Haematolgy Flashcards

1
Q

Differentiate the causes of megaloblastic and non-megaloblastic macrocytic anaemia

A

Megaloblastic: B12/folate deficiency (pregnancy, old age, veganism)
Non-megaloblastic: liver disease/ alcoholism/ multiple myeloma

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2
Q

Recall the 2 key symptoms and the 3 key signs of any anaemia

A

Symptoms: lethargy, breathlessness
Signs: pallor, tachycardia, breathlessness

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3
Q

Recall 3 signs of pernicious anaemia that might be present in addition to the normal signs of anaemia

A

Glossitis
Angular stomatitis
Mild jaundice

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4
Q

What signs of B12 deficiency may be present alongside an anaemia?

A

Peripheral neuropathy
Optic atrophy
Dementia

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5
Q

How are LFTs commonly deranged in macrocytic anaemia and why?

A

High bilirubin

Ineffective erythropoiesis, haemolysis

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6
Q

What blood tests are done to detect B12 and folate levels?

A

Serum cobalamin

Red cell folate

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7
Q

As well as macrocytic erythrocytes, what can be seen on a blood film in megaloblastic anaemia?

A

Hypersegmented neutrophils

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8
Q

What test is done to diagnose pernicious anaemia?

A

Schilling test

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9
Q

Recall the management of pernicious anaemia

A

IM hydroxycobalamin for life

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10
Q

What is the most likely cause of a microcytic anaemia?

A

Iron deficiency

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11
Q

Recall 4 causes of iron deficiency

A

GI blood loss
Small bowel disease
Vegan
Pregnancy

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12
Q

What size are the red cells in anaemia of chronic disease?

A

Microcytic

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13
Q

In what sort of anaemia may the patient develop brittle nails and hair?

A

Iron deficiency anaemia

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14
Q

What might an FBC show in microcytic anaemia?

A

Low Hb
Low MCV
High reticulocytes

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15
Q

Recall the iron, TIBC and ferritin seen in iron deficiency anaemia

A

Iron: low
TIBC: high
Ferritin: low

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16
Q

Does thalassaemia produce a microcytic or a macrocytic anaemia?

A

Microcytic

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17
Q

Systematically recall some causes of normacytic anaemia

A

Decreased production: vit B2/6 deficiency, ACD, aplastic anaemia
Increased destruction: haemolysis
Increased blood volume: fluids, pregnancy

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18
Q

Define aplastic anaemia

A

A pancytopaenia due to fewer haemotopoietic precursors

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19
Q

What symptoms and signs of aplastic anaemia will not be present in any other aetiology of anaemia?

A
Symptoms of thrombocytopaenia
Purpura
Easy bruising
Epistaxis
Petechiae
Also multiple infections due to leukocytopaenia
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20
Q

What are the 2 different types of disseminated intravascular coagulation?

A

Acute overt

Chronic non-overt

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21
Q

Differentiate the pathophysiology of acute overt and chronic non-overt disseminated intravascular coagulation

A

Acute overt: bleeding –> depletion of platelets and clotting factors
Chronic non-overt: thromboembolism –> generalised activation of coagulation system

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22
Q

What sort of infection is most likely to cause disseminated intravascular coagulation?

A

Gram negative sepsis

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23
Q

Describe the pathophysiology of DIC

A

Endothelial damage –> tissue factor release
Tissue factor activates thrombin
Thrombin depletes clotting factors and platelets
Thrombin activates fibrinolysis
Fibrinolysis releases fibrin, which occludes the microvasculature

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24
Q

Which patients are particularly at risk of disseminated intravascular coagulation?

A

Pretty much all severely unwell patients will get

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25
Q

Differentiate the signs that are present in acute and chronic disseminated intravascular coagulation

A

Acute: purpura, petechiae, ecchymoses, epistaxis
Chronic: signs of DVT/ arterial thromboses

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26
Q

Describe the FBC of someone with disseminated intravascular coagulation

A

Low Hb and platelets

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27
Q

What should be tested for alongside FBC in the blood in suspected disseminated intravascular coagulation

A

APTT (will be high)

FDP (will also be high)

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28
Q

Recall the pathophysiology of haemachromatosis

A

GENETIC increase in iron absorption, iron accumulates in tissues, organs damaged

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29
Q

Recall some late symptoms of haemachromatosis

A
T2DM
Bronzed skin
Hepatomegaly
Cirrhosis
Hypogonadism
Cardiac arrhythmias/ cardiomyopathy
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30
Q

Recall the results of haematinic investigations in haemachromatosis

A

Ferritin: high
Transferrin: low
[Transferrin]: high
TIBC: low

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31
Q

Systematically recall possible causes of haemolytic anaemia

A
  1. Hereditary
    a) membrane defects - elliptocytosis, hereditary spherocytosis
    b) haemaglobinopathies: thalassaemia, sickle-cell disease
    c) metabolic defects: G6PD deficiency, PKU deficiency
  2. Acquired
    Infection: sepsis, malaria
    Trauma
    Drugs
    Autoimmune
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32
Q

Recall 2 symptoms of haemolytic anaemia that are not present in other types of anaemia

A

Jaundice (due to increased red cell breakdown)

Haematuria (due to haemolysis)

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33
Q

Is haemolytic anaemia macrocytic or microcytic?

