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medicine phase 2 a > Haematology > Flashcards

Haematology Flashcards

1
Q

What are the key presentations of iron deficiency anaemia?

A

fatigue
pallor
weakness

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2
Q

What are the causes of microcytic anaemia?
(TAILS)

A

Thalassaemia
Anaemia of chronic diseasae
Iron deficiency anaemia
Lead poisoning
Sideroblastic anaemia

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3
Q

What can cause normocytic anaemia? (3A’s 2H’s)

A

acute blood loss
anaemia of chronic disease
aplastic anaemia
haemolytic anaemia
hypothyroidism

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4
Q

What is megaloblastic anaemia?

A

impaired DNA synthesis, cell grows larger instead of dividing

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5
Q

What causes megaloblastic anaemia?

A

B12 or folate deficiency

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6
Q

What can cause normoblastic macrocytic anaemia?

A

alcohol
reticulocytosis
hypothyroidism
liver disease
drugs e.g. azathioprine

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7
Q

What are the general symptoms of iron deficiency anaemia?

A

tiredness
shortness of breath
headaches
dizziness
palpitations

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8
Q

What are the specific symptoms of iron deficiency anaemia?

A

pica
hair loss

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9
Q

What are the specific signs of iron deficiency?

A

koilonychia (spoon shaped nails)
angular cheilitis
atrophic glossitis
brittle hair and nails

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10
Q

What are the 1st line investigations for iron deficiency anaemia?

A

serum ferritin
FBCs - reduced Hb, MCV
B12
folate

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11
Q

What other investigations might you do for iron deficiency anaemia?

A

FIT test
bone marrow biopsy
colonoscopy, GI investigations (cancer)

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12
Q

What is the 1st line management for iron deficiency anaemia?

A

oral iron replacement
ferrous sulphate

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13
Q

What is the 2nd line treatment for iron deficiency anaemia?

A

IV iron replacement

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14
Q

What is haemolytic anaemia?

A

a number of conditions that result in the premature destruction of RBCs

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15
Q

What are the key presentations of haemolytic anaemia?

A

pallor
jaundice
fatigue

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16
Q

What are the signs of haemolytic anaeamia?

A

splenomegaly
episodic dark urine (haemoglobinuria)

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17
Q

What are the symptoms of haemolytic anaemia?

A

SOB
dizziness
triggered by exposure to cold

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18
Q

What are the inherited haemolytic anaemias?

A

hereditary spherocytosis
hereditary elliptocytosis
thalassaemia
sickle cell anaemia
G6PD deficiency

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19
Q

What are the acquired haemolytic anaemias?

A

autoimmune and alloimmune anaemia (transfusion reactions and disease of newborn)
paroxysmal nocturnal haemoglobinuria
microangiopathic haemolytic anaemia
prosthetic valve related haemolysis

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20
Q

what is hereditary spherocytosis?

A

autosomal dominant conditions, RBCs are spheres and easily broken down when passing through spleen

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21
Q

How do you treat hereditary spherocytosis?

A

folate supplementation and splenectomy

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22
Q

How does hereditary elliptocytosis differ from spherocytosis?

A

RBCs are ellipse shape, presentation and treatment is the same as spherocytosis (folate supplementation and splenectomy)

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23
Q

What type of inheritance is G6PD deficiency?

A

X linked recessive

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24
Q

What triggers haemolysis in G6PD deficiency?

A

broad beans, infections, medications (anti-malarials)

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25
Q

What investigations might you do for G6PD deficiency?

A

blood film- Heinz bodies
G6PD enzyme assay

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26
Q

What is autoimmune haemolytic anaemia and what types can it be split into?

A

antibodies are created against the persons RBCs, it can be split into warm and cold type (symptoms occur at warmer or colder temps)

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27
Q

What can cause warm type autoimmune haemolytic anaemia?

A

it is mainly idiopathic

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28
Q

How does cold type autoimmune haemolytic anaemia work?

