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Haem Flashcards

1
Q

What are 2 types of ITP

A

Primary- no identifiable cause

Secondary- alongside a precipitant or associated condition

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2
Q

What is priapism

A

Extended and painful erection

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3
Q

Where are petechiae most commonly found

A

Lower limbs

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4
Q

Rare treatments for SCD

A

Bone marrow transplant

Gene therapy

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5
Q

Diagnosis of ITP

A

Diagnosis of exclusion so all tests normal except for low plts
No systemic signs
No organomegaly
No lymphadenopathy

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6
Q

Complications of SCD

A 
Avascular necrosis in bones
Stroke
MI
Splenic sequestration
Chronic cholecystisis and gall stones
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7
Q

Signs on examination of ITP

A

No lymphadenopathy
No organomegaly
No other signs
Just petechiae or bruising

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8
Q

What happens when renal pupillae is occluded

A

Necrosis- presents with haematuria and proteinuria

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9
Q

What happens to haptoglobin in intravascular haemolysis

A

Goes down

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10
Q

What make up majority of splenic sequestration cases

A

6 months to 2yrs infants with SCD

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11
Q

Complications of TTP

A

Stroke, MI and any thrombo-embolic events

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12
Q

What is low haptoglobin a sign of

A

Intravascular haemolysis

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13
Q

Definitive test for TTP

A

ADAMTS- 13 activity test- takes a month

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14
Q

Acquired non immune causes of thrombocytopenia

A

Infection
Drugs
Malignancy

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15
Q

Which drugs can cause thrombocytopenia

A

Anti platelets and any blood thinning drugs
Alcohol
Sulfa drugs
Quinine

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16
Q

How does SCD affect the spleen

A

Infarct which can lead to backup of blood in spleen- splenic sequestration

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17
Q

Where is SCD commonly found

A

People of african and South asia descent due to evolutionary protection against malaria

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18
Q

Risk factors for ITP

A

Women of childbearing age- tends to be chronic

People under age of 10 and above 65- tends to be more acute so presents after a viral infection for example

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19
Q

Treatment for SCD

A 
Vaccines 
Anitbiotics to treat any underlying infections
Fluids and oxygen
Opiods for crises
Transfusions
Penicillin prophylaxis
Hydroxyurea
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20
Q

What is name given to heterozygote SCD

A

Sickle cell trait

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21
Q

How does hydroxyurea work

A

Increases production of gamma Hb which composes HbF. HbF doesnt include HbS so reduces sickling

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22
Q

What is danger of splenectomy or dysfunctional spleen

A

Susceptible to encapsulated bacteria as they are normally opsonised and phagocytosed there

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23
Q

Findings of TTP investigations

A

FBC- platelets and RBC low
Blood film- reduced platelets and schistocytes present
Reticulocyte count- raised
Urinalysis- kidney issues common so protein in urine common and U and Es will be pathological
Haptoglobin- down