A

Macrocytic

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34
Q

Recall 3 abnormalities found on urinalysis in haemolytic anaemia

A
  1. High urobilinogen
  2. Haemaglobinuria
  3. Haemosiderinuria
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35
Q

What specific test is done for auti-immune haemolytic anaemia?

A

Direct Coomb’s test

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36
Q

What are the 2 categories of haemolytic uraemic syndrome

A

D+ (with diarrhoea, usually children)

D- (no prodromal illness)

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37
Q

Define haemolytic uraemic syndrome

A

Triad of microangiopathic haemolytic anaemia, thrombocytopaenia and acute renal failure

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38
Q

Explain the pathophysiology of HUS and TTP

A

Endothelial damage –> platelets aggregate
1a. Platelet aggregation –> thrombi formation –> promotion of intravascular haemolysis
1b. Platelet aggregation –> loads of vWF released –> small vessel thrombosis
Small vessel thrombi most likely to affect afferent glomerular arterioles which is why HUS + TTP –> renal failure

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39
Q

What is the difference between thrombotic thrombocytopaenic purpura and haemolytic uraemic syndrome

A

TTP has extra clinical manifestation of fever and CNS signs

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40
Q

Which pathogen is the most common cause of HUS and where it is usually found?

A

E Coli 0157

Contaminated water

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41
Q

Recall the symptoms of HUS

A

General sickness/ malaise
GI symptoms: bloody diarrhoea, severe colic
Renal symptoms: oligouria, haematuria

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42
Q

Describe the FBC results for someone with HUS and TTP

A

Normacytic anaemia
High neutrophils
VERY LOW platelets

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43
Q

What would an ABG show in HUS?

A

Lactic acidosis

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44
Q

What would a blood film show in HUS?

A

High reticulocytes and schistocytes

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45
Q

Recall 3 symptoms of haemophilia

A
  1. Haemarthroses
  2. Haematuria
  3. Deep bleeding
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46
Q

Recall 2 investigations that should be done in suspected haemophilia

A

Clotting factor assay

APTT

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47
Q

What is immune thrombocytopaenic purpura?

A

Immune destruction of platelets

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48
Q

What is the antibody that is implicated in immune thrombocytopaenic purpura?

A

Anti-GpIIb/ anti-GPIIIa

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49
Q

Recall the general signs/ symptoms of ITP

A

Easy bruising, epistaxis, visible purpura, menorrhagia

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50
Q

What will the result of a clotting screen be in ITP?

A

Normal

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51
Q

Why is it important to do a blood film in suspected ITP?

A

Need to rule out pseudothrombocytopaenia

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52
Q

What is the most common malignancy of childhood?

A

Acute lymphoblastic leukaemia

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53
Q

Systematically recall the symptoms of acute lymphoblastic leukaemia

A

Due to reduced synthesis of blood cells: anaemia, easy bruising, recurrent infections
Due to organ infiltration: tender bones, lymphadenopathy, meningism

54
Q

Systematically recall the signs of acute lymphoblastic leukaemia

A

Due to bone marrow failure: bruising, pallor

Due to tissue infiltration: lymphadenopathy, hepatosplenomegaly, retinal haemorrhage + papilloedema, testicular swelling

55
Q

What sort of anaemia results from acute lymphoblastic leukaemia?

A

Normacytic normachromic

56
Q

Which enzyme is elevated in the blood in cancer patients?

A

LDH

57
Q

Recall one blood test result that is a poor prognostic factor for acute lymphoblastic leukaemia

A

Uric acid high

58
Q

Recall 4 appropriate investigations in suspected acute lymphoblastic leukaemia

A
  1. Bloods (FBC, U&Es, LDH)
  2. Blood film and BM aspirate
  3. Immunophenotyping
  4. CXR to look for mediastinal lymphadenopathy
59
Q

Recall some signs of acute myeloblastic leukaemia that result from tissue infiltration, and aren’t seen in acute lymphoblastic leukaemia

A

Gum swelling/ bleeding
Skin rashes
CNS involvement: headache and diplopia

60
Q

What is the expected white cell count in acute myeloblastic leukaemia?

