Brainscape's Knowledge GenomeTM


Top Flashcards (Certified)
All Flashcards

Haematology and Immunology > Haematology > Flashcards

Haematology Flashcards

(387 cards)

Start Studying
1
Q

What are the components of blood?

A

Plasma, Buffy coat and red blood cells

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

What products are found in the Buffy coat?

A

Platelets

White cells

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

What products are found in the plasma?

A

Clotting, coagulation factors

Albumin
Antibodies

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

List functions of blood.

A

Transport - Oxygen and CO2, Nutrients, Waste, Messages

Maintenance of vascular integrity

Protection from pathogens

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

How does blood maintain vascular integrity?

A

Prevent leaks - platelets, clotting factors

Prevent blockage - anticoagulants, fibrinolytic

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

List leukocytes (white cells).

A 
Meutrophil
Monocyte
Basophil
Eosinophil
Lymphocyte
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

What cells produce erythrocytes?

A

Erythropblast -> reticulocyte

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

Stem cell flux is regulated by what?

A

Hormones, growth factors e.g erythropoietin, G-CSF

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

Where is erythropoietin produced?

A

Made in kidney in response to hypoxia

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

What can you use to measure red blood cell production?

A

Reticulocytes

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

What is polycythaemia?

A

Too many red cells - in myeloid malignancies

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

What are consequences of anaemia?

A

Poor gas transfer, SOB, fatigue

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

What causes anaemia?

A

Deficiency in hamatinics e.g iron, folate, vit B12

or congenital - thalassaemia

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

What causes an increased loss in red blood cells?

A

Bleeding, haemolysis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

What is polychromasia seen on microscopy?

A

Cells in body that look grey - high reticulocyte count

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

What is the function of platelets?

A

Haemostasis (and immune)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
17
Q

What developing white cells produce platelets?

A

Megakaryocytes

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
18
Q

What regulates production of platelets?

A

Thrombopoietin produced in liver

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
19
Q

What do you see in relation to platelets and liver disease/

A

Low platelets

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
20
Q

What is the lifespan of platelets?

A

7 days

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
21
Q

What is thrombocytopenia?

A

Not enough platelets

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
22
Q

What are signs of thrombocytopenia?

A

Marrow failure

Immune destruction

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
23
Q

What is thrombocytosis?

A

Too many platelets

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
24
Q

What is neutrophilia and what causes it?

