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Phase 2a Annabel > Haematology > Flashcards

Haematology Flashcards

1
Q

What are the 6 main cells to investigate on FBC?

A

RBC
Neutrophils
Lymphocytes
Platelets
Eosinophils
Monocytes

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2
Q

What are RBCs primarily used to diagnose on FBC?

A

Anaemias

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3
Q

What does a neutrophil value of >7.5 x10^4/L indicate?

A

Neutrophilia
Eg. Acute bacterial infection

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4
Q

What does a neutrophil value of <2 x 10^4/L indicate?

A

Neutropenia
Eg. Myeloma or lymphoma

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5
Q

What does a lymphocyte value of >3.5 x 10^4/L indicate?

A

Lymphocytosis
Eg. Chronic infection

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6
Q

What does a lymphocyte value of <1.3 x 10^4/L indicate?

A

Lymphocytopenia

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7
Q

What does a platelet value of >400 x 10^9/L indicate?

A

Thrombocytosis

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8
Q

What does a platelet value of <1.3 x 10^9/L indicate?

A

Thrombocytopenia

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9
Q

When are eosinophils elevated?

A

Parasitic infection

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10
Q

When are monocyte levels elevated?

A

Myelodyplastic syndromes

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11
Q

Define neutrophilia

A

Elevated neutrophil levels

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12
Q

Define neutropenia?

A

Low neutrophil levels

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13
Q

Define lymphocytosis

A

High lymphocyte levels

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14
Q

Define lymphocytopenia

A

Low lymphocyte levels

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15
Q

Define thrombocytosis

A

Elevated platelet levels

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16
Q

Define thrombocytopenia

A

Low platelet levels

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17
Q

What are 3 tests used for a clotting screen?

A

PT/INR (prothrombin time)
APTT (activated partial prothrombin time)
Fibrinogen

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18
Q

What is PT/INR used to investigate?

A

Coagulation speed through extrinsic pathway

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19
Q

How is INR calculated?

A

Patient PT
—————
Reference PT

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20
Q

What is normal INR without anticoagulants?

A

0.8-1.2

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21
Q

What is normal INR on warfarin?

A

2-3

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22
Q

What are 4 factors that may increase INR?

A
  1. Anticoags
  2. Liver disease
  3. Vit K deficiency
  4. DIC (disseminated intravascular coagulation)
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23
Q

What does APTT measure?

A

Coagulation speed through intrinsic pathway

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24
Q

What is the normal PT/INR time?