A

Can be variable

61
Q

In which demographic is acute myeloblastic leukaemia most common?

A

The elderly: incidence increases with age

62
Q

Define chronic lymphocytic leukaemia

A

Progressive accumulation of functionally incompetent lymphocytes which have monoclonal origin

63
Q

What causes lymphocytes to accumulate in CLL?

A

Failure of apoptosis

64
Q

Which type of leukaemia is most likely to be asymptomatic?

A

CLL

65
Q

Recall 2 signs of CLL on examination

A

Non-tender lymphadenopathy

Hepatosplenomegaly

66
Q

Recall 2 findings on the blood film in CLL

A

Small lymphocytes

Smudge cells

67
Q

With which other disease does CLL overlap?

A

Non-hodgkn’s lymphoma

68
Q

What type of cell is hyperproliferating in CML?

A

Granulocyte precursors

69
Q

What translocation occurs to produce the Philadelphia chromosome, and what sort of cancer does this predispose to?

A

Chromosomes 9 and 22
BCR-ABl fusion
Chronic myeloid leukaemia

70
Q

In what sort of pateints is CML most likely to occur?

A

Men (4 x more common than in women)

71
Q

Recall the 3 phases of CML

A

Stable chronic (4-6 years)
Accelerated (3-6 months)
Acute (blast transformation)

72
Q

Recall a sign that is present in 90% of CML patients

A

Splenomegaly

73
Q

What is the most likely aetiology of hypercobalaminaemia?

A

Haematological neoplastic cause

74
Q

Define Hodgkin’s lymphoma

A

Neoplasm of lymphoid cells originating in the lymph nodes

75
Q

How is Hodgkin’s lymphoma diagnosed?

A

Histopathologically, based on presence of REED-STENBERG cells

76
Q

What are reed-stenberg cells and in which disease do they appear in the blood film?

A

Bi-nucleate lymphocytes

Hodgkin’s lymphoma

77
Q

What is the main symptom of Hodgkin’s lymphoma?

A

Painless enlarging neck mass, that may become painful following alcohol consumption

78
Q

How are the lymph nodes described in lymphoma?

A

Painless, firm and rubbery

79
Q

What staging system is used to stage lymphoma?

A

Ann Arbor

80
Q

What sign may be present on the skin of someone with hodgkin’s lymphoma/ non-hodgkin’s lymphoma

A

Hodgkin’s: excoriations

Non-Hodgkin’s: rashes eg. mycosis fungoides

81
Q

In which types of cancer are ESR and CRP likely to be elevated?

A

Hodgkin’s/non-Hodgkin’s lymphomas

82
Q

What % of Non-Hodgkin’s lymphomas involve B cells, T cells and NK cells?

A

85% B cells

15% T and NK cells

83
Q

Recall one viral cause of non-Hodgkin’s lymphoma

A

EBV virus, causing Burkitt’s lymphoma

84
Q

Recall 3 places where a mass may appear due to non-Hodgkin’s lymphoma

A

Neck
Groin
Axilla

85
Q

Which type of lymphoma is most common?

A

Non-Hodgkin’s

86
Q

Recall one common electrolyte imbalance in non-Hodgkin’s lymphoma

A

Hypercalcaemia

87
Q

Recall one difference in the FBC between Hodgkin’s lymphoma and non-Hodgkin’s lymphoma

A

In Hodgkin’s = neutrophilia, in non-Hodgkin’s = neutropaenia

88
Q

What is multiple myeloma a proliferation of?

A

Plasma cells

89
Q

Recall 2 symptoms of multiple myeloma

A

Symptoms: bone pain, recurrent infection

90
Q

Recall 3 signs of multiple myeloma

A

Heart problems (tachycardia, flow murmur, heart failure)
Carpal tunnel
Macroglossia
(why???)

91
Q

What is a key finding on the blood film in multiple myeloma?

A

Rouleaux

= suggestive of high protein

92
Q

Why is an X ray important to do in suspected multiple myeloma?

A

Osteolytic lesions will be seen

93
Q

What is myelodysplasia?

A

A group of syndromes where the immature blood cells in the blood do not mature normally causing chronic pancytopaenia

94
Q

Recall the 5 subgroups of myelodysplasia

A

Refractory anaemia
Refractory anaemia and ringed suderoblasts
Refractory anaemia with excess blasts
Refractory anaemia with excess blasts in transformation
Chronic myeloMONOCYTIC leukaemia

95
Q

What is the most common cause of secondary myelodysplasia?

A

Chemotherapy/ radiotherapy

96
Q

What are the symptoms of a myelodysplastic syndrome?