A

Too many neutrophils

Infection and inflammation

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
25
What regulates production of neutrophils?
Granulocyte-colony stimulating factor (G-CSF)
26
When is G-CSF used therapeutically?
Neutropenia | Mobilisation of stem cells
27
What is neutropenia?
Decreased production of neutrophils
28
What can cause neutropenia?
Temporary phenomena in sepsis Drugs Marrow failure
29
What cells make up the reticuloendothelial system?
Monocytes
30
What do monocytes differentiate into?
Macrophages | Dendritic cells
31
What is the function of eosinophils?
Parasitic infections | Allergies
32
List two causes of lymphocytosis.
Infectious mononucleosis | Pertussis
33
What cells are important in the adaptive immune system?
Lymphocytes
34
List causes of lymphopenia.
Post-viral | Lymphoma
35
What are the three subtypes of lymphocytes?
B, T, NK cells
36
Where are lymphocytes produced?
B cells - Bone marrow | T cells - thymus
37
In B cell maturation, when in the pathway can ALL occur?
Errors at progenitor V cell splicing in bone marrow
38
Where do most lymphomas arise from?
Within lymph node during somatic hypermutation
39
What do class 1 HLA molecules display?
Internal antigens on all nucleated cells Self antigen
40
What do class 2 HLA molecules display?
Display antigens eaten by professional APCs
41
What is the purpose of HLA molecules?
Immune cells read HLA- barcode on cells to identify self vs non-self or uninfected vs infected cells
42
List systemic diseases that affect the blood.
RA - Anaemia, Folate deficiency, immune haemolysis, neutrophilia, immune thrombocytopenia
43
What is felty syndrome?
Large spleen and thrombocytopenia linked to that. Early lymphoma.
44
What is a paraprotein?
Malignancy or early malignancy of plasma cell --> one type of cell (too much plasma)
45
What causes too little plasma proteins?
Haemophilia (little clotting factors or abnormal)
46
What is the normal range of Haemoglobin?
Male - 135-170 | Female - 120-160
47
What is the normal range of platelets?
150-400
48
What is the normal range of WBC?
4-10
49
What is the normal range of neutrophils?
1.5-7
50
List diagnostic tools for haematological disorders.
``` FBC Clotting times for factors and platelets Chemical assays - B12, iron, folate Marrow aspirate and trephine biopsy, lymph node biopsy Imaging ```
51
List common haematological treatments.
Replacement Transplantation Drugs
52
If you are blood group A, what do you have antibodies against?
B
53
If you are blood group B, what do you have antibodies against?
A
54
If you are blood group O, what do you have antibodies against?
A and B
55
If you are blood group AB, what do you have antibodies against?
NO antibodies
56
What blood type is the universal donor?
O
57
What blood type is the universal recipient?
AB
58
If you are blood group A, there is anti-B in the plasma. Who can you donate FFP to?
A and O | instead of A and AB in red cells
59
What happens if RhD- individuals can make anti-D if exposed top RhD+?
Transfusion reactions, haemolytic disease in newborns
60
What are blood donations tested for?
HepB/C/E, HIV, Syphilis Variably for HTLV1, malaria, West Nile virus, Zika virus
61
List indications for red cell transfusions
Correct sever acute anaemia Improve QoL if uncorrectable anaemia Prepare patient for recover Reverse damage caused by own red cells e.g sickle cell
62
How long do you transfuse blood over?
2-4 units
63
How long do you transfuse platelets over?
20-30 minutes
64
What are indications for platelet transfusion?
Massive haemorrhage Bone marrow failure Prophylaxis for surgery Cardiopulmonary bypass
65
Compare storage temperature and time of red cells and plasma?
Red cells - 4 degrees Platelets - 22 degrees, 7 days help life
66
In patients receiving chemo, what are platelets used for?
Preventing intracranial haemorrhage
67
Where does 1 unit of FFP come from?
1 unit of blood
68
How is FFP stored?
Frozen, need 30 mins to thaw
69
What are indications for FFP?
Massive haemorrhage DIC with bleeding Prophylactic
70
What lab tests are carried out for FFP?
PT and APTT
71
What lab tests are carried out for cryoprecipitate?
Fibrinogen
72
When dealing with the blood bank, what is required to get blood?
Blood sample- EDTA Two sample policy Group and screen/save Cross match
73
What is group and screening?
ABO and RhD type Checked against historical records Screen for all all-antibodies in serum
74
What is Coombs test?
Used when looking for other IgG antibodies in lab --> indicates a condition known as hemolytic anemia, in which your blood does not contain enough red blood cells because they are destroyed prematurely.
