleaux formation, plasma cells and cytopenias

Increased ESR, increased calcium

Urinalysis and electrophoresis show Bence Jones protein

PLAIN X-RAY = LYTIC PUNCHED OUT LESIONS (PEPPER POT SKULL AND OESTEOPOROSIS)

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36
Q

What is the management of myeloma

A
  1. Supportive
    - Bone pain (Analgesics but not NSAIDS
    - Bisphosphates (Zolendronate to decrease fractures and bone pain)
    - Transfusions for anaemia
    - Hydration 3L/day for renal impairment
    - Broad spectrum antibiotics for infections
  2. Chemotherapy
    (i) Cyclophosphamide, thalidomide, dexamethasone)
    (ii) Vincristine, adriamycin, dexamethasone)
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37
Q

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

A

Abnormal proliferation of plasma cells leading to immunoglobulin secretion and causing organ dysfunction especially to the kidney

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38
Q

What disease often precedes myeloma

A

Monoclonal gammopathy of undetermined significance (MGUS)

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39
Q

What is MGUS

A

A common disease with paraprotein present in the serum but no myeloma

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40
Q

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

A

Immunoglobulin light chains with kappa or lamda lineage

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41
Q

What would you expect to seen on the blood film of someone with myeloma

A

Rouleaux formation (Aggregation of RBCs)

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42
Q

What is the definition of leukaemia

A

Malignant proliferation of haemopoietic cells

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43
Q

What are the 4 types of leukaemia

A
  1. Acute myeloid leukaemia
  2. Acute lymphoblastic leukaemia
  3. Chronic myeloid leukaemia
  4. Chronic lymphocytic leukaemia
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44
Q

What are the risk factors for leukaemia

A 
Congenital (Downs syndrome)
Environmental 
 - Radiotherapy 
-Chemotherapy 
 - Benzene
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45
Q

What investigations would you do in someone with suspected leukaemia

A 
Blood film 
Bone marrow biopsy 
Lymph node biopsy 
Immunotyping (Classifying tumour cells by antigen expression profile)
Cytogenetics (Karyotyping, FISH, PCR)
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46
Q

What is the definition of acute lymphoblastic leukaemia

A

Acute malignant transformation of lymphoid progenitor cells

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47
Q

What age group is acute lymphoblastic leukaemia most common in

A

Most common cancer in children especially 2-5 years

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48
Q

What are the symptoms of ALL

A

Bone marrow failure leading to anaemia, thrombocytopenia and leukopenia

Infiltration of tissue (Lymphadenopathy, CNS involvement, hepatosplenomegaly, bone pain)

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49
Q

What investigations would be conducted in someone with suspected ALL

A 
Increased WCC
Decreased RBC + Platelets 
BM aspirate = 20% leukaemia blast cells 
Cytogenetics = philadelphia chromosome
CT and CXR for lymph node involvement
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50
Q

What is the management of ALL

A
  1. Supportive (Blood products, antifungals, antibiotics)
  2. Chemotherapy (Vincristine, dexamethasone and intrathecal methotrexate)
  3. Bone marrow transplant best for younger patients
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51
Q

Define acute myeloid leukaemia

A

Acute malignant transformation of myeloid progenitor cells

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52
Q

What age range is AML most common in

A

Mean age 65-70

but also 10-15% of childhood leukaemia

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53
Q

What is the aetiology of AML

A

Neoplastic proliferate of myeloblasts
Preceding haematological disorder or prior chemotherapy
Exposure to ionising radiation

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54
Q

What are the signs and symptoms of AML

A
  1. Bone marrow failure
    - anaemia
    - infection (decreased WCC)
    - DIC
  2. Infiltration
    - Gum infiltration
    - Hepatosplenomegaly
    - Skin involvement
    - Bone pain
    - Lymphadenopathy
    - Orchidomegaly
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55
Q

What investigations for AML

A

Blood film = anaemia and thrombocytopenia but increased WCC

Bone marrow biopsy (>20% blast cells)
Auer rods are diagnostic of AML over ALL)

