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
What inheritance pattern does hereditary spherocytosis have?
Autosomal dominant
26
How does hereditary spherocytosis present?
Jaundice, gallstones, splenomegaly and aplastic crisis
27
What is seen on blood film in hereditary spherocytosis?
Spherocytes
28
What is the treatment for hereditary spherocytosis?
Folate supplementation, splenectomy
29
What inheritance pattern is hereditary elliptocytosis?
Autosomal dominant
30
What inheritance pattern is G6PD deficiency?
X-linked recessive
31
What can trigger a G6PD deficiency crisis?
Infections, medications and fava beans
32
What does G6PD deficiency present with?
Jaundice (usually in the neonatal period), gallstones, anaemia, splenomegaly
33
What is seen on blood film in G6PD deficiency?
Heinz bodies
34
What is the diagnostic test for G6PD deficiency?
G6PD enzyme assay
35
Which drugs can precipitate a G6PD crisis?
Primaquine (antimalarial), ciprofloxacin, sulfonylureas, sulfasazine
36
What is the management of autoimmune haemolytic anaemia?
Blood transfusion Prednisolone Rituximab Splenectomy
37
What is alloimmune haemolytic anaemia? When does this occur?
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
38
What is a haematological complication of prosthetic heart valves?
Prosthetic valve haemolysis
39
How is prosthetic valve haemolysis managed?
Monitoring Oral iron Blood transfusion Revision surgery
40
What causes thalassaemia?
Genetic defect in protein chains which make up haemoglobin
41
What is haemoglobin made up of?
2 alpha and 2 beta globin chains
42
What is the inheritance pattern of thalassaemia?
Autosomal recessive
43
Why can people with thalssaemia have pronounce foreheads and malar eminences?
The bone marrow expands to produce extra RBCs for chronic anaemia
44
What are the signs and symptoms of thalassaemia?
Low MCV Fatigue, pallor Jaundice, splenomeagly and gallstones Poor growth and development
45
What are the investigtions for thalassaemia and what do they show?
FBC shows microcytic anaemia Haemoglobin electrophoresis shows globin abnormalities DNA testing shows the abnormality
46
Why should people with thalassaemia have regular checks of ferritin levels?
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.
47
What is the management for alpha thalassaemia?
Monitoring FBC Blood transfusions Splenectomy Bone marrow transplant
48
What are the signs of thalassaemia major?
Severe microcytic anaemia Splenomegaly Bone deformities
49
What are the 3 classes of beta thalassaemia?
Thalassaemia minor thalassaemia intermedia Thalassaemia major
50
What is the management of thalassaemia major?
Tranfusions Iron chelation Splenectomy bone marrow transplant can be curative
51
What is the variant of haemoglobin which is present in sickle cell anaemia?
haemoglobin S
52
What is the inheritance pattern of sickle cell anaemia?
Autosomal recessive
53
What is the difference between sickle cell anaemia and sickle cell disease?
One copy of HbS codes for the sickle cell trait, 2 copies means sickle cell anaemia
54
What is the sickle cell gene protective against?
Malaria
55
When is sickle cell disease tested for?
On the newborn screening heel prick test at 5 days of age
56
What are some complications of sickle cell disease?
Anaemia Stroke Infection Avascular necrosis Pulmonary hypertension Priapism CKD Sickle cell crisis Acute chest syndrome
57
What is the management of sickle cell disease?
Antibiotic prophylaxis Hydroxyurea (stimulates production of fetal haemoglobin) Blood transfusion Bone marrow transplant
58
What is the management for a sickle cell crisis?
Admit Keep warm Treat infection IV fluids Analgesia Aspirate penis in priapism
59
What are the features of vaso-occulsive crisis? (also known as painful crisis)
Distal ischaemia Dehydration Raised haematocrit Pain Fever
60
What is splenic sequestion crisis?
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
What commonly triggers aplastic crisis?
Parvovirus B19
62
What are the diagnostic signs and symptoms of acute chest syndrome?
Fever or respiratory symptoms WITH new infiltrates seen on CXR
63
What is the management of acute chest syndrome?
Antibiotics Blood transfusion Incentive spirometry Artificial ventilation
64
What is pancytopenia?
anaemia, leukopenia and thrombocytopenia
65
What ages do the 4 types of leukaemia affect?
Under 5 and Over 45= ALL Over 55=CLL Over 65= CML Over 75= AML
66
What are the symptoms of leukaemia?
Non-specific Fatigue, fever, pallor, failure to thrive, petechiae, abnormal bleeding, lymphadenopathy, hepatosplenomegaly
67
What are the main investigations for leukaemia?
Urgent FBC Blood film Bone marrow biopsy
68
What is the pathophysiology of ALL?
Acute proliferation of B lymphocytes. Excessive production of these cells causes them to replace other cells leading to pancytopenia
69
What is on a blood film in ALL?
Blast cells
70
Which other disease is ALL associated with?
Down's syndrome
71
Which 2 types of leukaemia are associated with philadelphia chromosome translocation?
ALL and CML
72
What is it called when CLL transforms into high-grade lymphoma?
Richter's transformation
73
What is seen on blood film in CLL?
Smear or smudge cells
74
What are the 3 phases of CML?