A

antibodies cause agglutination of RBCs at cold temperatures, they are destroyed by the spleen

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29
Q

How do you manage autoimmune haemolytic anaemia?

A

blood transfusions
prednisolone
rituximab (monoclonal antibody against B cells)
splenectomy

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30
Q

What two scenarios can alloimmune haemolytic anaemia occur?

A

haemolytic transfusion reactions
haemolytic disease of the newborn

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31
Q

What happens in haemolytic transfusion reactions?

A

immune system produces antibodies against antigens of transfused RBCs

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32
Q

What happens in haemolytic disease of the newborn

A

mothers antibodies attack foetus RBCs

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33
Q

What three features result from the destruction of RBCs in haemolytic anaemia?

A

anaemia
splenomegaly - spleen filled with destroyed blood cells
jaundice - unconjugated bilirubinaemia

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34
Q

What are the 1st line investigations for haemolytic anaemia?

A

FBCs -shows normocytic anaemia
blood film shows schistocytes (RBC fragments)
direct Coombs test

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35
Q

What is the direct Coombs test?

A

tests for circulating antibodies that have the potential to induce RBC haemolysis

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36
Q

What is the gold standard investigation for haemolytic anaemia?

A

reticulocyte count (increased >1.5%)

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37
Q

What is the first line treatment for haemolytic anaemia?

A

removing offending agent/underlying condition/supportive care (folic acid + transfusion)

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38
Q

What is aplastic anaemia?

A

pancytopenia (deficiency of all components of blood)
hypocellular marrow (decreased production in haematopoietic cell lineages)
and no abnormal cells

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39
Q

What are the key presentations of aplastic anaemia?

A

recurrent infections
fatigue
pallor
bruising

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40
Q

What are the signs for aplastic anaemia?

A

tachycardia
signs of congenital aetiology - persistent warts, osteoporosis, hearing loss, short stature

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41
Q

What is the main symptom of aplastic anaemia?

A

dyspnoea

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42
Q

What is the first line investigation for aplastic anaemia?

A

FBCs
reticulocyte
bone marrow biopsy (gold standard)

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43
Q

How do you manage aplastic anaemia?

A

immunosuppressive therapy
blood transfusions
antibiotic/fungal agents
stem cell transplant

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44
Q

What inheritance pattern is sickle cell anaemia?

A

autosomal-recessive single gene defect in beta chain of haemoglobin

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45
Q

What are the key presentations of sickle cell anaemia?

A

often picked up because of heel prick test
dactylitis

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46
Q

What are signs of sickle cell anaemia?

A

pallor, jaundice, tachycardia
acute chest syndrome:
chest pain, fever, tachypnoea, dyspnoea, hypoxaemia

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47
Q

What are the symptoms of sickle cell anaemia?

A

bone pain, lethargy, dizziness, visual floaters, SOB, cold peripheries

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48
Q

What are the first line investigations for sickle cell anaemia?

A

peripheral blood smear
FBC/reticulocyte
Hb electrophoresis (separates normal and abnormal Hb)

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49
Q

What are the gold standard investigations for sickle cell anaemia?

A

Hb electrophoresis
high performance liquid chromatography (HPLC)

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50
Q

What is the general management for sickle cell anaemia?

A

abx prophylaxis - penicillin V
hydroxycarbamide - stimulate HbF production
blood transfusions
bone marrow transplant (curative)

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51
Q

What is splenic sequestration crisis (sickle cell)?

A

blockage of blood flow to spleen - leads to severe anaemia and hypovolaemiac shock

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52
Q

How is a splenic sequestration crisis managed?

A

fluid resus, blood transfusion
splenectomy if recurrent

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53
Q

What is an aplastic crisis (sickle cell)?

A

halt in creation of new blood cells triggered by parvovirus B19

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54
Q

What is the management in sickle cell anaemia with vaso-occlusive crisis?