Lymph node biopsy

Immunophenotyping

Cytogenetics

BM aspirate = leukaemia blast cells

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56
Q

What is the management of AML

A

Supportive care as for ALL
Chemotherapy
Bone marrow transplant

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57
Q

Define chronic myeloid leukaemia

A

Chronic malignant transformation of myeloid cells (Basophils, eosinophils, neutrophils)

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58
Q

What age group is CML most common in

A

Middle aged 40-60 years

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59
Q

What is the cause of chronic myeloid leukaemia

A

Myeloproliferative disorder with clonal proliferation of myeloid cells

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60
Q

What are the symptoms of CML

A

wt loss, fever, night sweats
Massive hepatosplenomegaly
Bruising and bleeding

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61
Q

What is the key diagnostic feature of CML

A

Philadelphia chromosome
Reciprical translocation t(9,22) leading to formation of BCR-ABL fusion gene leading constitutive tyrosine kinase activity = increase cell growth

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62
Q

What investigations would you conduct in someone with suspected CML

A

Increased WBC
Decreased Hb and platelets
BM cryogenic analysis is Philadelphia +ve

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63
Q

What is the treatment of CML

A

Imatinib = tyrosine kinase inhibitor

Allogenic Stem cell transplant

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64
Q

Define chronic lymphocytic lymphoma

A

Chronic malignant transformation of mature lymphoid cells

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65
Q

What is the aetiology of CLL

A

Clone of mature B cells

Mutation of 11q or 17p

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66
Q

What are the features of CLL

A

Symmetrical painless lymphadenopathy
Hepatosplenomegaly
Anaemia, infection, bleeding, BM failure
Wt loss, fever, night sweats

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67
Q

What are the investigations in someone with CLL

A

Increased WCC
Anaemia
Immunophenotyping to distinguish it from NHL

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68
Q

What is the management of CLL

A

Supportive
chemotherapy (Cyclophosphamide, fludarabine, rituximab)
Radiotherapy

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69
Q

Define anaemia

A

Low Hb concentration due to a low red cell mass or increased plasma volume

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70
Q

How can you determine if bone marrow production of RBC’s is the issue in anaemia

A

Look at reticulocyte number (Immature RBC’s)

  • If high then removal the issue
  • If low then production the issue
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71
Q

What is the normal haemoglobin concentration for men and women

A

Male = 133-166g/L

Female = 110-147g/L

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72
Q

What are the physiological changes seen in anaemia

A

Reduced HB = reduced O2 transport = hypoxia = compensatory mechanisms such as increased tissue perfusion, increased O2 transfer to tissues and increased RBC production

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73
Q

What are the pathological changes seen in anaemia

A

Heart and liver fat change
Ischaemia
Skin and Nail atrophy
Aggravate angina

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74
Q

When might you see microcytic hypo chromic RBCs on blood film

A

Iron deficiency

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75
Q

When might you see polysegmented neutrophils with microcytic anaemia

A

B12 deficiency

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76
Q

What are the non-specific symptoms of anaemia

A 
Fatigue
Dyspnoea 
Headaches 
Faintness 
Anorexia 
Palpitations
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77
Q

What are the signs of anaemia

A

Conjunctival pallor
Hyperdynamic circulation
Palpitation
Tachycardia

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78
Q

What organs are responsible for the removal of RBC’s

A
  1. Spleen
  2. Liver
  3. Bone marrow
  4. Blood loss
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79
Q

How do we classify the different types of anaemia

A

By mean corpuscular volume which is the average volume of the RBC’s (Their size)

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80
Q

What are the normal ranges for MCV for males and females

A 
Male = 81.8 - 96.3fl 
Females = 80.0- 98.1fl
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81
Q

What are the three different classifications of anaemia

A

Microcytic
Normocytic
Macrocytic

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82
Q

What are the causes of microcytic anaemia

A 
Iron deficiency 
Chronic disease (BM/kidney problems)
Thalassaemia 
Sideroblastic anaemia 
Lead poisoning
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83
Q

