Haematology Flashcards

1
Q

What are reticulocytes?

A

Immature RBCs

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2
Q

What makes platelets?

A

Megakaryocytes

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3
Q

Where do B cells mature?

A

The bone marrow

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4
Q

Where do T cells mature?

A

The thymus

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5
Q

What is anisocytosis and what is it a feature of?

A

Anisocytosis is variation in the size of RBCs. Myelodysplasic syndrome

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6
Q

When are target cells seen on blood film?

A

In iron deficiency anaemia and post-splenectomy

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7
Q

When are heinz bodies seen on blood film?

A

G6PD and alpha thalassaemia

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8
Q

When are lots of reticulocytes seen on blood film?

A

Haemolytic anaemia

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9
Q

What are schictocytes?

A

fragments of RBCs

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10
Q

When are smudge cells seen on blood film?

A

In CLL

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11
Q

what are the 5 common causes of microcytic anaemia?

A
TAILS:
T halassaemia
A naemia of chronic disease
I ron deficiency
L ead poisoning
S ideroblastic anaemia
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12
Q

What are the 5 common causes of normocytic anaemia?

A
3 As, 2Hs
Acute blood loss
Anaemia of chronic disease
Aplastic anaemia
Haemolytic anaemia
Hypothyroidism
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13
Q

What are the causes of macrocytic anaemia?

A

Megaloblastic (due to deficiency of folate or B12)
Alcohol
Azathrioprine
Liver disease

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14
Q

What is Pica?

A

A symptom seen in iron deficiency anaemia. It is a dietary craving for things such as dirt

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15
Q

What causes pernicious anaemia?

A

Lack of vitamin B12

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16
Q

Where is intrinsic factor produced and what is its function?

A

Parietal cells in the gastric mucosa. It is essential for the absorption of vitamin B12

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17
Q

Why does pernicious anaemia cause a lack of vitamin B12?

A

It is an autoimmune condition where antibodies form against parietal cells or intrinsic factors

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18
Q

What are the symptoms of pernicious anaemia?

A

Peripheral neuropathy
Loss of vibration or proprioception
Visual changes
Mood or cognitive changes

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19
Q

How do you test for pernicious anaemia?

A

Intrinsic factor antibody

Gastric parietal cell antibody

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20
Q

How is pernicious anaemia treated?

A

Cyanocobalamin orally or IM hydroxycobalamin

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21
Q

What is the protocol of there is folate deficiency alongside B12 deficiency?

A

Treat B12 first. Treating B12 deficiency with folate too can lead to subacute combined degeneration of the cord

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22
Q

Name some inherited haemolytic anaemias

A
Hereditary spherocytosis
Hereditary elliptocytosis
Thalassaemia
Sickle cell anaemia
G6PD deficiency
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23
Q

What are the 3 features of haemolytic anaemia?

A

Anaemia
Jaundice
Splenomegaly

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24
Q

What investigations should be done if haemolytic anaemia is suspected?

A

FBC (normocytic anaemia)
Blood film (shows schistocytes)
Direct Coombs test (positive in haemolytic anaemia)

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25
Q

What inheritance pattern does hereditary spherocytosis have?

A

Autosomal dominant

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26
Q

How does hereditary spherocytosis present?

A

Jaundice, gallstones, splenomegaly and aplastic crisis

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27
Q

What is seen on blood film in hereditary spherocytosis?

A

Spherocytes

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28
Q

What is the treatment for hereditary spherocytosis?

A

Folate supplementation, splenectomy

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29
Q

What inheritance pattern is hereditary elliptocytosis?

A

Autosomal dominant

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30
Q

What inheritance pattern is G6PD deficiency?

A

X-linked recessive

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31
Q

What can trigger a G6PD deficiency crisis?

A

Infections, medications and fava beans

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32
Q

What does G6PD deficiency present with?

A

Jaundice (usually in the neonatal period), gallstones, anaemia, splenomegaly

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33
Q

What is seen on blood film in G6PD deficiency?