A

May be asymptomatic, or exhibit similar symptoms to any blood neoplasm due to loss of functional mature blood cells - anaemia, recurrent infections, easy bruising

97
Q

Describe the epidemiology of myelofibrosis

A

Really rare

98
Q

What is myelofibrosis?

A

Disorder of haematopoietic stem cells characterised by progressive bone marrow fibrosis

99
Q

What is the main histopathological finding on the blood film of someone with myelofibrosis?

A

Increased number of abnormal megakaryocytes

100
Q

What sign will always be present in myelofibrosis and why?

A

Splenomegaly, due to extramedullary haematopoiesis

101
Q

Define polycythaemia

A

Higher than normal concentration of haemoglobin

102
Q

Differentiate between relative polycythaemia and polycythaemia vera

A

Relative - normal MCV and decreased plasma volume

True - increased MCV

103
Q

Differentiate the aetiology of primary and secondary polycythaemia

A

Primary: clonal proliferation of myeloid cells
Secondary: increased erythropoietin

104
Q

Recall 5 symptoms of polycythaemia

A
Angina
Chorea
Gout
Headache
Visual changes
105
Q

Recall 3 signs of polycythaemia

A
  1. Red complexion
  2. Conjunctional suffusion (redness)
  3. 75% have splenomegaly
106
Q

What investigation is done to differentiate between polycythaemia vera and relative polycythaemia?

A

Isotope dilution

107
Q

Describe the erythropoietin level in polycythaemia vera and relative polycythaemia

A

Polycythaemia vera: low

Relative polycythaemia: high

108
Q

Differentiate sickle cell anaemia and sickle cell disease

A

SCA: homozygous HbS
SCD: heterozygous HbS and HbC OR HbS and Beta-thalassaemia

109
Q

What is the gene mutation in HbS?

A

Glutamic acid replaced with valine at position 6 on beta globin gene

110
Q

Recall 4 symptoms of sickle cell anaemia

A
  1. Pneumococcal infection due to autosplenectomy
  2. Abdominal and bone pain
  3. CNS symptoms: fits and strokes
  4. In children: dactylitis
111
Q

Recall 2 signs of a sickle cell crisis

A

Acute chest syndrome

Priapism

112
Q

Recall 2 abnormal erythrocytes seen on a blood film of someone with sickle cell disease

A

Target cells

Howel-Jolly bodies

113
Q

Recall one medical treatment option for sickle cell

A

Hydroxyurea - induces HbF production

114
Q

Which virus is especially dangerous to sickle cell patients and why?

A

Parvovirus B19

Aplastic crisis

115
Q

What is the life expectancy of someone with sickle cell anaemia?

A

50

116
Q

What is the inheritance pattern of thalassaemia?

A

Autosomal recessive

117
Q

Recall the 4 types of alpha thalassaemia

A
Number of gene deletions:
4 = HbBarts: fatal in utero
3 = HbH (causes anaemia and splenomegaly)
2 = alpha 0, no anaemia
1 = alpha +, no anaemia
118
Q

Recall the 3 types of beta thalassaemia

A

Major: homozygous
Intermedia: causes a microcytic anaemia, with fewer alpha chains and more gamma chains
Trait: heterozygous and asymptomatic

119
Q

Systematically recall the symptoms of beta thalssaemia

A

Due to anaemia: pallor, malaise, dyspnoea
Due to increased red cell lysis: jaundice
Due to extramedullary erythropoiesis: splenomegaly
Frontal bossing

120
Q

Is the anaemia caused by thalassaemia macro/microcytic?

A

Microcytic

121
Q

Where is vitamin B12 absorbed following consumption?

A

Terminal ileum

122
Q

What cofactor is required for Vit B12 absorption?

A

Intrinsic factor

123
Q

What is the pathophysiology of pernicious anaemia?

A

Autoimmune condition directed against intrinsic factor - so Vit B12 can’t be absorbed

124
Q

Why does metformin have a side effect of anaemia?

A

Impairs Vit B12 absorption

125
Q

Recall the symptoms of B12 deficiency

A

Anaemia symptoms
Palpitations
Headache
Neurological symptoms - paraesthesia and visual disturbance

126
Q

What would be seen on a blood film of someone with vit B12 deficiency?

A

Hypersegmented neutrophils

127
Q

Which 2 antibodies are implicated in pernicious anaemia?

A

Anti-IF

Anti-parietal cell

128
Q

What type of bleeding is exhibited in Von Willebrand disease?

A

Mucocutaneous

129
Q

Differentiate the 3 different types of VWF disease

A

Type 1: depleted volume of well-functioning VWF
Type 2: adequate volume of ill-functioning VWF
Type 3: NO VWF

130
Q

Recall 3 symptoms of VWF disease

A

Easy bruising
Epistaxis
Blood in stools/ urine