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24
Q

What are some conditions assocaited with secondary ITP

A

HIV
Hep C
Lupus

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25
Pathophysiology of TTP
Antibodies formed against enzyme that cleaves VwF meaning VwFs circulate without inhibition leading to spontaneous and excessive thrombous formation which cause ischaemia and shearing of RBCs
26
3 factors that increase sickling
Acidosis Dehydration Hypoxia
27
TTP pentad of symptoms
``` Renal disease Microangiopathic haemolytic anaemia Thrombocytopenic purpura Neurological symptoms Fever ```
28
What happens to Hb after RBC breakdown
Binds to haptoglobin
29
Dangers of transfusions
Iron overload | Antibodies produced against future infections
30
What is petechiae
Small red or purple spots on skin or mucosal membranes that indicate small capillary bleeds
31
Risk factors for SCD
Family inheritance
32
What is particular SCD symptom for men
Priapism
33
What is result of splenic infarcts over time
Auto-splenectomy where it fibroses down to become very small
34
Presentation of TTP
Prodrome for a week Neurological symptoms such as confusion, drowsiness, focal symptoms and coma Digestive symptoms including vomiting, diarrorhoea and abdo pain Bleeding signs such as bruising and purpura Fever
35
Define TTP
Microangiopathic haemolytic anaemia and thrombocytopenic purpura coupled.
36
Investigations and findings for ITP
FBC- low platelets and no other abnormalities Blood film- fewer platelets which can be larger in size Bone marrow biopsy- increased megakaryocytes due to need for production, no dysplasia and normal cytogenics
37
Signs of increased inconjugated bilirubin
Scleral icterus Jaundice Gallstones
38
Dactylitis
Swollen joints on the back of hands and feet
39
Investigations of TTP
``` FBC- RBC and Plt Blood film Reticulocyte count Urinalysis and U and Es Haptoglobin ```
40
What is TTP
Thrombotic thrombocytopenic purpura
41
Define sickle cell disease
Autosomal recessive disease in beta chain of Hb resulting in the formation of sickle cell shaped RBCs due to agglutination of Hb
42
Site of extramedullary haematopoeisis
Liver- hepatomegaly
43
Common symptoms of SCD
Painful crises anywhere from legs to chest Swollen dorsa of hands for feet joints- dactylistis DUE TO VASO-OCCLUSIVE CRISES
44
Risk factors for TTP
Black Age 20-50 Stresses on body such as infection, pregnancy and maligancy
45
What does excessive recycling of haptoglobin bound to Hb lead to
Increased unconjugated bilirubin
46
Acquired immune causes of thrombocytopenia
ITP TTP AI disorder Anti phospholipid syndrome
47
Differentials of thrombocytopenia by category
Hereditary Acquired immune Acquired non immune
48
What is living with sickle cell trait like
Comfortable- only have problems during hypoxia such as altitude and exercise
49
What is ITP
Immune thrombocytopenic purpura
50
Symptoms of ITP
Any sign of bleeding, normally on legs No signs of alternative cause such as lymphadenopathy, systemic symptoms or splenomegaly No drugs which cause thrombocytopenia
51
What happens to bone in anaemia
Increased production of reticulocytes resulting in new bone formation due to medullary expansion
52
How does medullary expansion appear on examination
Enlarged cheeks
53
What is complication when SCD RBCs occlude pulnmary arteries
Acute chest syndrome- blockage results in build up of deoxygenated blood which sets up negative feedback loop as pulnomary arteries constrict in hypoxia
54
Define ITP
Isolated thrombocytopenia in absence of identifiable cause. Involves autoimmune antibody destruction of peripheral platetels. IgG produced by spleen
55
Which encapsulated bacteria does splenectomy increase risk of
Strep pneumoniae Haemophilus influenzae Neissera meningitidis Salmonella
56
How does medullary expansion appear on head XR
"hair on ends" appearance to skull- increased haematopoeisis
57
Investigations for SCD
``` Newborn blood spot in a lot of countries FBC Blood smear Protein electrophoresis Urinalysis LFTs Lung function ```
58
3 causes of microangiopathic haemolytic anaemia
DIC TTP HUS
59
Blood features of DIC
``` Low Plts Anaemia Low fibrinogen Increased PT/APTT Increase D-dimer ```
60
Blood features of HUS
Decreased Hb Increased Bilirubin Uraemia Reduced plts
61
Hereditary cause of haemolytic anaemia
Red cell membrane- hereditary spherocytosis Enzyme deficiency- G6PD deficiency Haemoglobinopathy
62
Acquired causes of haemolytic anaemia
Autoimmune Drugs MAHA Infection
63
What is ALP in myeloma
Normal- as plasma cell suppress osteoblasts
64
What is telengiecstasia
Condition which widened venules cause threadlike red lines or patterns on the skin
65
What is inheritance of hereditary telengiecstasia
Autosomal dominant
66
Where do telengiecstasia occur
``` Skin Mucous membranes Lungs Liver Brain ```
67
What would most likely cause of microcytic anaemia in patient with high platelets
Slow GI bleed
68