A

Heinz bodies

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34
Q

What is the diagnostic test for G6PD deficiency?

A

G6PD enzyme assay

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35
Q

Which drugs can precipitate a G6PD crisis?

A

Primaquine (antimalarial), ciprofloxacin, sulfonylureas, sulfasazine

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36
Q

What is the management of autoimmune haemolytic anaemia?

A

Blood transfusion
Prednisolone
Rituximab
Splenectomy

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37
Q

What is alloimmune haemolytic anaemia? When does this occur?

A

Foreign blood cells or antibodies are circulating in a patient’s blood which causes an immune reaction which leads to haemolysis. This can occur in transfusion reactions or haemolytic disease of the newborn

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38
Q

What is a haematological complication of prosthetic heart valves?

A

Prosthetic valve haemolysis

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39
Q

How is prosthetic valve haemolysis managed?

A

Monitoring
Oral iron
Blood transfusion
Revision surgery

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40
Q

What causes thalassaemia?

A

Genetic defect in protein chains which make up haemoglobin

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41
Q

What is haemoglobin made up of?

A

2 alpha and 2 beta globin chains

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42
Q

What is the inheritance pattern of thalassaemia?

A

Autosomal recessive

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43
Q

Why can people with thalssaemia have pronounce foreheads and malar eminences?

A

The bone marrow expands to produce extra RBCs for chronic anaemia

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44
Q

What are the signs and symptoms of thalassaemia?

A

Low MCV
Fatigue, pallor
Jaundice, splenomeagly and gallstones
Poor growth and development

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45
Q

What are the investigtions for thalassaemia and what do they show?

A

FBC shows microcytic anaemia
Haemoglobin electrophoresis shows globin abnormalities
DNA testing shows the abnormality

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46
Q

Why should people with thalassaemia have regular checks of ferritin levels?

A

To ensure they are not suffering with iron overload.

Iron overload occurs in thalassaemia as a result of faulty creation of red blood cells, recurrent transfusions and increased absorption of iron in response to the anaemia.

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47
Q

What is the management for alpha thalassaemia?

A

Monitoring FBC
Blood transfusions
Splenectomy
Bone marrow transplant

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48
Q

What are the signs of thalassaemia major?

A

Severe microcytic anaemia
Splenomegaly
Bone deformities

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49
Q

What are the 3 classes of beta thalassaemia?

A

Thalassaemia minor
thalassaemia intermedia
Thalassaemia major

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50
Q

What is the management of thalassaemia major?

A

Tranfusions
Iron chelation
Splenectomy
bone marrow transplant can be curative

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51
Q

What is the variant of haemoglobin which is present in sickle cell anaemia?

A

haemoglobin S

52
Q

What is the inheritance pattern of sickle cell anaemia?

A

Autosomal recessive

53
Q

What is the difference between sickle cell anaemia and sickle cell disease?

A

One copy of HbS codes for the sickle cell trait, 2 copies means sickle cell anaemia

54
Q

What is the sickle cell gene protective against?

A

Malaria

55
Q

When is sickle cell disease tested for?

A

On the newborn screening heel prick test at 5 days of age

56
Q

What are some complications of sickle cell disease?

A
Anaemia
Stroke
Infection
Avascular necrosis
Pulmonary hypertension
Priapism
CKD
Sickle cell crisis
Acute chest syndrome
57
Q

What is the management of sickle cell disease?

A

Antibiotic prophylaxis
Hydroxycarbamide (stimulates production of fetal haemoglobin)
Blood transfusion
Bone marrow transplant

58
Q

What is the management for a sickle cell crisis?

A
Admit
Keep warm
Treat infection
IV fluids
Analgesia
Aspirate penis in priapism
59
Q

What are the features of vaso-occulsive crisis? (also known as painful crisis)

A
Distal ischaemia
Dehydration 
Raised haematocrit
Pain
Fever
60
Q

What is splenic sequestion crisis?

A

A complication of sickle cell disease. RBCs block blood flow in the spleen which leads to an enlarged and painful spleen. It can result in severe anaemia and circulatory collapse

61
Q

What commonly triggers aplastic crisis?