What are the causes of normocytic anaemia

A 
Acute blood loss 
Chronic disease 
Combined haematinic deficiency (Iron and B12 deficiency)
BM failure 
Renal failure 
Pregnancy
Hypothyroidism
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84
Q

What are the causes of macrocytic anaemia

A 
B12 deficiency 
Folate deficiency 
Anti-folate drugs (Methotrexate and Phenytoin)
Excess alcohol or liver disease
Hypothyroidism 
Cytotoxics (Hydroxycarbamide)
Reticulocytosis
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85
Q

What is the normal daily intake of iron

A

15-20mg

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86
Q

What are the specific signs of iron deficiency anaemia

A

Koilonychia (spoon shaped nails)

Angular stomatitis (Mouth corners inflamed)

Atrophic glossitis

Plummer Vinson

Brittle Nails and Hair

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87
Q

Describe the pathophysiology of iron deficiency anaemia

A

Lack of iron means no haem production leading to smaller RBCs and microcytic anaemia

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88
Q

What are the causes of iron deficiency anaemia

A

Blood loss

  • Menorrhagia
  • Hookworm
  • GI bleeding

Increased demand
- pregnancy

Decreased intake
- diet

Malabsorption

  • Coeliac
  • Crohns
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89
Q

What are the investigations for someone with suspected iron deficiency anaemia

A

Blood film = Microcytic hypochromic anaemia, low reticulocyte

Low ferritin 
Low Fe
Low Hb 
Low MCV 
Increased TIBC 
Ansiocytosis, poikliocytosis, pencil cells
Upper and lower GI endoscopy
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90
Q

How do you manage someone with iron deficiency anaemia

A

Oral iron = ferrous sulphate

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91
Q

What are the side effects of ferrous sulphate

A 
Nausea 
Abdominal discomfort 
Diarrhoea 
Constipation 
Black stools
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92
Q

What is the pathophysiology of B12 deficiency

A

Lack of B12/intrinsic factor means B12 is not absorbed in the terminal ileum leading to big fragile RBC due to impairment of DNA synthesis

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93
Q

What are the causes of vitamin B12 deficiency

A

Decreased intake
- vegan

Decreased intrinsic factor

  • Pernicious anaemia
  • Post-gastrectomy

Terminal ileum

  • Crohn’s
  • Illeal resection
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94
Q

What are the specific signs of B12 deficiency

A 
Glossitis 
Paraesthesia 
Optic Atrophy
Peripheral neuropathy
Symptoms of anaemia 
Lemon tinge due to pallor and mild jaundice
Subacute Cord Degeneration
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95
Q

What are the investigations for someone with suspected B12 deficiency

A 
Decreased WCC
Intrinsic factor antibodies 
Parietal cell antibodies 
Coeliac Abs 
Schilling test
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4
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96
Q

What is the management for someone with B12 deficiency

A

B12 replacement with hydroxocobalamin

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97
Q

What is pernicious anaemia

A

Autoimmune atrophic gastritis causes by auto antibodies against parietal cells or intrinsic factor

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98
Q

What are the causes of folate deficiency

A

Decreased intake
- Poor diet

Increased demand

  • Pregnancy
  • Malignancy

Malabsorption

  • Coeliac
  • Crohn’s

Drugs

  • EtOH
  • phenytoin
  • Methotrexate
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99
Q

What are haemoglobinopathies

A

Disorders of quality = abnormal molecule or variant haemoglobin ie. sickle cell disease

Disorders of quantity = reduced production ie. alpha or beta thalasaaemia

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100
Q

What is the structures of normal haemoglobin

A

2 alpha and 2 beta subunits

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101
Q

What is the structure of foetal haemoglobin

A

2 alpha and 2 gamme subunits

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102
Q

What is the inheritance pattern in sickle cell anaemia

A

Autosomal recessive

103
Q

Where is sickle cell disease most prevalent

A

Africa, caribbean and ME

104
Q

What is the pathogenesis of sickle cell disease

A

Point mutation in the B-globin gene causing glutamic acid to valine AA change which causes HbA to be changed to HbS