A

analgesia
treatment of priapism

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55
Q

What is the management for sickle cell anaemia with acute chest syndrome?

A

abx or antiviral for infection
blood transfusions
incentive spirometry
artificial ventilation

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56
Q

What can all types of leukaemia lead to?

A

pancytopenia (low RBCs, platelets, WBCs)

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57
Q

When are the different kinds of anaemia most common? (ALL CeLLmates have CoMmon AMbitions)

A

acute lymphocytic leukaemia (ALL) - under 5 and over 45
Chronic lymphoid leukaemia (CLL) - over 55
Chronic myeloid leukaemia (CML) - over 65
Acute myeloid leukaemia (AML) - over 75

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58
Q

What are the key presentations of leukaemia?

A

fatigue
fever
pallor
petechiae
abnormal bleeding
hepatosplenomegaly
lymphadenopathy
(children or young adults with petechiae or hepatosplenomegaly should be referred to the hospital)

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59
Q

What blood tests are used if leukaemia is suspected?

A

FBCs (pancytopenia)
blood film - look for abnormal cells and inclusions
lactate dehydrogenase (raised)

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60
Q

What is the gold standard investigation for leukaemia?

A

bone marrow biopsy (taken from the iliac crest)
bone marrow trephine provides a better assessment but takes a few days

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61
Q

What is acute lymphoblastic leukaemia?

A

malignant change in lymphocyte precursor cell, causes acute proliferation of one type of lymphocyte (normally B lymphocyte)

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62
Q

When is it most common to see acute lymphoblastic leukaemia?

A

2-4 year olds, associated with downs syndrome

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63
Q

What gene translocation is ALL associated with?

A

philadelphia chromosome translocation

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64
Q

What is seen on a blood film in ALL?

A

blast cells

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65
Q

What is the typical treatment for ALL?

A

chemo - methotrexate
steroids - prednisolone

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66
Q

What is chronic lymphocytic leukaemia?

A

chronic proliferation of one type of lymphocyte - typically B lymphocyte

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67
Q

What do patients typically present with with CLL?

A

often asymptomatic
anaemia
bleeding
weight loss
infections
(most common leukaemia in adults)

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68
Q

What can CLL cause in patients? (hint: type of anaemia)

A

warm autoimmune haemolytic anaemia

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69
Q

CLL can transform into high-grade lymphoma. What is this transformation called?

A

Richters transformation

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70
Q

What is seen on a blood film in CLL?

A

smear or smudge cells - fragile RBCs are ruptured and leave a smudge on the film

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71
Q

What is the typical treatment for CLL?

A

chemo - fludarabine
can be used alongside rituximab and cyclophophamide

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72
Q

What is chronic myeloid leukaemia?

A

When an abnormal blast cell takes up a high proportion of the blood (10-20%)

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73
Q

What are the three phases of CML?

A

chronic phase
accelerated phase
blast phase

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74
Q

What are the features of the chronic phase of CML? (time/symptoms)

A

last around 5 years
normally asymptomatic
may be diagnosed accidentally with raised WCC

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75
Q

What are the features of the accelerated phase of CML?

A

abnormal blast cell takes up high proportion of blood - symptomatic:
anaemia
thrombocytopenia
immunocompromised

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76
Q

What are the features of the blast phase in CML?

A

higher proportion of blast cells (>30%)
severe symptoms
pancytopenia
fatal

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77
Q

What gene translocation is CML associated with?

A

Philadelphia chromosome translocation

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78
Q

What is the typical treatment for CML?

A

tyrosine kinase inhibitor (imatinib)

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79
Q

When does acute myeloid leukaemia typically present?

A

middle age (most common acute leukaemia in adults

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80
Q

What myeloproliferative disorders can transform into AML?

A

polycythaemia ruby vera
myelofibrosis

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81
Q

What can be seen on a blood film in AML?