A

10-13.5s

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25
What is the normal APTT time?
35-45s
26
What are 3 conditions where PT is normal and APTT is prolonged?
Haemophilia A Haemophilia B VWF disease
27
What is the normal fibrinogen -> fibrin time?
12-14s
28
Define anaemia
Low haemoglobin in the blood below the reference for the patients age and sex
29
How long does a RBC live?
120 days
30
Is anaemia a disease?
No- it is a result of an underlying disease
31
What is measured after an anaemia diagnosis?
Mean corpuscular/cell volume (MCV)
32
What are the 3 main classifications of anaemia?
Microcytic Normocytic Macrocytic
33
When should transfusion be considered for anemia?
Hb <70g/L Or Hb <80/L and cardiac comorbidity
34
What are the symptoms of anaemia?
Fatigue Dysponea Headache Dizziness Palpitations
35
What are 4 signs of anaemia?
Pallor Conjunctival pallor Tachycardia Inc. respiratory rate
36
What level indicate microcytic anaemia?
<80
37
What are 5 causes of microcytic anaemia?
TAILS Thalassaemia Anaemia of chronic disease (sometimes!) Iron deficiency (MC) Lead poisoning Sideroblastic anaemia
38
What is the most common cause of anemia worldwide?
Iron deficiency
39
Outline the pathophysiology of iron deficiency anaemia
Insufficiency of iron to produce RBCs
40
What are 4 causes of Fe deficient anaemia?
Blood loss- assume until proven otherwise! Pregnancy Impaired absorption Poor diet
41
What are 3 causes of blood los that may result in Fe deficiency anaemia?
Menorrhagia GI bleeding Hookworm
42
What are 2 causes of impaired absorption that can result in Fe deficiency anaemia?
Coeliac or Chrons (as iron absorbed in GI tract) Low stomach acid
43
When may Fe deficiency occur in infants?
Premature Delayed mixed feeding (breast milk has low iron)
44
What are the symptoms of Fe deficiency anaemia?
General anaemia symptoms Pruritis (itchy) Nails changing - koilonychia Angular stomatitis Atrophic glossitis
45
What is koilonychia?
Spoon shaped nails
46
What is angular stomatitis?
Ulceration at corners of mouth
47
What is atrophic glossitis?
absence of papillae on tongue- looks smooth
48
How is Fe deficiency anaemia diagnosed on FBC and film?
Microcytic and hypochromic (pale) RBC Poikilocytosis and anisocytosis Howell Jolly bodies (nucleated RBC)
49
What is poikilocytosis?
Variation in RBC shape
50
What is anisocytosis?
Variation in RBC size
51
How is Fe deficiency anemia diagnosis confirmed?
Serum ferritin is LOW- reflects low amount of stored iron
52
What other bloods indicate Fe deficiency anaemia?
Low serum Fe High TIBC (total iron binding capacity) Low transferrin saturation Low reticulocytes
53
What should be investigated when Fe deficiency anaemia occurs in older people?
Colonoscopy for GI bleed
54
How is Fe deficiency anemia treated?
Treat underlying cause Oral Fe eg. Ferrous sulphate or ferrous gluconate In extreme cases IV iron
55
What are the side effects of ferrous sulphate?
Black/green stools Nausea Diarrhoea or constipation
56
What are the normal percentages of haemoglobin in an adult human?
HbA = 97% HbA2 (delta) = 2% HbF (foetal) = 1%
57
What are thalassaemias?
Genetic disease of unbalanced Hb synthesis with underproduction of one globin chain
58
What is the normal structure of a Hb?
Haem + 2 alpha + 2 beta
59
How can thalassaemias lead to haemolysis?
Precipitation of imbalanced globin chains in precursors -> cell damage and death of precursors -> precipitation of imbalanced globin chains in mature cells -> haemolysis
60
Where are thalassaemias common?
Areas from Mediterranean to Far East - in areas with prevalent malaria
61
Define beta thalassaemia
Reduced B chain synthesis Excess A chain synthesis
62
Define alpha thalassaemia
Reduced A chain synthesis Excess B chain synthesis
63
Which thalassaemia is most common?
Beta thalassaemia
64
What types of haemoglobin are increased in beta thalassaemia?
HbA2 (delta) HbF (gamma)
65
What type of genetic mutation causes beta thalassaemia?
Autodom recessive Often caused when genes MUTATED on chromosome 11
66
What type of gene mutation causes alpha thalassaemia?
Autodom recessive Often caused by gene DELETIONS on chromosome 16
67
What are the 3 categories of beta thalassaemia?
B- thalassaemia minor B- thalassaemia intermedia B- thalassaemia major
68
What type of B-thalassaemia is a carrier heterozygous state?
B-thalassaemia minor
69
What are the symptoms of B-thalassaemia minor?
Asymptomatic Mild or absent anaemia Hypochromic (pale) RBC
70
How is B-thalassaemia minor distinguished from iron deficiency anaemia?
Serum ferritin and iron stores are normal May have slightly raised HbA2 and usually raised HbF
71
Outline the pathophysiology of B-thalassaemia intermedia
reduced + absent beta genes OR Reduced + reduced beta genes
72
What are the symptoms of B-thalassaemia intermedia?
Symptomatic with moderate anaemia- doesn’t require regular transfusions Splenomegaly Recurrent leg ulcers Gallstones Bone deformities
73
Outline the pathophysiology of B-thalassaemia minor
Normal + reduced beta OR Normal + absent beta
74
Outline the pathophysiology of B-thalassaemia major
Absent + absent beta genes FULL autorecessive
75
When does B-thalassaemia major present?
In first year of life
76
What are the symptoms of B-thalassaemia major?
Failure to thrive + bacterial infections Severe anaemia at 3-6 months old “Chipmunk” face - bone expansion especially around cheekbones and teeth protrude Extramedullary haematopoesis -> hepatosplenomegaly
77
How is B-thalassaemia major diagnosed?
Microcytic Large and small pale RBCs Normal serum ferritin High reticulocytes
78
What is the gold standard test for thalassaemia diagnosis?
Hb electrophoresis (testing for different types of haemoglobin)
79
How is B-thalassaemia major treated?