105
Q

What is the HbS globin chain problematic

A

HbS is insoluble when deoxygenated = sickling

Sickle cells have decreased life span = haemolysis

Sickle cells get trapped in microvascular = thrombosis

106
Q

When do the clinical features of sickle cell disease begin to show

A

Manifest from 3-6 months due to decreased foetal Hb

107
Q

What can be a trigger for the manifestation of sickle cell disease

A

Infection
Cold
Hypoxia
Dehydration

108
Q

What is the presentation of sickle cell anaemia

Remember sickled

A 
Splenomegaly 
Infarction 
Crises (pulmonary)
Kidney disease 
liver and lung disease 
Erection 
Dactylitis
109
Q

What is thalassaemia

A

Point mutations or deletions leading to diminished synthesis of one or more globin chains leading to unmatched haemoglobin molecules which damages the RBC membrane causing their destruction

110
Q

Where is thalassaemia most common

A

Mediterranean

Far East

111
Q

What is the classification of beta thalassaemia

A
  1. Thalassaemia major
    - Transfusion dependent
  2. Thalassaemia intermedia
    - Less severe anaemia but can survive without regular blood transfusions
  3. Thalassaemia carrier/heterozygote
    - Asymptomatic
112
Q

What is the clinical presentation of thalassaemia major

A

Age of presentation at 6-12 months
Clinical presentation is severe anaemia symptoms with failure to thrive, jaundice and extra medullary erythropoiesis including frontal bossing, maxillary overgrowth and HSM

113
Q

What is the treatment of thalassaemia major

A 
Regular transfusion 
Iron chelation 
Endocrine supplementation 
Bone health 
Psychological support
114
Q

What do you need to monitor in thalassaemia major

A 
Ferritin 
Cardiac and liver MRI
Endocrine testing 
Gonadal function 
Diabetes screening 
Growth and puberty 
Vit D, Calcium and PTH
Thyroid 
Dexa Screening
115
Q

What are the complications of blood transfusions for thalassaemia

A

Iron overload as there is not way to eliminate the excessive iron leading to developing haemosiderosis

116
Q

Describe the inheritance pattern of membranopathies

A

Autosomal dominant

117
Q

Name 2 common membranopathies

A

Spherocytosis

Elliptocytosis

118
Q

Describe the physiology of membranopaties

A

Deficiency of red cell protein (Spectrin) caused by a variety of genetic lesions which causes RBC to become spherical and rigid so gets stuck in the spleen leading to extravascular haemolysis

119
Q

What do membranopathies predispose you to?

A

Gall stones

120
Q

What is the treatment for membranopathies

A

Folic acid and splenectomy

121
Q

What is the significance of parvovirus and haemolytic anaemia

A

Parvovirus is a common infection in children and can lead to a dramatic drop in Hb in patients who already have a reduced cell lifespan

122
Q

What are enzymopathies

A

Inherited enzyme deficiencies that lead to shortened red cell lifespan from oxidative kinase

123
Q

Name 2 common enzymopathies

A

G6PD deficiency

Pyruvate kinase deficiency

124
Q

Where is G6PDD most common

A

Mediterranean and Mid/Far East

125
Q

How do you diagnose G6PD

A

Screening for NADPH
G6PD assay with reticulocytes showing increased G6PD

Look on blood fillm for Heinz bodies and bite cells

126
Q

What are the symptoms of G6PD

A

Mainly asymptomatic but crises are characterised by
haemolysis
Jaundice
Anaemia

127
Q

What things might precipitate G6PD

A 
Broad beans 
Infection 
Drugs 
 - Primaquine 
Sulphonamides 
Nitrofurantoin
Quinolones 
Dapsone
128
Q

Define polycythaemia

A

Too many RBCs

129
Q

What are the two types of polycythaemia

A

Primary = polycythaemia rubra vera

Secondary = Smoking, Lung/heart disease, excess EPO, altitude

130
Q

Describe the pathophysiology of primary polycythaemia

A

Bone marrow overactivity leading to increased RBC, WBC and Plt caused by JACK2 mutation