A

blast cells with rods in their cytoplasm called Auer rods

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82
Q

What are the chemotherapies used to treat AML?

A

cytarabine and an anthracycline drug (daunorubicin)

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83
Q

What are the typical treatments for leukaemia?

A

chemotherapy and steroids

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84
Q

What other therapies can be used to treat leukaemias?

A

radiotherapy
bone marrow transplant
surgery

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85
Q

What are some complications of leukaemia?

A

failure
stunted growth in kids
neurotoxicity
infertility
secondary malignancy
cardiotoxicity
tumour lysis syndrome

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86
Q

What is tumour lysis syndrome?

A

release of uric acid from destroyed cells during chemo, can cause acute kidney injury

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87
Q

What medications can reduce uric acid levels in tumour lysis syndrome?

A

allopurinol or rasburicase

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88
Q

What other chemicals can be released in tumour lysis syndrome that need to be monitored?

A

potassium and phosphate

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89
Q

What is Hodgkin’s lymphoma (HL)?

A

malignancy arising from mature B cells

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90
Q

What are the key presentations of HL?

A

lymphadenopathy

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91
Q

What are the signs of HL?

A

Fever
weight loss
night sweats

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92
Q

What would blood tests show in HL?

A

raised lactate dehydrogenase - non specific

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93
Q

What is the gold standard investigation for HL?

A

excisional lymph node biopsy

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94
Q

When is HL most common?

A

peaks at 20 and 75 yrs

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95
Q

What is the a key finding on lymph node biopsy in HL?

A

Reed-Sternberg cells - abnormally large B cells with multiple nuclei that have nucleoli inside them

96
Q

What staging system is used for HL and non-HL?

A

Ann Arbor staging

97
Q

What is stage 1 lymphoma?

A

confined to one region of lymph nodes

98
Q

What is stage 2 lymphoma?

A

more than one region but only either above or below the diaphragm

99
Q

What is stage 3 lymphoma?

A

affects lymph nodes above and below the diaphragm

100
Q

What is stage 4 lymphoma?

A

involvement of non-lymphatic organs such as liver or lungs

101
Q

What is the chemo management for HL? (ABVD)

A

doxorubicin (adriamycin)
bleomycin
vinblastine
dacarbazine
(sometimes followed with radiotherapy)

102
Q

Name a common non-Hodgkins lymphoma

A

Burkitt lymphoma
MALT lymphoma (mucosal-associated lymphoid tissue)
diffuse large B cell lymphoma

103
Q

What is Burkitt lymphoma associated with?

A

EBV, HIV and malaria

104
Q

What is MALT lymphoma associated with/

105
Q

What pesticide is associated with non-HL?

A

trichloroethylene

106
Q

What chemo combination is most commonly used in non-HL? (CHOP)

A

cyclophosphamide
doxorubicin
vincristine
prednisolone

107
Q

What other treatments may be used in non-HL?

A

monoclonal antibodies - rituximab
radiotherapy
stem cell transplant

108
Q

What is myeloma?

A

a cancer of plasma cells that causes rapid multiplication and the production of one type of antibody (usually IgG) called a monoclonal paraprotein.

109
Q

What is MGUS?

A

monoclonal gammopathy of undetermined significance. There is an abundance of one type of antibody without features of myeloma or cancer

110
Q

What is smouldering myeloma?

A

progression of MGUS, premalignant

111
Q

What is myeloma bone disease?

A

increased osteoclast and decreased osteoblast activity due to interaction of cytokines from plasma cells - increased risk of fractures

112
Q

How can myeloma effect the kidneys?

A

can cause myeloma renal disease - tubules can become blocked by the immunoglobulins

113
Q

How does myeloma effect plasma viscosity?

A

it increases it

114
Q

What can increased plasma viscosity lead to?