Transfusion every 2-4 weeks Iron chelating agents to prevent iron overload Large dose of ascorbic acid to inc. iron in wee Marrow transplant if severe Folic acid
80
What are 2 iron chelating agents?
Oral deferiprone SC desderrioxamine
81
What are the side effects of iron chelating agents?
Pain Deafness Cataracts Retinal damage
82
What are long term complications of transfusion?
Progressive increase in body iron load - deposited in liver and spleen -> cirrhosis - deposited in endocrine glands and heart -> diabetes, hypocalcaemia, death
83
What is HBH made from?
4 beta chains
84
What are the symptoms of a single gene A-thalassaemia?
Normal bloods- carrier
85
What are the symptoms of 2 gene A-thalassaemia deletion?
Microcytosis with/without mild anaemia
86
Outline the pathophysiology of 3 gene deletion A-thalassaemia?
Severe reduction in alpha chain synthesis -> HbH disease
87
What are the symptoms of 3 gene deletion A-thalassaemia?
Moderate anaemia Splenomegaly
88
Outline the pathophysiology of 4 gene deletion A-thalassaemia
Deletion of both genes on both chromosomes -> ONLY HbH Bart’s is present
89
What is the prognosis of 4 gene A-thalassaemia deletion?
Incompatible with life - Babies can die in uterine, stillborn or die very shortly after birth
90
How does 4 gene deletion A-thalassaemia present?
Pale Oedematous Hydrous fetalis - enormous liver and spleen
91
Define sideroblastic anaemia
Defective Hb synthesis within mitochondria causing iron to be stuck in mitochondria
92
What causes sideroblastic anaemia?
X-linked B6 deficiency Idiopathic
93
Outline the pathophysiology of sideroblastic anaemia
Ineffective erythropoiesis -> inc. Fe absorption -> deposition in RBC
94
How would sideroblastic anaemia appear on a blood test?
Ringed sideroblastics High serum ferritin High serum iron
95
How is sideroblastic anaemia treated?
Hereditary may respond to pyridoxine (B6)
96
Define anaemia of chronic disease
Anaemia secondary to chronic disease If body is ill, bone marrow will be too -> anaemia
97
Describe the MCV of anaemia of chronic disease
Usually normocytic but can be microcytic
98
What is the most common type of anaemia in hospital patients?
Anaemia of chronic disease
99
What are 5 conditions associate with anaemia of chronic disease?
TB Chrons Rheumatoid arthritis SLE Malignant disease
100
Outline the pathophysiology of anaemia of chronic disease
Decreased Fe release from marrow -> inadequate erthyropoetin response (drives RBC production) -> decreased RBC survival
101
What are the symptoms of anaemia of chronic disease?
Fatigue, headaches Dysponea Angina (CHD) Anorexia
102
How is anaemia of chronic disease diagnosed?
Low serum Fe Low TIBC
103
When may RBCs be microcytic in anaemia of chronic disease?
Rheumatoid arthritis Chrons
104
How is anaemia of chronic disease treated?
Treat underlying cause Erythropoietin can raise haemoglobin level
105
What MCV is normocytic?
80-95
106
What are the 2 categories of normocytic anaemia?
Haemolytic Non- Haemolytic
107
What are 5 types of haemolytic normocytic anaemia?
Sickle cell Hereditary spherocytosis G6PD deficiency Malaria Autoimmune haemolytic
108
What are 2 types of non-haemolytic normoplastic anaemias?
CKD Aplastic
109
What is an example of a RBC membrane defect?
Hereditary spherocytosis
110
Define hereditary spherocytosis
Autodom condition affecting the RBC membrane via causing a spectrin deficiency
111
Outline the pathophysiology of hereditary spherocytosis
RBC loses part of membrane -> Na+ moves out -> Sa/V ratio decreases -> RBCs become spherocytic -> more rigid -> cant pass through spleen so become trapped and destroyed via haemolysis
112
What are the symptoms of hereditary spherocytosis?
General anaemia Neonatal jaundice Splenomegaly Can have gallstones
113
How is hereditary spherocytosis diagnosed?
Spherocytes and reticulocytes Raised serum bilirubin and urinary urobilinogen Negative Coomb’s test (rules out autoimmune)
114
How is hereditary spherocytosis treated?
Folate supplements+ splenectomy
115
Define G6PD deficiency
Deficiency of glucose-6-phosphate dehydrogenase
116
What causes G6PD deficiency?
Heterogenous X linked- affects men primarily but can affect women via lyonisation
117
What countries are most affected by G6PD deficiency?
Africa Mediterranean Middle East SE Asia
118
Outline the pathophysiology of G6PD deficiency?
G6PD is vital for providing NADPH which can protect the RBC from oxidative damage -> reduced = more susceptible to damage -> shorter lifespan
119
What are the symptoms of G6PD deficiency?
Usually asymptomatic Can cause HAEMOLTIC/OXIDATIVE CRISIS
120
What can cause oxidative/haemolytic crisis?
Aspirin Antimalarials Antibacterial Fava beans can make it worse!
121
What is oxidative crisis/stress?
disturbance in the balance between the production of reactive oxygen species (free radicals) and antioxidant defenses
122
How is G6PD deficiency diagnosed?
Blood count normal between attacks In attacks: - Irregularly contracted cells - Bite cells and Heinz bodies - Increased reticulocytes - Low G6PD right after the attack
123
How is G6PD treated?
Stop offending drugs or fava beans Blood transfusion to save life Splenectomy isn’t usually helpful
124
What are the symptoms of an oxidative crisis?
Rapid anaemia Jaundice
125
What are the 2 types of autoimmune haemolytic anaemia?
Cold and warm subtypes
126
What is the most common subtype of autoimmune haemolytic anaemia?
Warm
127
Outline the pathophysiology of autoimmune haemolytic anaemia
Autoantibodies bind to RBCs and cause haemolysis
128
How is autoimmune haemolytic anaemia diagnosed (dd = hereditary spherocytosis)?
Positive direct Coombs test
129
What are 2 non-haemolytic anaemia?
CKD Aplastic anaemia
130
Why does anaemia occur in CKD?
Fall in erythropoietin
131
What are the 3 characteristics of CKD anaemia?