131
Q

What are the signs of polycythaemia

A

Hyperviscosity = headaches, dizziness, visual disturbances, thrombosis

Pruritis after hot bath (Aquagenic pruritus)

Erythromelagia

Signs - red face and hepatosplenomegaly

132
Q

What investigations for polycythaemia

A

FBC - High RBC, WCC and Plt
BM is hyper cellular and erythroid
JAK2 is 99% +ve

133
Q

What is the management of polycythaemia

A

Venesection in young patients or hydroxycarbamide (BM suppression in older patients)

Aspirin

134
Q

What is the complication of polycythaemia

A

Can progress to acute myeloid leukaemia

135
Q

Where are platelets produced and what are they produced from

A

In the bone marrow from megakaryocyte fragments

136
Q

Which hormone regulates platelet production

A

Thrombopoietin

137
Q

What is the lifespan of platelets

A

7-10 days

138
Q

What is the normal platelet count

A

150-400x10^9/L

139
Q

How are platelets removed from the circulation

A

By the spleen

140
Q

What surface proteins are found on a platelet

A

ABO
HPA
HLA class I
Glycoproteins

141
Q

What causes a platelet to become activated

A

Adhesion to collagen via GPIa

Adhesions to VWF by GPIb and IIb/IIIa

142
Q

What does platelet activation lead to

A

Release of alpha granules containing PDGF, Fibrinogen, VWF and PF4

Release of dense granules containing nucleotides (ADP), Ca2+ and serotonin

Membrane phospholipids acitivate clotting factors II, V and X

Change in shape to spiculated shape

143
Q

Describe the process of platelet activation in primary haemostasis

A

1) Platelets adhere to vascular endothelium via collagen & vWF (von Willebrand factor)

2) Binding of platelets to collagen stimulates cytoskeleton shape change within the
platelets, and they ‘spread’ out

3) This increases their surface area and results in their activation, leading to the release of platelet granule contents including ADP, fibrinogen, thrombin and calcium. These components facilitate the clotting cascade ending with the production of fibrin.
4) Aggregation of platelets then occurs, which involves the cross-linking of activated platelets by fibrin
5) Activated platelets also provide a negatively charged phospholipid surface, which allows coagulation factors to bind and enhance the clotting cascade.

144
Q

What tests can you carry out to determine platelet function

A

FBC for number

Blood film for appearance

Platelet function analysis for function

FLow cytometry for surface proteins

145
Q

What problems can cause bleeding

A
  1. Injury/trauma
  2. Vascular disorders (Abnormality of collagen = Ehlers Danlos Syndrome)
  3. Low platelets (Thrombocytopenia)
  4. Abnormal platelet function
  5. Defective coagulation (Haemophilia)
146
Q

What clinical features would you seen in someone with platelet dysfunction

A 
Mucosal bleeding 
 - Epistaxis 
 - Gum bleeding
 - Menorrhagia
Easy bruising 
Petechiae 
Purpura 
Traumatic haemorrhage (Inc subdural)
147
Q

What are the causes of low platelets

A
  1. Production failure
    - Congenital (Thrombocytopenia)
    - Acquired (Drugs, BM suppression, Marrow failure, BM replacement)
  2. Increased removal (immune, consumption (Bleeding), Splenomegaly)
  3. Artefactual (EDTA induced clumping)
148
Q

What are the causes of impaired platelet function

A
  1. Congenital (Platelet storage disorders =
    - Glanzmann (GpIIb/IIIa deficiency)
    - Bernard Soulier (GPIb deficiency)
    - Von Willebrand Disease (Lack of VWF which binds platelets to damaged endothelium)
  2. Acquired
    - uraemia
    - Drugs (Aspirin, clopidogrel, NSAIDs)
149
Q