What must you check if patient presents with microcytic anaemia
Platelets to see if evidence of bleeding
69
What does normal RDW suggest
There is a homgenous population of RBCs
70
What does a high RDW suggest
Aniscocytosis of RBCs
71
What causes pernicious anaemia
Malabsorption of B12 caused by failure to produce intrinsic factor
72
Blood finding of pernicious anaemia
Macrocytosis
73
What happens to neutrophils in pernicious anaemia
Hypersegmented
74
How to distinguish anaemia of chronic disease from a mixed picture of B12 and Iron deficiency
RDW is normal in ACD but wide in mixed picture
75
What happens in general malabsorption normally to MCV
Is normal as mixture of B12 and iron deficiency however RDW will be skewed as cells all different sizes
76
What would WCC be in a normal infection
Maybe 11-12
77
What would WCC be in pneumonia
15
78
What would WCC be in sepsis
15-25/30 depending on severity
79
What happens to WCC in malaria
Low/normal
80
Blood findings of malaria
Low/normal WCC Anaemic Platelet low
81
How would an immediate transfusion reaction present
``` Fever Rigor Hypotenisve Tachy Chest pain Dark urine ```
82
What would present acutely with rigor, fever, hypotenisve, tachy, chest pain and dark urine
Immmediate transfusion reaction
83
What can cause microcytic anaemia
IDA | Beta thalassaemia heterozygosity
84
What can cause IDA
Bleed in GI tract | Diet
85
What could ferritin be in normocytic anaemia
Normal or high
86
What causes normocytic anaemia
Chronic disease such as rheumatoid
87
When would you suspect beta thalassaemia heterozygosity
Normal healthy person who presents with very low MCV
88
Causes of macrocytic anaemia
``` Alcohol Myelodysplaisa Hypothyroid Liver disease Fotale/B12 deficiency ```
89
Pnemoninc for causes of macrocytic anaemia
Alcoholics may have liver failure
90
Clues for alcoholic macrocytosis
History | Raised GGT
91
Clues for myelodysplasia macrocytosis
Pancytopenia
92
Clues for hypothyroidism macrocytosis
Hx | Low T4 High TSH
93
Clues for liver diseases macrocytosis
Hx | Exam
94
Clues for folate deficiency macrocytosis
Hx small bowel disease or gastrectomy
95
What is pancytopenia
Low platelets, WCC and RBC
96
Presentation of polycythaemia
``` Pruritus after hot bath Thromboses Gangrene Headache Choreiform movements Blurred vision tinnitus ```
97
``` What presents with Pruritus after hot bath Thromboses Gangrene Headache Choreiform movements Blurred vision tinnitus ```
Polycythaemia
98
Management of SCD painful crises
Analgesia Oxygen IV fluids Abx
99
Management of stroke SCD
Blood transfusion
100
Why does SCD lead to gall stone and chronic cholecystitis
Chronic haemolysis
101
Pnemonic for multiple myeloma
``` CRAB Calcium Renal failure Anaemia Bone ```
102
Calcium presentation of multiple myeloma
Hypercalcaemia- polyuria, polydipsia and constipation
103
Renal presentation of multiple myeloma
Urea and creatinine elevated
104
Anaemia presentation of multiple myeloma
Breathlessness Lethary FBC
105
Bone presentation of multiple myeloma
Bone pain | Osteoporosis- fracture
106
Fractures seen in osteoporosis
Neck of femur Wrist- colles fracture Vertebral
107
What are some acute presentations of multiple myeloma
Infection | Cord compression
108
What causes anaemia with high reticulocytes
Myelodysplasia Haemolytic crises Haemorrhages
109
What causes anaemia with low reticulocyte count
Blood transfusion Parvovirus B19 infection Aplastic crises in SCD
110
What are patients with low reticulocytes normally presenting with
Aplastic crisis where not producing enough RBCs
111
What drug can cause macrocytosis
Azathioprine
112
How does where GI bleed is affect type of anaemia
Location- eg distal ileum affects B12 absorption
113
Microcytic anaemia causes
IDA Thalassaemia ACD
114
Normocytic anaemia causes
Acute bleed Aplastic anaemia Mixed anaemia from crohns
115
Investigations for microcytic anaemia
Haematinics | Hb electrophoresis
116
Investigations for macrocytic anaemia
B12 Folate DAT test
117
Diseases that can cause B12 deficiency
Pernicious anaemia | Crohns
118
How can haematological malignancies be split up
Lymphoma Leukaemia Other
119
What comes under other in haem malignancies
Myeloma Myelofibrosis Myelodysplasia Polycythaemia vera
120
How are leukaemias split up
Lymphoid Myeloid Are either acute or chronic
121
How are lymphomas split up
Hodgkins | Non-hodgkins
122
Similarities between lymphoma and leukaemia
Abnormal proliferation of lymphocytes
123
What is difference between lymphoma and leukaemia
The location of abnormalities | Location of leukaemia is in blood but lymphoma in the lymph nodes
124
Differences between acute and chronic leukaemia in terms of differentiation
Abnormal differentiation in acute but normal in chronic therefore mature cells present in chronic but immature in acute
125
Pathophysiology of hodgkins lymphoma
B cell abnormally differentiates within the lymph node leading to neoplasm with exact mechanism unclear but is assocaition with HIV and EBV virus infections