Normocytic Chronic Decreased reticulocytes
132
How is autoimmune haemolytic anaemia treated?
Blood transfusion Prednisolone Rituximab (monoclonal Ab against B cell) Splenectomy
133
Define aplastic anaemia
Rare stem cell disorder of pancocytopenia (reduction of blood cells) and aplasia of bone marrow- essentially bone marrow stops making haemopoetic stem cells
134
What are the causes of aplastic anaemia?
Idiopathic Inherited eg. fanconis anaemia Benzene and sniffing glue Chemotherapy Antibiotics Infection: EBV, HIV, TB
135
Outline the pathophysiology of aplastic anaemia
Reduction in erythropoetic cells and fault in remaining cells -> bone marrow can not be repopulated -> deficiency in major blood cells
136
What are the symptoms of aplastic anaemia?
Because of RBC, WBC and platelet deficiency - Anaemia - Increased infection - Bleeding - Bleeding gums and blood blisters in mouth
137
How is aplastic anaemia diagnosed?
FBC: pancocytopenia and low reticulocytes Bone marrow: hypocellular marrow and increased fat spaces
138
How is aplastic anaemia treated?
Treat underlying cause Broad spectrum antibiotics RBC and platelet transfusion Bone marrow transplants
139
When may a patient with aplastic anaemia need immunosuppressive therapy?
>40 <40 with severe disease and no HLA sibling donor Transfusion dependent
140
What is used for immunosuppressive treatment in aplastic anaemia?
Antithymocyte globulin (ATG) Ciclosporin
141
Define sickle cell anemia
Autosomal recessive single gene defect in beta chain of haemoglobin
142
What gene is affected by sickle cell anaemia?
HBB
142
Who is primarily affected by sickle cell anaemia?
African - Also India, Middle East and Southern Europe
143
What mutation causes sickle cell anaemia?
Single base mutation of GAG -> GTG which causes an amino acid substitution from glutamic acid -> valine on chromosome 11
144
What is sickle cell haemoglobin known as?
HbS
145
What are the characteristics of HbS?
Flexibility is reduced and cells become rigid, and eventually they will become irreversibly sickled
146
What are the affects of sickling on haemoglobin?
Shortened RBC survival -> haemolysis Impaired passage through micro circulation -> obstruction of small vessels and tissue infarction
147
Why may patients with sickle cell anaemia feel well?
The cells release their oxygen more readily than normal RBCs usually!
148
What are the symptoms of heterozygous sickle cell anaemia?
Usually none! Can experiments hypoxia in unpressurised plane or under anaesthetic
149
What are the general symptoms of homozygous SS anaemia?
Dactylitis(hands and feet swelling episode) Anaemia Jaundice Splenomegaly Delayed puberty
150
What are the symptoms of SSA vaso-occlusive crisis?
Dactylitis due to vaso-occlusion of small vessels in children Pain in long bones in adults CNS infarctions in children -> stroke, seizures
151
How often does vaso-occlusive crisis occur?
Depends on patient- can be every day to once a year
152
What is acute chest syndrome?
Vaso-occlusive crisis of the pulmonary vasculature
153
What is the most common cause of disease in adults with SSA?
Pulmonary hypertension and chronic lung disease
154
What causes acute chest syndrome?
Infection Fat embolism Pulmonary infarction (cells get stuck in vessel)
155
What are 3 symptoms of acute chest syndrome?
SOB CP Hypoxia
156
What causes pulmonary hypertension in patients with SSA?
Only 10% Repeated chest crisis and repeated thromboembolism + intravascular haemolysis
157
What is aplastic crisis?
Occurs in SSA Rapid fall in haemoglobin due to failure of erythopoesis
158
What causes aplastic crisis?
Parvovirus Erythrovirus B19
159
What is splenic sequestration?
Sickle cells become trapped in spleen
160
What are the complications of splenic sequestration?
Fibrotic spleen Painful enlargement In children need URGENT transfusion
161
How is SSA diagnosed?
Heel prick at birth FBC: 60-80Hb and raised reticulocytes Positive sickle solubility test
162
What is the gold standard diagnosis for SSA?
Hb electrophoresis - Shows 80-95% HbS and NO HbA
163
How is sickle cell anaemia treated?
Hydroxycarbamide (raises HbF) Avoid precipitating factors Stem cell transplant
164
How are complicated attacks of SSA treated?
IV fluids Analgesia Oxygen and antibiotics Transfusion
165
What MCV value indicates macrocytic anaemia?
95 <
166
What are the 2 subcategories of macrocytic anaemia?
Megaloblastic Non-megaloblastic
167
What are 2 types of megaloblastic anaemia?
B12 deficiency Folate deficiency
168
What are 3 causes of non-megaloblastic anaemia?
Hypothyroidism Alcohol excess Liver disease
169
What is the main cause of B12 deficiency anaemia?
Pernicious anaemia
170
Where is B12 found?
NOT PLANTS Meat Fish Dairy
171
How is B12 absorbed?
Binds to transcobalamin-1 in mouth -> binds to intrinsic factor -> absorbed in terminal ileum
172
What produces intrinsic factor?
Parietal cells in the stomach
173
Where is B12 absorbed?
Terminal ileum
174
Outline the pathophysiology of B12 deficiency anaemia
Lack of B12 -> lack of thymidine -> lack of DNA synthesis -> delayed nuclear maturation -> large and decreased RBCs
175
Why does B12 deficiency anaemia take years to manifest?
Body’s stores last around 4 years
176
Define pernicious anaemia
Autoimmune disorder where the parietal cells are attacked -> atrophic gastritis -> loss of intrinsic factor production -> B12 malabsorption
177
What are 3 causes of B12 anaemia?
Diet Malabsorption Pernicious anaemia
178
What are the risk factors of B12 anaemia?
Elderly Female Vegan Fair hair and blue eyes Blood group A Thyroid and Addisons
179
What are the symptoms of B12 deficiency anaemia?
General anaemia Lemon-yellow skin (pallor + jaundice) Glossitis Angular stomatitis
180
How is pernicious anaemia (specifically) diagnosed?