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
What is seen on a blood film in AML?
Auer rods
76
How is leukaemia generally managed?
Chemotherapy Steriods Bone marrow transplant
77
What is tumour lysis syndrome?
There is a release of uric acid from cells which are being destroyed by chemotherapy
78
What is the management of tumour lysis syndrome?
Allopurinol and rasburicase to reduce the high uric acid levels
79
Which ages does Hodgkin's lymphoma affect?
Has a bimodal age distribution between 20 and 75 years
80
What are the risk factors for Hodgkin's lymphoma?
HIV EBV Automimmune conditions Family history
81
What is the presentation of Hodgkin's lymphoma?
Lymphadenopathy Pain in lymph nodes when drinking alcohol Fever, Weight loss, Night sweats
82
Investigations for hodgkin's lymphoma?
Lactate dehydrogenase is raised Lymph node biopsy is key diagnostic test
83
What is found on lymph node biopsy in Hodgkin's lymphoma?
Reed-sternberg
84
Which staging system is used in Hodgkin's lymphoma?
Ann Arbor staging
85
What is the management Hodgkin's lymphoma?
Chemotherapy and radiotherapy
86
What is the name of the monoclonal antibody which can be used to treat non-hodgkin's lymphoma?
Rituximab
87
Which cells is myeloma a cancer of?
Plasma cells
88
What are plasma cells?
B lymphocytes which have become activated to produce a certain antibody
89
What can be found in the urine of myeloma patients?
Bence jones proteins
90
What causes myeloma bone disease?
Increased osteoclast activity and decreased osteoblast activity
91
Where does myeloma bone disease usually affect?
skull, spine, long bones and ribs
92
what metabolic effect does myeloma bone disease?
Causes hypercalcaemia
93
What are the 4 main features of myeloma?
CRAB Calcium is elevated Renal failure Anaemia Bone lesions/ pain
94
What are the main investigations for myeloma?
FBC: anaemia Blood film U&E: renal failure & hypercalcaemia Bence jones in the urine Bone marrow aspiration to look for plasma cells Whole body MRI to look for bone lesions
95
What is necessary to confirm the diagnosis of myeloma
Bone marrow biopsy
96
What can be seen on an x-ray of myeloma?
Punched out lesions Lytic lesions Raindrop skull
97
What is the management of myeloma?
Chemo with: borteazomid, thalidomide and dexamethasone Stem cell transplant Bisphosphonates for bone disease
98
What are the three main types of myeloproliferative disorder?
primary myelofibrosis Polycythaemia vera Essential thrombocythaemia
99
What is ruxolitnib?
JAK2 inhibitor
100
What is myelofibrosis?
Proliferation of megakaryocytes leads to fibrosis of bone marrow
101
What are the main signs of polycythaemia vera?
Conjunctival pletheroa plethoric complexion splenomegaly
102
What is the management of polycythaemia vera?
Venesection Aspirin Chemo
103
What is myelodyplastic syndrome?
Myeloid bone marrow cells not maturing properly and therefore not producing healthy blood cells. Causes anaemia, neutropenia and thrombocytopenia
104
What is there often on the blood film in myelodysplastic syndrome?
blasts
105
What is the management of myelodysplastic syndrome?
Supportive transfusions Chemo Stem cell transplant
106
What can thrombocytopenia present with?
Bruising, nosebleeds, bleeding gums, heavy periods, blood in urine or stools
107
What are the 4 main differentials for prolonged bleeding?
Thrombocytopenia haemophillia A and B Von Willebrand disease DIC
108
What is the management of ITP
Prednisolone IV immunoglobulins Rituximab Splenectomy
109
What is the inheritance pattern of von willebrand disease?
Autosomal dominant
110
What is the presentation of vonwillebrand disease?
Bleeding gums epistaxis menorrhagia Heavy bleeding during operations
111
What is the management of von willebrand disease
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
Which factor is there is deficiency of in Haemophillia A?
VIII
113
Which factor is there is deficiency of in Haemophillia B?
IX
114
What is the inheritance pattern of haemophillia?
X linked recessive
115
What are signs and symptoms of haemophillia?
Spontaneous haemorrhage Haemoarthritis Haematuria
116
What is the management of haemophillia?
Infusion of VIII or IX
117
Which syndrome is the main risk factor which predisposes patients to getting clots?
Anti-phospholipid syndrome
118
What is a contraindication for compression stockings?
Peripheral arterial disease
119
What are the symptoms of DVT?
Calf swelling Dilated superficial veins Tenderness to the calf Oedema Colour changes to the leg
120
Which score is used to predict the likelihood of a patient having a DVT or PE?
Well's score
121
What is the diagnostic test for DVT?
ultrasound doppler of the leg
122
What is the diagnostic tes for PE?
CT pulmonary angiogram (ventilation perfusion scan if contrindicated)
123
What is the initial management of DVT?
DOAC e.g. apixaban
124
When are inferior vena cava filters used?
In cases of recurrent PE or those who are unsuitable for anticoagulation
125
What is Budd-Chiari syndrome?
When a blood clot develops in the hepatic vein, blocking the outflow of blood
126
What does Budd-Chiari syndrome present with?
Abdominal pain, hepatomegaly, ascites