126
What are cells present in hodgkins lymphoma
Hodgkins cells- large undifferentiated B cells | Reed steenberg cells- multinucleated plasma cells joined together
127
Can hodgkins cells or reed steenberg cells produce ABs
No they are "crippled"
128
What happens in non-hodgkins lymphoma
Neoplasm of B or T cells that grow in nodes but DONT have reed steenberg cells
129
What is most common lymphoma
Non-hodgkins
130
Differences between non hodgkins and hodgkins lymphoma
H tends to just affect local nodes within tits group whereas non affects multiple groups and also other organs H contains reed steenberg cells
131
Non lymph node effects of non-hodgkins lymphoma
Mets anywhere Spinal chord compression Bone marrow suppression as crowds out other cells there
132
What is myeloma
Cancer of plasma cells producing excessive monoclonal Igs
133
Investigations for myeloma
``` ESR Calcium U&E Renal Elctrophoresis ```
134
Protein electrophoresis findings in myeloma
Normally would see polyclonal bands however in myeloma will see one large monoclonal band
135
Presentation of non-hodgkins lymphoma
Painless lymphadenopathy B symptoms Pruritus
136
Obs findings of anaemia
Tachypnoea | Tachycardia
137
What is angular cheilitis
Reddening of angles of mouth
138
Where do you see angular cheilitis in
IDA
139
Categories for IDA causes and examples of each
Increased loss- peptic ulcer, cancer, menstruation, IBD Reduced uptake- coeliac, diet, IBD Increased need- pregnancy, breastfeeding
140
To restore iron levels what are best foods to eat
Meats as plant based iron harder to absorb
141
What is most common cause of IDA worldwide
Parasitic infection of ascariasis or schistosomiasis
142
How does chronic disease lead to anaemia
Leads to release of cytokines that means hepcidin is upregulated
143
When hepcidin is upregulated what does this do
Reduced uptake via ferroportin Increased storage in ferritin Reduced transport via transferrin
144
Why is ferritin an acute phase protein
Logic is that in an infection the bacteria will want to use iron stores so body protects its store
145
How to differentiate between IDA and ACD
TIBC/ transferrin will be reduced in ACD but elevated in IDA | Ferritin will be low in IDA but elevated in ACD or normal
146
What is TIBC the same as
Transferrin
147
What type of anaemia is ACD
Microcytic or normo
148
Out of beta and alpha thalassaemia which is rarer
Alpha
149
Which disease is SCD and thalassaemia protective against
Falciprum malaria
150
Which groups of people often present with thalassaemia
Those from malaria belt countries | Cyprus especially
151
On what chromosome do alpha thalassaemias develop from
16
152
On what chromosome do beta thalassaemias develop from
11
153
What are the 4 alpha defects in thalassaemia
Alpha+ Alpha Hb H Hb barts
154
What happens when have alpha + or alpha0
Mild anaemias as only missing 1 or 2 alpha genes respectively
155
What happens when have Hb H
Missing 3 alpha genes leading to severe anaemia
156
What happens when have Hb Barts
Death in utero- hydrops fetalis syndrome where fluid overloaded
157
What are 3 types of beta thalassaemia
Beta minor Beta intermedia- just know exists Beta major
158
What happens when have beta minor thalassaemia
Mild anaemia with very low MCV
159
What happens when have beta major thalassaemia
Severe anaemia presenting in early life
160
Difference between alpha and beta thalassaemias in their presentation
Beta between 3-6 months Alpha from birth Due to that in first few months are synthesising Beta as have lots of HbF still
161
What Hb is elevated in beta thalassaemia
HbA2 | Alpha and delta to compensate
162
What predisposes to SCD events
Hypoxia Dehydration Acidosis Infection
163
Features of SC trait
Asymptomatic and resistant to falciprum malaria
164
Mutation leading to SC trait
Point mutation of B globin chain Chr 11 | Autosomal recessive
165
Causes of microcytic anaemia
IDA Thalassaemia ACD SCD
166
Difference in life span between sickle cells and normal RBC
10-20 days as opposed to 100 days
167
What are howell jolly bodies
Nuclear remnants of RBCs
168
What are howell jolly bodies a marker of
Splenic function deteriorating
169
Blood film findings of SCD
Sickle cells | Howll jolly bodies if splenic function affected by vaso-occlusive crises
170
Management of SCD crisis
Oxygen Abx Pain relief IV fluids
171
Crises caused by SCD and how they are managed
Stroke-> transfusion Splenic sequestration-> splenectomy Chronic cholecystitis-> cholecystectomy
172
What happens in splenic sequestration
As sickle cells need clearing the spleen does this however can remove too many leading to splenomegaly and anaemia from accumulation of cells in spleen
173
Difference in spleen problems with age
When younger sequestration a problem however as get older due to recurrent occlusive crises leads to hyposplenism
174
Problems of hyposplenism
Susceptibility to infection as an important immune organ- white pulp
175