Parietal cell antibodies Intrinsic factor antibodies- 50% (GS)
181
When do neurological symptoms appear in pernicious anaemia?
VERY low levels of B12
182
What are some neurological symptoms of B12 deficiency anaemia?
Symmetrical parasthesia in fingers and toes Loss of vibration sense and proprioception Dementia and psychiatric problems
183
How is B12 deficiency anaemia treated?
If not pernicious treat cause Malabsorption = injections Dietary = oral B12 IM hydrocycolbamin
184
How does B12 deficiency anaemia show up on blood film?
Macrocytic Megaloblasts Low reticulocytes
185
What are megaloblasts?
Hypersegmented nucleated neutrophils (6+) and basophilic
186
What is folate found in?
Green veg - Spinach - broccoli - Nuts - yeast - Liver
187
Why does folate deficiency manifest much quicker than B12 deficiency anaemia?
Body stores only last 4 months
188
Where is folate absorbed?
Duodenum and proximal jejunum
189
Outline the pathophysiology of folate deficiency anaemia
Needed for DNA synthesis so folate deficiency -> impaired DNA synthesis -> delayed nuclear maturation -> large RBCs and reduced production
190
How can folate deficiency affect foetal development?
Can cause neural tube defects eg. Spina bifida
191
What are 4 causes of folate deficiency?
MC: poor intake eg. Poverty, alcoholics, elderly Malabsorption Increased demand eg. Pregnancy Anti folate drugs
192
What are 2 drugs that can cause folate deficiency?
Methotrexate Trimethoprim
193
What are the signs of folate deficiency anemia on bloods?
Macrocytic cells Oval macrocytes (large RBCs) Hypersegmented neutrophil polymorphs Low serum folate
194
How is folate deficiency anaemia treated?
Folic acid tablets daily for 4 months Give with B12 unless patient is known to have normal B12
195
Why is B12 given alongside folic acid tablets?
In low B12 states, folate can precipitate subacute combined degeneration of the spinal cord
196
How can alcohol cause anaemia?
Toxic to RBCs Depletes folate
197
How can hypothyroidism cause anaemia?
Interference with erythropoietin
198
How can liver disease cause anaemia?
Liver decompensated cirrhosis associated with macrocytic anaemia
199
What are the 2 main types of blood cancer?
Leukaemia Lymphoma
200
What are the 4 main subtypes of leukaemia?
Acute lymphoblastic leukaemia (ALL) Acute myeloid leukaemia (AML) Chronic myeloid leukaemia (CML) Chronic lymphocytic leukaemia (CLL)
201
Define leukaemia broadly
Presence of rapidly proliferating immature blast cells in the bone marrow that are non-functional
202
Outline the basic pathophysiology of leukaemia
1. Cells rapidly proliferate but have no function -> energy wasting -> less energy to make useful cell 2. Rapid replication takes up lots of space in the bone marrow -> no space or nutrients for other cells to grow 3. Normal cells not made so non functional cells enter bloodstream
203
What cells are proliferated in AML?
Myeloblasts
204
What 3 cells do myeloblasts give rise to?
Basophils Neutrophils Eosinophils
205
What is the prognosis of untreated AML?
Death in 2 months
206
What is the most common acute leukaemia in adults?
AML
207
What are 4 risk factors of AML?
associated with myelodysplastic syndromes Down’s syndrome Age Chemo and radiation
208
What are the clinical presentations of AML?
BM failure: anaemia, infections and bleeding Hepatomegaly and Splenomegaly Gum hypertrophy Thrombocytopenia
209
How is AML diagnosed?
Bone marrow biopsy to differentiate from ALL ( min 20% blasts) Auer rods Leukocytosis Blood film morphology- min 20% (GS)
210
What is given to people undergoing chemo to prevent tumour lysis syndrome?
Allopurinol Chemo releases uric acid which can accumulate -> malignancy
211
How is AML treated?
Chemo (cytarabine and anthracycline) Blood, platelet and marrow transfusion ATRA for APL subtype Fluids
212
What are the complications of AML?
Infection Common organisms present oddly
213
The proliferation of which cell causes ALL?
Immature lymphoid (lymphoblasts) cells
214
Which cells do immature lymphoid cells give rise to?
B lymphocytes T lymphocytes
215
What is the most common childhood cancer?
ALL
216
Who does ALL most commonly affect?
People aged 2-4
217
What are some risk factors for ALL?
Down’s syndrome Ionising radiation and X-rays during pregnancy Genetics
218
What cells are proliferated in the bone marrow in ALL in adults and children?
Adults = all T cells Children = all B cells
219
What are the symptoms of ALL?
BM failure: anaemia, infection, bleeding Bone pain Hepatosplenomegaly Lymphadenopathy CNS infiltration
220
What is the prognosis of ALL?
Good prognosis! :)
221
How is ALL diagnosed?
FBC: WCC high, **blast cells** on film and in BM TdT Pancytopoenia BM biopsy >20 % (GS) Lumbar puncture for CNS involvement
222
How is ALL treated?
Blood, marrow and platelet transfusion Chemo and allopurinol Neutropenic prophylaxis when in chemo
223
Define chronic myeloid leukaemia
Uncontrolled proliferation of mature myeloid cells
224
Which cells rise from myeloid cells?
Myeloblasts (->eosinophils, basophils, neutrophils) Precursors of: Platelets RBCs Monocytes
225
Outline the epidemiology of CML
Almost exclusively adults Slightly more common in males More than 80% have Philadelphia chromosome
226
What chromosome is affected by Philadelphia chromosome?
22
227
Outline the aetiology of the Philadelphia chromosome
Translocation between chromosome 9 and 22 BCR fuses with ABL -> BCR/ABL -> oncogene Oncogene has tyrosine kinase activity -> stimulates cell division
228
What are the 3 phases of CML?
1. Chronic = little if any symptoms 2. Accelerated phase = increasing symptoms (Splenomegaly, anaemia, thrombocytopenia) 3. Blast transformation = acute leukaemia symptoms + death (over 30% blasts in blood)
229
What are the symptoms of CML?