A

easy bruising/bleeding
purple discolouration of extremities
heart failure
vision loss

115
Q

What are the key presentations of myeloma? (CRAB)

A

calcium (elevated
renal failure
anaemia
bone lesions/pain

116
Q

When should you consider investigations for myeloma?

A

anyone over 60 with persistent bone pain

117
Q

What first line investigations should you do for myeloma?

A

FBC - low WCC
Calcium - raised
ESR - raised
plasma viscosity - raised

118
Q

What are the 2nd line investigations for myeloma? (BLIP)

A

Bence jones protein (urine electrophoresis)
serum free Light-chain assay
serum Immunoglobulin
serum Protein electrophoresis

119
Q

What is the gold standard investigation for myeloma?

A

bone marrow biopsy

120
Q

How can you assess for bone lesions in myeloma?

A

whole body MRI or CT
skeletal survey (X-ray)

121
Q

What is the initial chemotherapy for myeloma?

A

bortezomid
thalidomide
dexamethasone

122
Q

What prophylaxis is needed for chemotherapy with thalidomide?

A

VTE prophylaxis with aspirin or LMWH

123
Q

How can you manage myeloma bone disease?

A

bisphosphonates (suppress osteoclasts)
radiotherapy
orthopaedic surgery (stabilise bones)
cement augmentation of fractures/lesions

124
Q

Name three myeloproliferative disorders

A

primary myelofibrosis
polycythaemia vera
essential thrombocythaemia

125
Q

What cell line undergoes proliferation in primary myelofibrosis?

A

haematopoietic stem cell

126
Q

What cell line undergoes proliferation in polycythaemia vera?

A

erythroid cells

127
Q

What cell line undergoes proliferation in essential thrombocythaemia?

A

megakaryocytes

128
Q

myoproliferative disorders are associated with which genes?

A

JAK2
MPL
CALR

129
Q

What is polycythaemia?

A

a high concentration of RBCs in the blood

130
Q

What are the types of polycythaemia?

A

absolute (increased number of erythrocytes) - primary and secondary
relative (normal RBCs, reduced plasma)

131
Q

what is myelofibrosis?

A

the proliferation of a cell line leads to fibrosis of the bone marrow

132
Q

Why does myelofibrosis happen?

A

in response to cytokines (typically fibroblast growth factor) from proliferating cells

133
Q

What can myelofibrosis lead to?

A

anaemia and leukopenia

134
Q

what is it called when blood cell production starts to happen in other areas like the liver and spleen?

A

extramedullary haematopoiesis

135
Q

What can extramedullary haematopoiesis lead to?

A

hepatosplenomegaly

136
Q

What are the typical systemic symptoms of myelofibrosis?

A

fatigue
weight loss
night sweats
fever

137
Q

What signs may show in myelofibrosis due to underlying problems?

A

anaemia (not in polycythaemia)
abdominal pain (splenomegaly)
ascites, varices, abdo pain (portal hypertension)
bleeding and petechiae (low platelets)
thrombosis, red face (raised RBCs)
infections (low WCC)

138
Q

What are the key presentations of polycythaemia vera?

A

conjunctival plethora (redness in conjunctiva)
a ruddy complexion (red)
splenomegaly

139
Q

What would and FBC show in polycythaemia vera?

140
Q

What would an FBC show in primary thrombocythaemia?

A

raised platelet count

141
Q

What would an FBC show in myelofibrosis (due to primary MF, PV or ET)

A

anaemia
High or low WCC
high or low platelets

142
Q

What are the gold standard tests for myelofibrosis?

A

bone marrow biopsy
bone marrow aspiration - dry
genetic testing or JAK2, MPL, CALR

143
Q

How can you manage polycythaemia?

A

venesection - to keep Hb down
aspirin VTE prophylaxis
chemo - hydroxycarbamide

144
Q

How do you manage primary myelofibrosis if symptomatic/severe?

A

allogenic stem cell transplant
chemo - ruxolitinab (JAK2 inhibitor)
supportive management for anaemia/hypertension/splenomegaly

145
Q

How do you manage essential thrombocythaemia?