How is SCD diagnosed
Hb electrophoresis | Blood film
176
Complications of SCD
``` Priapism Haemolytic anaemia Aplastic crisis Acute chest syndrome Dactylitis ```
177
Conservative management of SCD
Trigger avoidance | Vaccination
178
Medical management of SCD
Vaccinations Hydroxyurea Prophylactic Abx
179
Surgical management of SCD
Bone marrow transplant
180
What ABx is given prophylactically in SCD
Penicillin V as gives protection against encapsulated bacteria- meningitis
181
Why are vaccinations given in SCD
Infections can trigger crises and hyposplenism
182
Which is replaced first folate or B12
B12 due to neurological implications
183
How long do B12 stores last
3-4 years
184
Blood film findings of folate or B12 deficiency
Macrocytic anaemia | Hypersegmented neutrophils
185
What causes megaloblastica anemia
B12 or folate
186
What are hypersegmented neutrophils seen on blood film of
Megaloblastic anaemia
187
What can cause folate but not B12 deficiency
Anti-folate drugs | Pregnancy
188
Why doesnt pregnancy cause B12 deficiency
B12 stores 3-4 years
189
What causes B12 deficiency but not folate deficiency
Pernicious anaemia | Malnutrition
190
Why doesnt malnutrition tend to cause foalte deficiencies
In vedgetarians diet there is plenty of folate but not B12 as is normally found in meat
191
What causes both folate and B12 deficiency
Alcoholism Crohns Coeliac
192
3 ways alcohol causes macrocytic anaemia
1. alcohol itself causes non-megaloblastic anaemia 2. alcholism leads to poor dietary intake as get calories from alcohol itself 3. alcohol can affect gut causing malabsorption
193
How long does it take folate sotres to run out
3-4 months
194
How does B12 cause neuro sx
Needed for building the myelin sheath
195
What is physical examination test for B12
Romberg
196
What is physical examination test for folate
Schillings
197
What antibodies are made in pernicious anaemia
Anti parietal cell | Anti intrinsic factor
198
2 ways B12 affects the nervous system
Subacute combined degeneration of the chord | Peripheral sensory nerves
199
How does subacute degeneration of spinal chord present due to B12 deficiency
Corticospinal tract myopathy | Dorsal column tract- vibration, pressure
200
Who does pernicious anaemia tend to affect
60 year olds
201
What presents with bilateral myopathy, dorsal column problems
B12 deficiency
202
In exclusively what type of haemolysis does haptoglobin get used up in
Intravascular- doesnt happen in extravascular
203
What must be avoided in people with G6PD deficiency
Anything that causes oxidative stress like broad beans
204
Blood features of any haemolysis
Anaemic Unconjugated bilirubin high LDH raised *haptoglobin low in intravascular
205
What is inheritance of G6PD
X linked recessive
206
What are heinz bodies
Intracellular inclusions of denatured Hb that are only present during haemolytic anaemia due to oxidative stress
207
Is G6PD the only predisposing enzymopathy to oxidative stress
No but just happens to be the most common
208
What are bite cells formed from
Previous removal of heinz bodies by spleen
209
What in inheritance of hereditary spherocytosis
Autosomal dominant
210
What do you see heinz bodies and bite cells in
Oxidative stress leading to haemolysis- most commonly G6PD
211
In which group of people do you see hereditary spherocytosis
White northern europeans
212
In hereditary spherocytosis what are people deficient in
Beta spectrin or ankyrin which leads to weak cytoskeleton so cells cant maintain biconvex shape
213
What is the osmotic fragility test
Give patient hypotonic saline
214
What is result of osmotic fragility test in HS
HS cells are more likely to lyse as their cytoskeleton is much weaker
215
What is an aplastic crisis
When you fail to only produce red cells
216
What can lead to aplastic crises in HS sufferers
Parvovirus B19 infection- invades RBC precursors
217
Why are HS sufferers more prone to aplastic crises
HS cells have a lifespan of only 30 days as their shape leaves them very vulnerable to damage in microcirculation
218
What are schistocytes
Fragmented RBCs produced after after microangiopathic haemolytic anaemia
219
GI features of Ecoli 0157
Abdo pain | Bloody diarrorhoea
220
Triad of HUS
AHA AKI Thrombocytopenia
221
Pathophysiology of HUS
Ecoli 0157 releases toxin that binds to endothelial cells within the kidney and damages them- damaged endothelium leads to platelet plug formation which leads to microthrombi production. These then shear RBCS and cause AKI
222
Examination findings of HUS
Jaundice | Conjuctival pallor
223
Pathophysiology of DIC
Severe inflammatory response initiated that activates clotting factors everywhere which exhausts platelets and clotting factors. These then break down and lead to MAHA. Also due to exhaustion of clotting factors this predisposes to bleeding
224
Some triggers for DIC
``` Pancreatitis Sepsis Obstetric problems Cancers Trauma Transfusion reaction ```
225
Signs on examination of DIC