**MASSIVE Splenomegaly** Symptomatic anaemia gout Hyperviscosity (headaches and thrombotic events)
230
How is CML diagnosed?
High WBC Low Hb Hypercellular BM Karotyping for Philadelphia chromosome (BCR-ABL) (~80%)
231
How is CML treated?
Tyrosine kinase inhibitor - 1st line = imatinib Chemo in blast crisis
232
What is a complication of blast crisis?
Pancytopenia - All cells deficient in blood
233
What can CML progress to if untreated?
AML
234
Define CLL
Chronic proliferation of a single type of well differentiated lymphocyte
235
What cell is most commonly proliferated in CLL?
B-lymphocyte
236
What are the risk factors of CLL?
Older age Male Mutations, trisomies, deletions Can be triggered by pneumonia
237
Outline the pathophysiology of CLL
Failure of cell apoptosis- B cells accumulate
238
What are the symptoms of CLL?
Can be asymptomatic (diagnosed on FBC) Anaemia and prone to infection Hepatosplenomegaly Non tender lymphadenopathy
239
How is CLL diagnosed?
Normal or low Hb Raised WBC (especially lymphocytes) **Smudge cells** on blood film
240
What do smudge cells indicate?
CLL
241
What do Auer rods indicate?
AML
242
What is the Rai staging system used for?
CLL
243
What does a 0 on the rai staging indicate?
Clonal lymphocytosis
244
What does a 1 on Rai staging indicate?
Lymphocytosis and lymphadenopathy
245
What does 2 on Rai staging indicate?
Lymphocytosis and hepatomegaly
246
What does 3 on Rai staging indicate?
Lymphocytosis and anaemia
247
What does 4 on Rai staging indicate?
Lymphocytosis and thrombocytopenia
248
How is symptomatic CLL treated?
Radiotherapy and chemotherapy IVIg (IV Ig)
249
What is a major complication of CLL?
Richters transformation
250
What is Richter’s transformation?
B cells massively accumulates in lymph nodes -> lymphadenopathy -> aggressive non-Hodgkin’s lymphoma
251
What is the prognosis of CLL?
1/3 wont progress 1/3 progress slowly 1/3 progress actively
252
What are the 2 types of lymphoma?
Hodgkin lymphoma Non-Hodgkin lymphoma
253
What differentiates Hodgkins lymphoma from Non-Hodgkins?
Hodgkins has Reed-Sternberg cells Non-Hodgkins doesnt Hodgkins = Neutrophilia Non-Hodgkins = neutropenia
254
What are Reed-Sternberg cells?
“Owl eye” nuclei cells Abnormally large B cells with multiple nuclei and nucleoli - looks like an owl!
255
Define lymphoma
Disorders caused by malignant proliferations of lymphocytes -> accumulate in lymph nodes -> lymphadenopathy -> can enter blood or organ
256
Define lymphadenopathy
Enlarged lymph nodes
257
Outline the epidemiology of Hodgkin’s lymphoma
Peaks in teens and elderly Male
258
What are the risk factors of Hodgkin’s lymphoma?
EBV infection Affected siblings Autoimmune conditions Post transplant
259
What are the symptoms of Hodgkin’s lymphoma?
Enlarged non-tender rubbery superficial lymph nodes Pain after drinking alcohol B symptoms
259
What are the 3 “B symptoms”?
Fever Weight loss Night sweats
259
What is the GS for Hodgkin’s lymphoma diagnosis?
Excision lymph node biopsy - Shows Reed-Sternberg cells
259
What are the 2 types of Hodgkin’s lymphoma?
Classical Hodgkin’s lymphoma (cHL) Nodular lymphocyte predominant Hodgkin’s Lymphoma (NLPHL)
259
What type of cell are visible in cHL?
Reed-Sternberg cells with mirror image nuclei
259
How do the cells appear in NLPHL?
Reed Sternberg variant: popcorn cell
259
What would a Philadelphia chromosome indicate?
CML
260
What are bloods like in Hodgkin’s lymphoma?
Increased LDH Increased ESR Decreased Hb
261
How is Hodgkin’s lymphoma classified?
Ann Arbor classification
262
What does I on Ann Arbor staging indicate?
Confined to a single lymph node region
263
What does II on Ann Arbor staging indicate?
Involvement of 2 or more nodal areas on the same side of the diaphragm (above or below)
264
What does III on Ann Arbor staging indicate?
Involvement of nodes on both sides of the diaphragm
265
What does IV on Ann Arbor staging indicate?
Spread beyond the lymph nodes Eg. Liver or bone marrow
266
What does an A on Ann Arbor staging indicate?
No systemic symptoms other than pruritus
267
Define pruritus
Severe itching of skin
268
What does a B on Ann Arbor staging indicate?
Presence of B symptoms
269
How is Ia and IIa Hodgkin’s lymphoma treated?
Radiotherapy and short courses of chemo (ABVD)
270
How is stage IIa to IVb Hodgkin’s lymphoma treated?
Combination chemo- ABVD A- Adriamycin B- Bleomycin V- Vinblastine D- Dacarbazine
271
What are 4 side effects of radiotherapy?
Increased risks of secondary malignancies IHD Hypothyroidism Lung fibrosis
272
What are 5 side effects of chemotherapy?
Myelosuppression Nausea Alopecia Infection Infertility
273
When does febrile neutropenia occur?
Recent high dose of chemotherapy or on carbimazole
274
What 3 symptoms indicate worse prognosis for Hodgkin’s lymphoma?
B symptoms Low Hb Raised LDH
275
What are the symptoms of febrile neutropenia?
Fever Tachycardia Sweats Tachypnoea
276
How is febrile neutropenia treated?
piperacillin and tazobactam
277
What is the most common type of non-Hodgkin’s lymphoma?
DLBCL- diffuse large B-cell lymphoma
278
What are 3 types of non-Hodgkin’s lymphoma?
DLBCL Burkitts lymphoma Follicular
279
Outline the aetiology of non-Hodgkin’s lymphoma
Strong link between EBV and Burkitts lymphoma Inc. risk with family history Hep C H. Pylori
280
What are the symptoms of non-Hodgkin’s lymphoma?
Superficial symmetrical lymphadenopathy- painless and rubbery B- symptoms Extranodal disease
281
How is non-Hodgkin’s lymphoma diagnosed?
Lymph node excision or BM biopsy- NO Reed-Sternberg cells CT/MRI for staging
282
How is Non-Hodgkin’s lymphoma classified?
Low grade: slowly growing, advanced at presentation High grade: nodal presentation
283
What is the prognosis of low grade non-Hodgkin’s lymphoma?
Incurable but goodish prognosis - median survival 9-11 years
284