A

aspirin
chemo - hydroxycarbamide

146
Q

What is pernicious anaemia?

A

lack of intrinsic factor which leads to B12 deficiency

147
Q

What does vitamin B12 do?

A

production of blood cells and myelination of nerves

148
Q

What are the key presentations of pernicious anaemia?

A

fatigue
pallor
peripheral neuropathy

149
Q

What are the neurological symptoms of pernicious anaemia?

A

paraesthesia (pins and needles)

150
Q

What is the 1st line investigation for pernicious anaemia?

A

intrinsic factor antibody

151
Q

What are the 1st line treatments for pernicious anaemia with no neurological features?

A

oral replacement with cyanocobalamin

152
Q

What are the 2nd line treatments for pernicious anaemia?

A

IM injection of 1mg hydroxycobalamin 3x weekly for 2 weeks

153
Q

What is the treatment for pernicious anaemia with neurological symptoms?

A

1mg hydroxycobalamin injection every other day until symptoms improve

154
Q

What supplementation can be used to treat pernicious anaemia?

A

folic acid

155
Q

What can treating B12 deficient patients with folate lead to?

A

subacute combined degeneration of the cord

156
Q

What is glucose-6-phosphate deficiency (G6PD)?

A

inherited X linked enzyme where patients are susceptible to developing haemolytic anaemia?

157
Q

What does glucose-6-phosphate do for RBCs?

A

prevent oxidation damage by reducing NADP to NADPH

158
Q

What are the key presentations of G6P deficiency?

A

jaundice
pallor
dark urine

159
Q

What is the 1st line test for G6P deficiency?

160
Q

What is the gold standard test for G6PD?

A

G6PD spectrophotometric assay

161
Q

What is the 1st treatment for G6PD with acute haemolysis?

A

supportive care + folic acid + blood transfusion
(+/- splenectomy in chronic non-spherocytic haemolytic anaemia w/ splenomegaly)

162
Q

What is the treatment for G6PD in neonates with prolonged indirect hyperbilirubinemia?

A

phototherapy and exchange transfusion

163
Q

What are the key presentations of malaria?

A

fever/ history of fever
headache
weakness

164
Q

What are the first line investigations for malaria?

A

giemsa-stained thick and thin blood smears (gold standard)

165
Q

what is the first line treatment for malaria?

A

chloroquine or hydroxychloroquine

166
Q

What are the key presentations of HIV?

A

fevers/night sweats
fatigue
lymphadenopathy
weight loss
skin rashes
oral ulcers/thrush
angular cheilitis

167
Q

What are the 1st line investigations for HIV?

A

serum HIV enzyme-linked immunosorbent assay (ELISA)
HIV rapid test

168
Q

What is the gold standard test for HIV?

A

serum western blot

169
Q

What is the 1st line treatment for HIV?

A

antiretroviral therapy (ART)- INSTI, PI, NNRTI

170
Q

What can cause problems with production of platelets?

A

sepsis
B12 or folic acid deficiency
liver failure - thrombopoietin deficiency
leukaemia
myelodysplastic syndrome

171
Q

What can cause abnormal destruction of platelets?

A

medications
alcohol
ITP
TTP
heparin-induced thrombocytopenia
haemolytic-uraemic syndrome

172
Q

What symptoms might mild (<50x10^9/l) thrombocytopenia have?

A

easy bruising/bleeding
nosebleeds
bleeding gums
heavy periods
blood in urine or stools

173
Q

What are the risks of severe thrombocytopenia (<10x10^9/L)?

A

high risk of spontaneous cranial bleeding
or GI bleeds

174
Q

what is immune thrombocytopenic purpura (ITP)?

A

Where antibodies (IgG) are created against platelets and cause low platelet count

175
Q

What is secondary ITP?