Petechiae Haematuria Ecchymoses Jaundice
226
What are ecchymoses
Discolouration of skin due to underlying bleeding
227
Examination findings of DIC
Jaundice Conjunctival pallor Petechiae Ecchymoses
228
What is main difference between ITP and TTP
Autoimmune reaction against platelets in ITP
229
Is TTP coombs positive or neg
Neg
230
What is DAT test and what does it show
Direct Antiglobulin Test | Shows whether haemolytic anaemia is autoimmune or not
231
What happens in DAT/coombs test
Anti-human globulin antibodies are given to blood sample- if antibodies are present against RBC globulin antigen then the RBCs will agglutinate and be postive for autoimmune haemolytic anaemia
232
What drugs cant be used in G6PD
Anti-malarials (e.g. primaquine and chloroquine) Nitrofurantoin Quinolone antimicrobials (e.g. ciprofloxacin) Rasburicase Sulphonamides (e.g. co-trimoxazole)
233
What are risk factors for myelofibrosis
Old age | Radiation exposure
234
Physiologically what happens in myelofibrosis
Fibroblasts in bone marrow become overactive and proliferate in response to BM cancer which causes a crowding out effect that reduces out put of blood cells
235
What is myelofibrosis classed as
Philadelphia chr negative myelodysplasia
236
What are typical cells seen in myelofibrosis on blood film
Tear cells also known as dacrocytes
237
What are tear cells seen in
Myelofibrosis- could imagine as bm fibrosis squeezes out the cells affecting their shape as they leave
238
What do you see dry tap on aspirate in
Myelofibrosis
239
Which 2 conditions classically do you see massive splenomegaly in
CML | Myelofibrosis
240
Blood findnigs of myelofibrosis
Low WCC, RBC, Hb | High plts
241
How is polycythaemia classified
Into secondary and primary | Secondary is in response to a trigger than can either be appropriate or inappropriate
242
How is inappropriate polycythaemia classified
Into appropriate and inappropriate
243
Examples of appropriate secondary polycythaemia
Response to hypoxia- cyanotic heart disease, altitude, hypoxic lung disease like COPD
244
Example of inappropiate secondary polycythaemias
EPO secreting tumour
245
What is primary polycythaemia also called
Polycythaemia rubra vera
246
What condition is always JAK 2 positive
Polycythaemia rubra vera
247
Physiology of JAK 2 mutation
Leads to Polycythaemia rubra vera EPO is secreted in kidney and binds to JAK 2 receptor on haematopoietic cells which stimuluates red cell production. When its mutated it activates itself rather than by EPO meaning it is a true primary polycythaemia
248
What is polycythaemia rubra vera classed as
Philadelphia chr negative myelodysplasia
249
What is an essential thrombocytosis
When exclusively platelets are raised
250
What is aquagenic pruritis
Skin itching in absence of skin lesions particularly when make contact with water
251
How can polycythaemia rubra vera typically present
Asymptomatically but then in older people around 60 get symptoms
252
What are symptoms of polycythaemia rubra vera
Due to hyperviscosity of blood - stroke - headaches - lightheadedness - gangrene - eye problems - aquagenic pruritus
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What leads to aquagenic pruritis in PCV
overproduction of histamine
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Blood findings of PCV
High Hb High haematocrit Can be thrombocytosis
255
What does electrophoresis show in beta thalassaemia
Increase HbA2
256
What does haemoglobin H present with on examination
Signs of extreme anaemia | Splenomegaly
257
Blood film findings of haemoglobin H
Target cells | Heinz bodies
258
Best test for IDA
Serum ferritin (CRP must be normal to be accurate)
259
What do you see schistocytes in
Haemolytic anaemia | DIC
260
What do you see spheorcytes in
Haemolytic anaemia | HS
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What do you see target cells in
Obstructive jaundice Liver disease Haemoglobinopathies Hyposplenism
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How do you get target cells in obstructive jaundice
Ratio of cholesterol in RBC influenced by bile salts
263
Possible blood transfusion reactions
``` Tissue related lung injury Immediate haemolytic anemia Delayed haemolytic anaemia IgA deficiency Febrile non-haemolytic transfusion reaction ```
264
What happens in tissue related lung injury
2-6hrs after transfusion inflammatory process causes sequestration of neutrophils within the lungs and antibodies form against donor white cells which then attack cells in lungs with HLA antigens
265
How does tissue realted lung injury post transfusion present
Fever Hypotension Cyanosis Pulmonary oedema
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In haemolytic reactions to transfusions how does that present
``` Hyoptension Tachycardia N and V Abdo pain Loin pain ```
267
How to differentiate between delayed and immediate haemolytic reaction
Within 24 hours or not
268
How does IgA deficiency present after transfusions