What is an example of low grade non-Hodgkin’s lymphoma?
Follicular lymphoma
285
What is an example of high grade non-Hodgkin’s lymphoma?
DLBCL
286
What is the prognosis of high grade non-Hodgkin’s lymphoma?
Aggressive but often curable
287
How is non-Hodgkin’s lymphoma treated?
R-CHOP R- Rituximab C- Cyclophosphamide H- Hydroxy-daunorubicin O- Oncovin/ Vincristine P- Prednisolone
288
What is rituximab?
Monoclonal antibod targeting CD20 from B cell surface
289
What is the typical sign of Burkitts lymphoma?
Jaw lymphadenopathy in children
290
What is the difference between lymphadenopathy in Hodgkin’s and non-Hodgkin’s lymphoma?
Hodgkin’s is painful after alcohol, non-Hodgkin’s unaffected
291
Define febrile neutropenia
Temperature above 38 degrees in a patient with absolute neutrophil count (<1x10^9/L)
292
Define multiple myeloma
Neoplastic monoclonal proliferation of plasma cells
293
What are the 2 most common immunoglobulin excesses in MM?
IgG (55%) IgA (20%)
294
Outline the pathophysiology of MM
Excess of one Ig -> other Ig levels low -> accumulation of malignant cells -> BM failure
295
What are 3 complications of MM?
Bone disease Hypercalcaemia Renal failure
296
Outline the epidemiology of MM?
peaks at 70 years old Afro-carribeans
297
What are the clinical presentations of MM?
OLD CRAB OLD age Calcium elevated Renal failure (raised Ig) Anaemia Bone lytic lesions (back pain)
298
What does Rouleux formation on blood film indicate?
MM
299
What do Bence-jones proteins in urine indicate?
MM
300
How does MM show on bloods?
Normocytic normochromic anaemia Raised ESR **Rouleux formation** Electrophoresis shows “M spike” of proliferated Ig
301
How does MM show on U&Es?
High Ca High alkaline phosphotase **Bence jones protein in urine**
302
How does MM appear on X-ray?
Lytic ‘punched out lesions” - Pepper pot skull Fractures Osteoporosis
303
What 4 criteria are required for MM diagnosis?
Monoclonal protein band in serum or urine Inc. plasma cells on BM biopsy Hypercalcaemia/ renal failure/ anaemia Bone lesions
304
How is MM treated?
Chemo- CTD or VAD Stem cell transplant Biphosphonates Dialysis for real failure
305
What is an example of a Biphosphonate?
Zolendronate
306
What is CTD chemo?
For less healthy people (MM) Cyclophosphamide Thalidomide Dexamethasone
307
What is VAD chemo?
For healthier people (MM) Vincristine Adriamycin Dexamethasone
308
What is the differential diagnosis of MM?
MGUS- mammloid gammopathy of undetermined significance <10% plasma cells Asymptomatic
309
Define polycythaemia
Any increase in RBCs
310
Define erythrocytosis
An increase in RBC mass
311
What are 4 causes of polycythaemia?
Primary: Polycythaemia Vera Secondary: Hypoxia Increased EPO Dehydration
312
Define polycythaemia Vera
Malignant proliferation of a clone derived from one pluripotent stem cell (especially RBCs)
313
Outline the epidemiology of PV
Over 95% have JAK2 mutation >60
314
What is a JAK2 mutation?
Mutation from valine -> phenylalanine on chromosome 9
315
How does JAK2 mutation cause PV?
JAK2 is a cytoplasmic tyrosine kinase that transducers signals such as haemopoietic factors (erythropoietin)
316
What are the 2 major complications of PV?
Hyperviscosity causes: Thrombosis Haemmorage
317
What are the clinical presentations of PV?
Severe itching after hot bath or when warm Visual disturbances and headaches Erythromelalgia Plethoric complexion Hepatosplenomegaly
318
What is erythromelalgia?
Burning sensation in fingers and toes
319
How is PV diagnosed?
Raised WCC and platelets Raised Hb JAK2 mutation on genetic screen
320
How is PV treated?
No cure- aim to maintain normal BC Venesection: removal of 400-500ml of blood a week Chemo in high risk patients - Hydroxycaramide Aspirin
321
What is the function of thrombopoetin?
Stimulates production of platelets by megakaryocytes
322
Where is TPO produced?
Mainly the liver - Liver damage = reduced TPO = reduced platelets
323
What is the function of P2Y12?
Amplifies platelet activation Activates glycoprotein IIb/IIia
324
What activates P2Y12?
ADP
325
What is the function for Gp IIb/IIIa?
Receptor for fibrinogen and vWF Aids platelet adherence and aggregation
326
What are the 3 functions of vWF?
Brings platelets to endothelium Binds platelets Binds to factor VIII for protection
327
Define thrombocytopenia
Deficiency of platelets in the blood
328
What are 2 types of thrombocytopenia?
Immune thrombocytopenia purpura (ITP) - MC Thrombotic thrombocytopenic purpura (TTP)
329
What causes ITP?
Immune destruction of antibody coated platelets by IgG antibodies
330
Who is affected by primary/acute ITP?
Children age 2-6
331
What are the causes/risk factors of primary ITP?
History of recent viral infection - chickenpox (varicella zoster) or measles Can follow immunisation
332
What are the 2 features of primary ITP?
Muco-cutaneous bleeding Sudden self limiting purpura (red or purple spots)
333
Who is affected by secondary/chronic ITP?
Women Autoimmune disease such as SLE, thyroid issues, AHA CLL HIV and Hep C
334
What are the clinical presentations of ITP?
SYSTEMICALLY WELL **Purpuric rash** Easy bleeding Splenomegaly RARE
335
How is ITP diagnosed?
BM: thrombocytopenia with raised megakaryocytes Platelet autoantibodies in ~70%
336
How is ITP treated?
1. Prednisolone and IV IgG 2. Splenectomy or azathioprine
337
What type of drug is azathioprine?
Immunosuppressant
338
Outline the pathophysiology of TTP
Reduction in ADAMTS-13 -> no vWF degradation -> platelet aggregation and deposition -> microthrombi -> Overconsumption of platelets -> thrombocytopenia
339
What are 3 causes of TTP?
Idiopathic Autoimmune Cancer Pregnancy Drugs (quinine)
340
What are the symptoms of TTP?
SYSTEMICALLY UNWELL Florid purpura Fever Fluctuating cerebral dysfunction Haemolytic anaemia and AKI
341