A

all forms of ITP where associated medical conditions can be identified

176
Q

What are the key presentations of ITP?

A

bleeding
absence of systemic symptoms (weight loss, fever etc) absence of TP causing meds, absent spleno/hepatomegaly
absent lymphadenopathy
purpura

177
Q

What is the 1st line test for ITP?

A

FBC - high WCC, low Hb, low platelets

178
Q

What medicinal treatments are there for ITP?

A

corticosteroid - prednisolone
IV immunoglobulins
rituximab (monoclonal antibody against B cells)

179
Q

What surgical procedure may be done for ITP if the spleen is trapping platelets?

A

splenectomy

180
Q

What is thrombotic thrombocytopenic purpura (TTP)?

A

a syndrome that causes clots to develop in the small vessels

181
Q

In which protein is there an abnormality in TTP?

182
Q

What does the ADAMTS13 protein do?

A

inactivated von Willebrand factor to reduce platelet adhesion

183
Q

How do the blood clots in TTP effect RBCs?

A

They break them up causing haemolytic anaemia

184
Q

what are the key presentations of TTP?

A

non-specific prodrome
neurological symptoms:
coma, focal abnormalities, seizures, headache, confusion

185
Q

What are the first line investigations for TTP?

A

FBCs - raised WCC, low Hb, low platelets
raised bilirubin
raised creatine
schistocytes on blood smear

186
Q

What is the 1st line treatments for TTP?

A

plasma exchange + corticosteroids (prednisolone)
rituximab (monoclonal antibody against B cells)

187
Q

What is heparin induced thrombocytopenia (HIT)?

A

exposure to heparin causes the creation of antibodies against platelets

188
Q

How do the heparin induced antibodies effect platelets?

A

BOTH bind to platelets to activate clotting mechanisms
AND
break down platelets
leads to low platelets and hypercoagulable state

189
Q

How id HIT diagnosed?

A

HIT antibodies in blood

190
Q

What is alpha thalassemia?

A

disorders of haemoglobin synthesis in the alpha chains

191
Q

What are the key presentations of thalassemia?

A

microcytic anaemia
fatigue
pallor jaundice
splenomegaly

192
Q

What is the gold standard investigation for thalassemia?

A

Gap-PCR (shows DNA deletions or gene rearrangements)
FBC shows microcytic anaemia
Hb electrophoresis

193
Q

What is the treatment for mild/severe alpha thalassemia?

A

frequent blood transfusions + folate supplementation
chelation therapy (removes excess iron in blood)
splenectomy
bone marrow transplant can be curative

194
Q

What are the signs of beta thalassemia?

A

low height/weight
large head
misaligned teeth
spinal changes

195
Q

What is the 1st line management for people with beta thalassemia trait?

A

genetic counselling + iron advice

196
Q

What is the treatment for beta thalassemia intermedia?

A

blood transfusions (1st line)
chelation
splenectomy
genetic counselling
stem cell transplant

197
Q

What is the treatment for beta thalassemia major?

A

1st line: regular transfusion + iron monitoring + chelation + genetic counselling
splenectomy
stem cell transplant

198
Q

What is spherocytosis?

A

an autosomal dominant RBC abnormality, defects in structural membrane proteins

199
Q

What is elliptocytosis?

A

autosomal dominant condition, causes ellipse shaped RBCs

200
Q

What are the key presentations of membranopathy (spherocytosis/elliptocytosis)

A

pallor
jaundice
splenomegaly
fatigue

201
Q

What is the gold standard test for membranopathies?

A

NaCl osmotic fragility tests

202
Q

What is the first line treatment for membranopathies?

A

RBC transfusions

203
Q

What are alternative managements for membranopathies?

A

folic acid supplementation
splenectomy

204
Q

What are the 4 types of platelet disorders?

A

reduced production
increased clearance
sequestering in spleen
dilution

205
Q

What are the key presentations for platelet disorders?