Bronchospasm Laryngeal oedema Hypotension
269
How does febrile non haemolytic anaemia present
Fever RIgors Discomfort
270
What is von willebrand factor important in
Platelet adhesion and factor VIII function
271
Which pathway does von willebrand deficiency affect
Intrinsic
272
What is von willebrand disease
Deficiency of von willebrand factor
273
Blood findings of VwB
Normal PT PTT longer Total bleeding time longer Plt count normal
274
In pernicious anaemia what tends to be MCV
Very high- over 120
275
How can blood cancers present
Sign of failure of bone marrow- bleeding, infections, anaemia Recurrent or severe infections Accumulation of malignant cells Systemic signs
276
How can blood cancers present in terms of recurrent or severe infections
Gram negative septicaemia from neutropenia | Lympoid infections of herpes
277
Examination findings of cml
Hepatosplenomegaly
278
Examination findings of CLL
Hepatosplenomegaly | Lymphadenopathy
279
How can blood cancers present where pressure obstructs something
Lymphoid hyperplasia- liver, appendix, brain, bowel
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Causes of pancytopenia
``` B12 deficiency Aplastic anaemia Drug toxicity Radiation Blood cancer Mets to bone marrow ```
281
Difference between core biopsy and excisional biopsy in process
``` Core - per cutaneous US guided - day case - no wound Excision - GA with surgery - excision scar ```
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Uses of core biopsy vs excision biopsy
Core is good for non lymphoid matter so can see TB and mets | Excisional can do that as well as lymphoma diagnosis
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Limitation of core biopsy
Can't use it for lymphatic architecture so limited in lymphoma diagnosis
284
What can myeloma plasma cells do at bone sites
Cause bone expansile Infiltrate the bone marrow Form soft tissue tumours called plasmacytomas
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What is name of soft tissue tumours formed in myeloma
Plasmacytomas
286
What can myeloma plasma cells produce
Monoclonal IgA and IgG | Monoclonal kappa and lamda light chains
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What is found in urine of myeloma
Urine monoclonal light chains called bence jones protein
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What are bence jones proteins
Monoclonal light chains in the urine or blood of myeloma
289
What is a paraprotein
Intact monoclonal antibody
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What does SFLC stand for
Serum free light chain
291
What is immune paresis
When due to mass production of paraprotein in myeloma the other Igs are decimated
292
Bone presentation of myeloma
``` Normally proximal bones affected Pain Osteolytic lesions Osteopenia Easy fractures ```
293
Difference between prostate met and myeloma lesion
Prostate mets will have osteosclerosis in area around the lesion but myeloma lesions just a punched out lesion
294
Risk factors for myeloma
Black Male Elderly
295
Two types of bone biopsy
Aspirate- liquid bone marrow | Trephine- whole cut
296
Common sources of mets to bone marrow
Melanoma | Stomach adenocarcinoma
297
What is diaphoresis
Xs sweating
298
Sx of malaria
Cyclical fevers Diaphoresis Chills Headache
299
How does malaria present on blood film
Ring form of parasite within blood cell
300
What are 4 main forms of plasmodium parasite
Falciparum Malariae Ovale Vivax
301
Which plasmodiums are relapsing
Ovale | Vivax
302
Management plan for someone with IDA
Oral iron supplementation | If Hb doesnt improve move to IV supplement in forms of ferrous sulphate/fumarate
303
What is initial blood cell in process
Pluripotent haematopoeitic stem cell
304
Products of pluripotent haematopoeitic stem cell
Common myeloid progenitor | Common lymphoid progenitor
305
Products of small lymphocyte
T lymphocyte | B lymphocyte
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Products of common lymphoid progenitor
Small lymphocyte | NK cell
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What do B cells divide into
Plasma cells
308
Products of common myeloid progenitor
Myeloblast Eryhtocytes Megakaryocytes Mast cells
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What do myeloblasts differentiate into
Neutorophils Basophils Eosinophils Monocytes
310
What cells are AML and CML neoplasms from
Myeloblasts | Myeloid progenitor cell
311
What cells are ALL neoplasms from
Common lymphoid progenitor cell
312
What cells are CLL neoplasms from
Small lymphocytes
313
Where do essential thrombocytopenias arise from
Megakaryocytes or thrombocytes
314
Blood findings of myeloma
``` Raised ESR Anaemia Hypercalcaemia Raised creatinine Pancytopenia ```
315
What does raised ESR suggest physiologically
Increased proteins in the blood
316
How can you get headaches, blurred vision and SOB in myeloma
Due to hyperviscosity of blood from increased proteins
317