How is TTP diagnosed?
Schistocytes Decreased ADAMTS-13
342
How is TTP treated?
Plasma exchange (remove ADAMTS-13) IV methyprednisolone IV rituximab
343
What is the MC inherited bleeding disorder?
Von Willebrand disease
344
How is haemophilia inherited?
X-linked recessive
345
What does haemophilia A cause?
Factor 8 deficiency
346
What does haemophilia B cause?
Factor 9 deficiency
347
Is haemophilia A or B more common?
A
348
What are the symptoms of haemophilia?
Haemarthrosis- bleeding into joints] Haematoma- bleeding into muscles Spontaneous bleeding
349
How is haemophilia A diagnosed?
Low factor 8 on essay High APTT Normal PT
350
How is haemophilia B diagnosed?
Factor 9 high on essay High APTT Normal PT
351
Which pathway is affected in haemophilia?
Intrinsic
352
How is haemophilia A treated?
Recombinant factor 8 (major bleed) Desmopressin (Minor bleed)
353
How is haemophilia B treated?
Recombinant factor 9
354
What causes Von willebrands disease?
Autosomal dominant mutation of vWF gene on chromosome 12
355
What are the 3 subtypes of Von willebrand disease?
1: low vWF 2: abnormal vWF 3: undetectable vWF
356
What are the symptoms of Von willebrand disease?
Bruising and easy bleeding Epistaxis- nose bleeds Menhorragia
357
How is Von willebrand disease diagnosed?
high APTT normal PT Factor 8 and 9 normal Low vWF
358
How is Von willebrand disease treated?
Desmopressin VWF containing concentrate
359
What does DIC stand for?
Disseminated intravascular coagulation
360
Does DIC occur in isolation?
NO - crisis secondary to clinical disorder or trauma
361
What happens in DIC?
inappropriate activation of the clotting cascades, resulting in thrombus formation and depletion of clotting factors and platelets.
362
What are 3 causes of DIC?
Sepsis Major trauma APL (acute promyelotic leukaemia) Advanced cancer Obstetric complications
363
What are the symptoms of DIC?
Acute illness and shock Bleeding from nose, mouth, venupuncture sites Widespread Bruising
364
How is DIC diagnosed?
Severe thrombocytopenia Decreased fibrinogen Elevated D-dimer Prolonged PT, APTT
365
How is DIC treated?
Treat underlying condition Replace platelet Fresh frozen plasma (FFP)
366
What is tumour lysis syndrome?
Occurs when malignant cell break down and release their contents in chemotherapy
367
What patients are at risk of tumour lysis syndrome?
High grade disease Renal impairment Old age
368
How is tumour lysis syndrome treated?
Prevented by allopurinol Aggressive hydration May need dialysis
369
What are 2 causes of Hyperviscosity syndrome?
High levels of Igs (MM) High cell numbers eg. Leukaemia
370
What are 2 effects of Hyperviscosity syndrome?
Vascular stasis Hypoperfusion
371
What are 3 symptoms of Hyperviscosity syndrome?
Mucosal bleeding Vision changes SOB Neurological disturbances Gum bleeding
372
How is Hyperviscosity syndrome diagnosed?
Plasma viscosity level CT to exclude other signs FBC and Ig levels
373
How is Hyperviscosity syndrome treated?
Hydrated Avoid transfusion Plasmapheresis to remove Igs
374
What causes malaria?
Female anopheles mosquito carries: MC: plasmodium faliparum Also: p.Ovale, P.vivax
375
Outline the epidemiology of malaria
Africa Very young Very old Pregnancy
376
What enters the blood from mosquito saliva that can cause malaria?
Sporozoites
377
What do Sporozoites transform into in hepatocytes?
Merozoites
378
How do merozoites develop in RBCs?
Merozoites -> trophozoites -> schizont -> new Merozoites
379
What are the symptoms of malaria?
RECENT TRAVEL Fever Black water fever Hepatosplenomegaly Convulsions in children
380
How is malaria diagnosed?
Thick and thin blood film
381
How is malaria treated?
Quinine/chloroquine and doxycycline Severe = IV artesunate
382
What are 2 other names for EBV?
Glandular fever Infectious mononucleosis
383
How is EBV spread?
Saliva or bodily fluids
384
How is EBV diagnosed?
Atypical lymphocytes on blood film
385
What are 3 conditions associated with EBV?
Hodgkins Burkitts Nasopharyngeal carcinoma
386
How is EBV treated?
Usually self limiting!
387
How is HIV transmitted?
Bodily fluids: Sexual transmission Sharing needles
388
What are 2 types of HIV?
HIV-1: MC, most virulent HIV-2: LC, less virulent
389
Outline the 6 stages of HIV entering the body
1. HIV binds to Th 2. Endocytoses RNA and enzymes 3. Reverse transcriptase RNA->DNA 4. Integrase viral DNA integrated into host 5. Protein synthesis 6. Viral proteins and RNA exocytose and decrease CD4 and Th cells
390
What are the 4 stages of HIV?
1. Infection: CD4 dip 2. Clinical latency (years) 3. Symptoms 4. AIDS
391
What are the symptoms of HIV?
Fever Diarrhoea Night sweats Minor infections
392
What CD4 values indicate AIDS?
<200/mm^3
393
What are 3 AIDS defining conditions?
CMV - MC PCP (pneumonia) - MC Cytosporidium infection TB Kaposi sarcoma Toxoplasmosis Lymphomas
394
How is HIV diagnosed?
History and anti HIV Ig (ELISA) P24 ab Monitor HIV RNA copies and CD4
395
How is HIV treated?
HAART (highly active antiretroviral therapy) Reverse transcriptase inhibitors
396
Defibe myelodeplastic syndromes
myeloid bone marrow cells fail to mature
397
When are Heinz bodies present?
G6PD deficency Thalassaemia
398
How is symptomatic A-thalassaemia treated?
Bone marrow and blood transfusion Splenectomy
399
What is the difference between acute and chronic leukaemia?
Acute: impaired differentiation of precursor Chronic: excessive proliferation of mature cell
400
What are the symptoms of tumour lysis syndrome?
2 days after high dose chemo Dysuria Abdo pain Weakness
401
How is tumour lysis syndrome diagnosed?
potassium and phosphate are typically raised Low calcium Raised uric acid Electrolyte abnormalities
402
What is the GS for MM diagnosis?
Bone biopsy

Phase 2a Annabel (12 decks)

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