A

Parvovirus B19

62
Q

What are the diagnostic signs and symptoms of acute chest syndrome?

A

Fever or respiratory symptoms WITH new infiltrates seen on CXR

63
Q

What is the management of acute chest syndrome?

A

Antibiotics
Blood transfusion
Incentive spirometry
Artificial ventilation

64
Q

What is pancytopenia?

A

anaemia, leukopenia and thrombocytopenia

65
Q

What ages do the 4 types of leukaemia affect?

A

Under 5 and Over 45= ALL
Over 55=CLL
Over 65= CML
Over 75= AML

66
Q

What are the symptoms of leukaemia?

A

Non-specific

Fatigue, fever, pallor, failure to thrive, petechiae, abnormal bleeding, lymphadenopathy, hepatosplenomegaly

67
Q

What are the main investigations for leukaemia?

A

Urgent FBC
Blood film
Bone marrow biopsy

68
Q

What is the pathophysiology of ALL?

A

Acute proliferation of B lymphocytes. Excessive production of these cells causes them to replace other cells leading to pancytopenia

69
Q

What is on a blood film in ALL?

A

Blast cells

70
Q

Which other disease is ALL associated with?

A

Down’s syndrome

71
Q

Which 2 types of leukaemia are associated with philadelphia chromosome translocation?

A

ALL and CML

72
Q

What is it called when CLL transforms into high-grade lymphoma?

A

Richter’s transformation

73
Q

What is seen on blood film in CLL?

A

Smear or smudge cells

74
Q

What are the 3 phases of CML?

A

Chronic, accelerated and blast phase. Chronic lasts around 5 years and shows high WCC. Accelerated phase is when abnormal blast cells take up a high proportion of cells in the bone marrow. The blast phase is when blasts are >30% of blood and is often fatal

75
Q

What is seen on a blood film in AML?

A

Auer rods

76
Q

How is leukaemia generally managed?

A

Chemotherapy
Steriods
Bone marrow transplant

77
Q

What is tumour lysis syndrome?

A

There is a release of uric acid from cells which are being destroyed by chemotherapy

78
Q

What is the management of tumour lysis syndrome?

A

Allopurinol and rasburicase to reduce the high uric acid levels

79
Q

Which ages does Hodgkin’s lymphoma affect?

A

Has a bimodal age distribution between 20 and 75 years

80
Q

What are the risk factors for Hodgkin’s lymphoma?

A

HIV
EBV
Automimmune conditions
Family history

81
Q

What is the presentation of Hodgkin’s lymphoma?

A

Lymphadenopathy
Pain in lymph nodes when drinking alcohol
Fever, Weight loss, Night sweats

82
Q

Investigations for hodgkin’s lymphoma?

A

Lactate dehydrogenase is raised

Lymph node biopsy is key diagnostic test

83
Q

What is found on lymph node biopsy in Hodgkin’s lymphoma?

A

Reed-sternberg

84
Q

Which staging system is used in Hodgkin’s lymphoma?

A

Ann Arbor staging

85
Q

What is the management Hodgkin’s lymphoma?

A

Chemotherapy and radiotherapy

86
Q

What is the name of the monoclonal antibody which can be used to treat non-hodgkin’s lymphoma?

A

Rituximab

87
Q

Which cells is myeloma a cancer of?

A

Plasma cells

88
Q

What are plasma cells?

A

B lymphocytes which have become activated to produce a certain antibody

89
Q

What can be found in the urine of myeloma patients?

A

Bence jones proteins

90
Q

What causes myeloma bone disease?

A

Increased osteoclast activity and decreased osteoblast activity

91
Q

Where does myeloma bone disease usually affect?

A

skull, spine, long bones and ribs

92
Q

what metabolic effect does myeloma bone disease?

A

Causes hypercalcaemia

93
Q

What are the 4 main features of myeloma?

A
CRAB
Calcium is elevated
Renal failure
Anaemia
Bone lesions/ pain
94
Q

What are the main investigations for myeloma?