What can cause spinal chord compression in myeloma
Plasmacytoma | Vertebral body collapse
318
Differential to consider when have renal impairment and hypercalcaemia
Prostate mets | Myeloma
319
What is a myeloproliferative disorder
Where is neoplasm of myeloid pathway where all cells tend to elevated- name determined by what cell line predominates
320
Name of myeloproliferative disorder when RBC line predominates
Polycythaemia vera
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Name of myeloproliferative disorder when plt line predominates
Essential thrombocytosis
322
Name of myeloproliferative disorder when granulocyte line predominates
CML | Can be divided further if one cell predominates- ie eosinophilic leukaemia
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FBC findings of myelofibrosis
Anaemia | Can be panctopenic or all elevated
324
What is Hct in polycythaemia vera
High
325
How can polycythaemia vera present with low MCV
Become iron deficient as use it all up
326
Weird causes of PCV
Polycystic kidney disease | Hepatocellular carcinoma
327
What would exclude a peptic ulcer from IDA cause on gastroscopy
Clean based | No vessel present in the ulcer
328
What infection in children can give marked lymphocytosis
Whooping cough- bordetella pertussis
329
Who is CLL very common in
Elderly- normally picked up incidentally
330
Signs on examination of CLL
Lymphadenopathy | Hepatosplenomegaly
331
Presentation of CLL
Weight loss Lymphocytosis Lymphadenopathy Hepatosplenomegaly
332
Difference between warm and cold AIHA in terms of COOMBS
In warm COOMBs is positive at temp eaual to or above 37C(body temp) Cold COOMBs happens at temps below that
333
Difference between warm and cold AIHA in terms of physiology
Warm- IgG, complement cant be activated, extravascular | Cold- IgM, complement activated, intravascular
334
What are the proportions of AIHA
Extravascular- 70 Intravascular- 20 Drug- 10
335
How can warm AIHA be classified
Primary | Secondary- SLE, CLL
336
Causes of cold AIHA
Post infection | Lymphoproliferative conditions
337
Common post infection causes of AIHA
CMV Mycoplasma EBV
338
What condition are auer rods seen in
APML
339
What is the translocation in APML
15;17
340
What is APML
A subtype of AML which results in proliferation of promyelocytes
341
What is most worrisome complication of APML
DIC
342
What is translocation of CML
9;22 -> philadelphia chromosome | Present in 95% of CML cases
343
Blood count findings of CML
Elevated WCC Basophils Neutrophils Hypercellular bone marrow
344
What cancer does a latent EBV infection lead to
Burkitts lymphoma
345
How to describe splenomegaly in EBV
Mild
346
Thalassaemia on examination
Massive splenomegaly
347
What are elliptocytes
Oval shaped red cells
348
What are pencil cells
Hypochromic elliptocytes
349
What do pencil cells occur in
IDA
350
What do elliptocytes occur in
IDA | Hereditary elliptocytosis
351
What happens if take anti malarial in G6PD
Oxidative stress- SOB, pallor and bloody urine
352
What is stain used to differentiate between AML and ALL
Sudan black
353
Test used to diagnose SCD
Sodium metabisulphate
354
How to differentiate depressive dementia and frontotemporal dementia if has change in hobbies
With this could be either but fronto temporal will have speech changes too
355
Symptoms of frontotemporal dementia
Change in personality | Dysphasia
356
Step wise decline in cogntion
Vascular dementia
357
Which clotting factors are made by the liver
II. VII, IX, X- all Vit K dependant
358
Why when on warfarin does patient go through initial pro thrombotic stage
Because protein S and C are also vit K dependant and get affected first
359
Which pathway does heparin affect
APTT
360
Which pathway do haemophilias affect
APTT
361
When are packed red cell transfusions indicated
Catastrophic haemorrhage Hb less than 70 with signs of compromise- syncope, dyspnoea, tachy Hb less than 80 with known ischaemic heart disease and severe resp disease
362
What is target Hb for transfusions
Above 70 as risk of complications from too many is too great
363
What is most common coagulopathy causing DVTs in young people
Factor V leiden
364
What are coagulopathys causing prothrombotic states
Factor V leiden Protein S deficiency Protein C deficiency Antithrombin deficiency
365
Symptoms of VwB disease
Easy brusing | Epistaxis
366
Triad for B12 deficiency
Weakness Glossitis Fatigue
367
How much does 1 unit of packed red cells increase Hb
10-15
368
What is FFP used for
Corrects clotting factors- DIC eg
369
Early complications of transfusion
``` Anaphylaxis Transfusion association lung injury Febrile non-haemolytic reaction Acute haemolytic reaction Bacterial infection ```
370
Late complications of transfusion
Delayed haemolytic reaction Infection Iron overload
371
What is paroxysmal cold haemoglobinuria
Haemolysis triggered by cold such as washing hands and drinking cold water

Year 3 Conditions (33 decks)

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