Glanzmann diseases is a deficiency in what

A

GPIIb/IIIa fibrinogen receptor

150
Q

Bernard soupier disease is a deficiency in what

A

Reduction in GP1b/ VW receptor

151
Q

What are the two main causes of thrombocytopenia

A
  1. Decreased platelet production

2. Increased platelet destruction

152
Q

What are the causes of decreased platelet production

A
  1. Congenital thrombocytopenia
  2. Infiltration of BM
    - Leukaemia
    - Metastatic malignancy
    - Lymphoma
    - Myeloma
  3. Reduced platelet production by BM
    - Low B12
    - Reduced TPO
    - Medication (Methotrexate, chemo
    - Toxins
    - Infection (HIV, TB)
  4. Dysfunctional production of platelets in BM
    - Myelodysplasia
153
Q

What are the causes of increased platelet destruction

A
  1. Autoimmune thrombocytopenia
  2. Hypersplenism
  3. Drug related immune destruction (Heparin)
  4. Consumption of platelets
    - DIC
    - Thrombotic thrombocytopenc purport
    - Haemolytic uraemia syndrome
    - Haemolysis
    - Major haemorrhage
154
Q

How does clopidogrel affect platelet functions

A

Binds P2Y12 inhibitor

155
Q

How does aspirin affect platelet function

A

Irreversible inhibitor of COX1

156
Q

How does Tirofiban affect platelet function

A

Inhibits GP IIb/IIIa

157
Q

What is immune thrombocytopenia

A

IgG antibodies form to platelet and megakaryocyte surface glycoproteins

158
Q

What is the treatment for immune thrombocytopenia

A

Immunosuppressants
Treat underlying cause
Tranexamic acid (inhibits fibrin breakdown)

159
Q

Describe the pathophysiology of disseminated intravascular coagulation

A

Cytokine release in response to systemic inflammatory response syndrome leads to systemic activation of the clotting cascade which…

  1. Leads to microvascular thrombosis and organ failure
  2. Consumption of platelets and clotting factors leading to bleeding
160
Q

What is the treatment for DIC

A

Treat underlying cause
Supportive provision of
- platelets
- FFP

161
Q

What is thrombotic thrombocytopenia purpura

A

Spontaneous platelet aggregation in microvasculature

162
Q

What is the aetiology of thrombocytopenia purpura

A

Reduction in protease enzyme ADAMTS13 which leads to failure to break down high molecular weight VWF multimers leading to massive blood clots capable of causing infarcts

163
Q
  1. What is the characteristic genetic abnormality in chronic myeloid leukaemia?
    (i) ARTA Gene
    (ii) Philadelphia chromosome
    (iii) AML/ETO gene
    (iv) cMYC oncogene
A

Philadelphia chromosome

164
Q
  1. What class of drug best describes rituximab?
    (i) Cytotoxic chemotherapy
    (ii) Disease modifying therapy
    (iii) Monoclonal antibody
    (iv) Antibiotic
A

Monoclonal Antibody

165
Q
  1. Which age group is characteristically affected by Hodgkins lymphoma?
    (i) Children
    (ii) Teenagers and young adults
    (iii) Middle aged (40-60 years)
    (iv) Older age (>60 years)
A

Teenagers and young adults

Older age >60 years

166
Q
  1. How is myeloma bone disease usually assessed?
    (i) Plain X-ray
    (ii) Clinical assessment
    (iii) Isotope bone scan
    (iv) PET scan
A

Plain X-ray

167
Q
  1. What is the correct mechanism of action for anti-emtic drug ondanseteron
    (i) Peripheral D2 antagonist
    (ii) Central D2 antagonist
    (iii) Anti-cholinergic
    (iv) 5-HT3 antagonist
A

5-HT3 antagonist

168
Q
  1. What is the commonest cause of microcytic anaemia?
    (i) B12 deficiency
    (ii) Iron deficiency
    (iii) Haematologic malignancy
    (iv) Hereditary spherocytosis
A

Iron deficiency

169
Q
  1. In sickle cell anaemia what would you expect to see the reticulocyte count?
    (i) Absent
    (ii) Low
    (iii) Normal
    (iv) Raised
A

Raised

170
Q
  1. Bacterial infections usually causes?
    (i) Low lymphocytes
    (ii) Low neutrophils
    (iii) High lymphocytes
    (iv) High neutrophils
A