A

FBC (low WCC)
Calcium(++++)
ESR (+)
Plasma viscosity (+)

Followed by BLIP
B-bence jones
L-serum free Light chain assay
I- serum Immunoglobulins
P- serum Protein electrophoresis
95
Q

What is necessary to confirm the diagnosis of myeloma

A

Bone marrow biopsy

96
Q

What can be seen on an x-ray of myeloma?

A

Punched out lesions
Lytic lesions
Raindrop skull

97
Q

What is the management of myeloma?

A

Chemo with: borteazomid, thalidomide and dexamethasone

Stem cell transplant

Bisphosphonates for bone disease

98
Q

What are the three main types of myeloproliferative disorder?

A

primary myelofibrosis
Polycythaemia vera
Essential thrombocythaemia

99
Q

What is ruxolitnib?

A

JAK2 inhibitor

100
Q

What is myelofibrosis?

A

Proliferation of a cell line leads to fibrosis of bone marrow

101
Q

What are the main signs of polycythaemia vera?

A

Conjunctival pletheroa
ruddy complexion
splenomegaly

102
Q

What is the management of polycythaemia vera?

A

Venesection
Aspirin
Chemo

103
Q

What is myelodyplastic syndrome?

A

Myeloid bone marrow cells not maturing properly and therefore not producing healthy blood cells. Causes anaemia, neutropenia and thrombocytopenia

104
Q

What is there often on the blood film in myelodysplastic syndrome?

A

blasts

105
Q

What is the management of myelodysplastic syndrome?

A

Supportive transfusions
Chemo
Stem cell transplant

106
Q

What can thrombocytopenia present with?

A

Bruising, nosebleeds, bleeding gums, heavy periods, blood in urine or stools

107
Q

What are the 4 main differentials for prolonged bleeding?

A

Thrombocytopenia
haemophillia A and B
Von Willebrand disease
DIC

108
Q

What is the management of ITP

A

Prednisolone
IV immunoglobulins
Rituximab
Splenectomy

109
Q

What is the inheritance pattern of von willebrand disease?

A

Autosomal dominant

110
Q

What is the presentation of vonwillebrand disease?

A

Bleeding gums
epistaxis
menorrhagia
Heavy bleeding during operations

111
Q

What is the management of von willebrand disease

A

does not require day to day treatment. Management is in response to major bleeding or trauma.
Desmopressin (stimulates release of VWF)
VWF can be infused
Factor VIII infused

112
Q

Which factor is there is deficiency of in Haemophillia A?

A

VIII

113
Q

Which factor is there is deficiency of in Haemophillia B?

A

IX

114
Q

What is the inheritance pattern of haemophillia?

A

X linked recessive

115
Q

What are signs and symptoms of haemophillia?

A

Spontaneous haemorrhage
Haemoarthritis
Haematuria

116
Q

What is the management of haemophillia?

A

Infusion of VIII or IX

117
Q

Which syndrome is the main risk factor which predisposes patients to getting clots?

A

Anti-phospholipid syndrome

118
Q

What is a contraindication for compression stockings?

A

Peripheral arterial disease

119
Q

What are the symptoms of DVT?

A
Calf swelling
Dilated superficial veins
Tenderness to the calf
Oedema
Colour changes to the leg
120
Q

Which score is used to predict the likelihood of a patient having a DVT or PE?

A

Well’s score

121
Q

What is the diagnostic test for DVT?

A

ultrasound doppler of the leg

122
Q

What is the diagnostic tes for PE?

A

CT pulmonary angiogram (ventilation perfusion scan if contrindicated)

123
Q

What is the initial management of DVT?

A

Treatment dose of LMWH

124
Q

When are inferior vena cava filters used?

A

In cases of recurrent PE or those who are unsuitable for anticoagulation

125
Q

What is Budd-Chiari syndrome?

A

When a blood clot develops in the hepatic vein, blocking the outflow of blood

126
Q

What does Budd-Chiari syndrome present with?

A

Abdominal pain, hepatomegaly, ascites