A

petechiae/ecchymoses
retinal haemorrhage
bleeding at the back of the buccal mucosa

206
Q

What are the 1st line investigations for platelet disorders?

A

FBCs
peripheral smear

207
Q

What is Von Willebrand’s disease (VW)?

A

the most common inherited bleeding disorder due to abnormalities in the VWF

208
Q

There are many underlying genetic causes of VWD, what is the most common inheritance pattern?

A

autosomal dominant

209
Q

What are the key presentations of VW?

A

bleeding from minor wounds
post op bleeding
easy bruising
menorrhagia
(FH of bleeding is very relevant)

210
Q

What is the gold standard test for VW?

A

vWF multimer analysis

211
Q

What is the first line management for VW?

A

Desmopressin - stimulates vWF
vWF infusion
factor VIII infusion

212
Q

What can be given to pts experiencing menorrhagia with VW?

A

tranexamic acid
mefanamic acid
norethisterone
combined oral contraceptive pill
mirena coil

213
Q

What procedure may be required in extreme cases of VWD in women?

A

hysterectomy

214
Q

What deficiency causes haemophilia A?

A

Factor VIII

215
Q

What deficiency causes haemophilia B?

216
Q

What inheritance pattern is haemophilia?

A

X linked recessive

217
Q

How does haemophilia present in neonates?

A

intracranial haemorrhage
haematomas
cord bleeding

218
Q

What are the classic presentations of haemophilia A/B?

A

bleeding into muscles and joints (haemoathrosis)

219
Q

What is the gold standard investigation for haemophilia?

A

plasma factor VIII, IX assay
genetic testing

220
Q

What is the 1st line treatment for haemophilia?

A

IV clotting factors (VII, IX)

221
Q

What is a complication of IV clotting factor infusion?

A

antibodies being produced against the clotting factors

222
Q

What is the management for acute bleeding episodes or surgery in haemophilia?

A

infusions of clotting factor
desmopressin
antifibrinolytics

223
Q

Why is desmopressin given in haemophilia?

A

it activated Von Willebrand factor

224
Q

Give an example of an antifibrinolytic

A

tranexamic acid

225
Q

What is disseminated intravascular coagulopathy?

A

an acquired syndrome causing activation of coagulation pathways resulting in thrombi and platelet depletion

226
Q

What are the key presentations of disseminated intravascular coagulopathy?

A

oliguria
hypotension
tachycardia

227
Q

What are the 1st line investigations for disseminated intravascular coagulopathy?

A

platelet count (low)
fibrinogen (low)
D-dimer (high)
high PT/APTT

228
Q

What is the first line treatment for disseminated intravascular coagulopathy?

A

treatment of underlying cause (sepsis)

229
Q

What are other treatments for disseminated intravascular coagulopathy?

A

platelets/ coag factor inhibitors
heparin
cyroprecipitate (if low fibrinogen)

230
Q

What is glandular fever?

A

a clinical syndrome most commonly caused by epstein-barr virus infection

231
Q

What are the key presentations of glandular fever?

A

fever
pharyngitis
cervical or generalised lymphadenopathy
malaise

232
Q

What are the 1st line investigations for glandular fever?

A

FBCs, LFTs
EBV specific antibodies

233
Q

What is the gold standard investigation for glandular fever?

A

in-situ hybridisation

234
Q

What is the 1st line management for glandular fever?

A

supportive care
corticosteroid if w/ haemolytic anaemia
or w/ thrombocytopenia

235
Q

What happens in haemolytic disease of the newborn

A

mothers antibodies attack foetus RBCs

236
Q

What are the neurological symptoms of pernicious anaemia?

A

paraesthesia (pins and needles)

237
Q

What is the gold standard investigation for leukaemia?

A

bone marrow biopsy (taken from the iliac crest)
bone marrow trephine provides a better assessment but takes a few days

medicine phase 2 a (10 decks)

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