High neutrophils

171
Q
  1. Which best outlines the approach to the management of a patient with suspected febrile neutropenia
    (i) Encourage fluids and paracetamol
    (ii) Perform cultures and wait for results before antibiotics
    (iii) Perform cultures and start oral antibiotics
    (iv) Perform culrures and start broad spectrum IV antibiotics
    - Use tanzosin and gentamicin
A

(iv) Perform culrures and start broad spectrum IV antibiotics
- Use tanzosin and gentamicin

172
Q
  1. How does aspirin exert is antiplatelet effect?
    (i) ADP receptor antagonist
    (ii) Inhibition of cyclooxygenase
    (iii) Inhibition of glycoprotein IIb-IIIa
    (iv) Inhibition of PAR4 receptor
A

Inhibition of cycle-oxygenase

173
Q

What is polycythaemia

A

Too many red blood cells

174
Q

What are the primary causes of polycythaemia

A

Polycythaemia rubra vera - over-reactive bone marrow

175
Q

What are the reactive/secondary causes of polycythaemia

A 
Smoking 
Lung disease 
Cyanotic heart disease 
Altitude 
EPO and androgen excess
176
Q

What is polycythaemia rubra vera

A

Myeloproliferative disorder resulting in bone marrow overactivity resulting in predominantly increased WBC’s and platelets

177
Q

What mutation causes polycythaemia rubra vera in 95% of cases

A

Mutation in JAK2

178
Q

What is the clinical presentation of polycythaemia rubra vera

A 
Plethoric appearance 
Thrombosis 
Itching 
Splenomegaly 
Abnormal FBC
179
Q

What is the treatment for polycythaemia rubra vera

A

Aspirin
Venesection
Bone marrow suppressive drugs (Hydroxycarbamide)
If secondary then treat the underlying cause

180
Q

Define neutrophilia

A

Too many neutrophils

181
Q

What are the primary causes of neutrophilia

A

Chronic myeloid leukaemia

182
Q

What are the reactive causes of neutrophilia

A

Bacterial Infection
Malignancy
Inflammation

183
Q

What is lymphocytosis

A

Too many white blood cells mainly lymphocytes

184
Q

What are the primary causes of lymphocytosis

A

Chronic lymphoid leukaemia

185
Q

What are the reactive causes of lymphocytosis

A

Viral Infection
Inflammation
Malignancy

186
Q

Define thrombocytopenia

A

Not enough platelets

187
Q

Define thrombocytosis

A

Too many platelets

188
Q

What are the primary causes of thrombocytosis

A

Essential thrombocythaemia

189
Q

What are the reactive causes of thromocytosis

A

Infection
Inflammation
Malignancy

190
Q

Define Neutropaenia

A

not enough neutrophils

191
Q

Define normal neutrophil levels

A

1.7-6.5

192
Q

Define mild neutropaenia

A

1-1.7

193
Q

Define moderate neutropaeia

A

0.5-1.0

194
Q

Define a severe neutropenia

A

<0.5

195
Q

What are the causes of neutropenia

A
  1. Underproduction
    - marrow failure
    - marrow infiltration
    - Marrow toxicity ie drugs
  2. Increased removal
    - Autoimmune
    - Felty’s syndrome
    - cyclical
196
Q

What hormone is responsible for regulating RBC production?

A

Erythropoietin.

197
Q

What stimulates EPO?

A

Tissue hypoxia.

198
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.
199
Q

Where are platelets produced?

A

In the bone marrow. They are fragments of megakaryocytes.

200
Q

What is the definition of febrile neutropenia.

A

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

201
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.
202
Q

What is the presentation of febrile neutropenia?

A
  1. Pyrexia, 38°C.
  2. Generally unwell.
  3. Confusion.
  4. Hypotensive.
  5. Tachycardic.
203
Q

Describe the management of febrile neutropenia.

A
  1. Thorough history and examination.
  2. Bloods.
  3. Antibiotics within 1 hour!
204
Q

Give a risk factor for spinal cord compression.

A

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

205
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.
206
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.
207
Q

What is tumour lysis syndrome?

A

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

208
Q

Give 3 risk factors for tumour lysis syndrome.

A
  1. High tumour burden.
  2. Pre-existing renal failure.
  3. Increasing age.
209
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.
210
Q

What is hyperviscosity syndrome?

A

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

211
Q

Give 2 consequences of hyperviscosity syndrome

A
  1. Vascular stasis.

2. Hypoperfusion.

212
Q

Describe the presentation of hyperviscosity syndrome.

A
  1. Mucosal bleeding.
  2. Visual change.
  3. Neurological disturbances.
  4. Breathlessness.
  5. Fatigue.
213
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.
214
Q

What is the treatment for hyperviscosity syndrome?

A
  1. Keep hydrated!
  2. Avoid blood transfusion.
  3. Treat the underlying cause.
215
Q

What does rituximab target?

A

Targets CD20 on the surface of B.

216
Q

What is the characteristic genetic abnormality in chronic myeloid leukaemia?

A

t(9; 22) - philadelphia chromosome.

217
Q

How is myeloma bone disease usually assessed?

A

X-ray.

218
Q

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

A

Reticulocyte count is raised.

219
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.

220
Q

What clotting factors depend on vitamin K?

A

2, 7, 9 and 10.

221
Q

Haemophilia A is due to deficiency of what clotting factor?

A

Factor 8 deficiency

222
Q

Haemophilia B is due to deficiency of what clotting factor?

A

Factor 9 deficiency.

223
Q

What kind of anaemia could methotrexate cause?

A

Macrocytic due to folate deficiency.

224
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.
225
Q

Give 3 signs of haemolytic anaemia.

A
  1. Pallor.
  2. Jaundice.
  3. Splenomegaly.
226
Q

Give 3 signs of haemolytic anaemia.

A
  1. Pallor.
  2. Jaundice.
  3. Splenomegaly.
227
Q

Give 4 causes of haemolytic anaemia.

A
  1. GP6D deficiency.
  2. Sickle cell anaemia.
  3. Spherocytosis/elliptocytosis (membranopathies).
  4. Autoimmune haemolytic anaemia.
228
Q

Give 3 things that can cause coagulation disorders.

A
  1. Vitamin K deficiency.
  2. Liver disease.
  3. Congenital e.g. haemophilia.
229
Q

How does warfarin work?

A

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

230
Q

How does heparin work?

A

It activates antithrombin which then inhibits thrombin and factor Xa.

231
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.

232
Q

Give 3 causes of disseminated intravascular coagulation (DIC).

A
  1. Sepsis.
  2. Major trauma.
  3. Malignancy.
233
Q

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

A

All increased

234
Q

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

A

Decreased

235
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.
236
Q

Give 3 symptoms of DVT.

A

Unilateral warm, tender, painful, swollen leg.

237
Q

What forms the differential diagnosis for a DVT?

A

Cellulitis.

238
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.
239
Q

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

A

The Wells score.

240
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.
241
Q

Describe the management for a DVT.

A

Aim of management is to prevent a PE!

- Anticoagulants e.g. warfarin/heparin.

242
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.

243
Q

Where would you normally take a bone marrow biopsy from?

A

Posterior iliac crest.

244
Q

What is the most important medical treatment for DVT prophylaxis?

A

LMWH.

245
Q

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

A

Iron binding capacity will be raised.

246
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.

247
Q

Describe the treatment for iron deficiency anaemia.

A

Ferrous sulphate tablets.

248
Q

What is aplastic anaemia?

A

When bone marrow stem cells are damaged -> pancytopenia.

249
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.

250
Q

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

A

Hydroxycarbamide.

251
Q

Give 3 clinical features of a patient with a membranopathy.

A
  1. Jaundice.
  2. Anaemia.
  3. Splenomegaly.
252
Q

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

A

G6PD protects cells against oxidative damage.

253
Q

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

A
  1. Hb.
  2. RCC.
  3. PCV.
254
Q

Give 3 symptoms of polycythaemia.

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

Phase 2 (16 decks)

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