75
If you are pregnant and RhD- and developed anti-D (nearly always IgG which crosses placenta). What can happen to baby?
If baby RhD+ (inherited from father) --> haemolytic disease - die in utero or at birth
76
How is haemolytic disease prevented in newborn with mother who is RhD-?
Prophylactic anti-D (routine at 28/40)
77
What should you monitor if giving anti-D?
Monitor antibody titres Doppler USS Intrauterine transfusions
78
What causes neonatal alloimmune thrombocytopenia?
Similar process to anti-D but for platelets
79
List cellular therapies that use blood donations?
Leucapheresis - bone marrow harvests, donor lymphocyte infusions Gene therapies
80
What is anaemia?
Reduction in red cells or haemoglobin content
81
List aetiologies of anaemia.
Blood loss Increased destruction Lack of production Defective production
82
What are reticulocytes?
Immature RBCs
83
What substances are required for red cell production?
Metals e.g iron Vitamins e.g B12, folic acid Hormones e.g erythropoietin , GM-CSF
84
How long does a RBC live?
120 days
85
How are RBCs broken down?
In reticuloendothelial system Globin --> AAs reused Haem --> iron recycled to Hb, uncongucated bilirubin
86
What 3 components make up RBCs?
Membrane Enzymes Haemoglobin
87
What causes congenital anaemias?
Genetic defects in RBC - membrane, enzymes, Hb
88
What happens if there are defects in the cell membrane of RBC?
Increased cell destruction
89
What is hereditary spherocytosis?
Red cell membrane disorder - one of most common, autosomal dominant RBCs are spherical - removed from circulation faster by reticuloendothelial system
90
How do patients with hereditary spherocytosis present?
Anaemia Jaundice (neonatal) Splenomegaly Pigment gallstones
91
How do you treat hereditary spherocytosis?
Folic acid Transfusion Splenectomy if anaemia very severe
92
what are the two main enzyme pathways in RBCs?
Glycolysis | Pentose phosphate shunt
93
Give an example of an enzyme abnormality of RBCs.
G6-PD Cells vulnerable to oxidative damage (since normal function is to protect)
94
What does G6PD deficiency confer protection against?
Malaria
95
How does G6PD deficiency present?
Variable: anaemia, neonatal jaundice, splenomegaly, pigment gallstones
96
What can precipitate G6PD deficiency?
Drugs Broad beans Infection Acute illness
97
List two enzyme deficiencies of RBCs.
G6DP deficiency | Pyruvate kinase deficiency
98
What happens to the oxygen dissociation curve if acidosis?
Shifts to right
99
What happens to the oxygen dissociation curve if increased temperature?
Shifts to right
100
What happens to the oxygen dissociation curve if increased DBG?
Shifts to right
101
What is normal adult haemoglobin made of/
Alpha genes, beta chains, little delta and gamma chains
102
In terms of chains, what is normal adult haemoglobin made from?
Hb A = aabb
103
What are the two main types of thalassaemias (reduced or absent globing chains?
Alpha and beta
104
Give an example of a mutation that leads to structurally abnormal globing chain/
HbS (sickle cell) - beta chain abnormality combined to normal alpha chain
105
What are consequences of HbS?
haemolysis --> vaso-occlusion
106
How do patients with sickle cell disease present?
Painful vaso-occlusive crises - bone Stroke Chest crisis Increased infection risk Chronic haemolytic anaemia - gallstones, aplastic crises Sequestrain crises - spleen (Hyposplenia), liver
107
What is a sickle cell crisis?
Severe pain
108
How do you manage acute sickle cell crisis?
``` Opiates within 30 mins of presentation for one hour Hydration Oxygen Consider antibiotics Blood transfusion (HbF doesn't sickle) ```
109
What bones present with a painful crisis in children vs adults
Adults - long bone, back and hip Children - small bones
110
What life long prophylaxis is given for sickle cell disease?
``` Vaccination Penicillin (and malarial) prophylaxis Folic acid Disease modifying drugs - hydroxycarbamide Bone marrow transplant Gene therapy ```
111
What causes thalassaemia?
Reduced or absent globing chain production - in alpha or beta genes
112
If you don't inherit any alpha chains from parents, can you make HbF?
No, incompatible with life Also dangerous for mother
113
If you don't inherit any beta chains from parents, can you make HbF?
Yes with alpha and gamma chains Compatible with life but severe anaemia - need regular transfusions
114
Name a transfusion dependent thalassaemia.
Beta thalassaemia major
115
What are signs of beta thalassaemia major?
``` Failure to thrive Bony deformaties Splenomegaly Growth retardation Ineffective bone marrow expands ```
116
How do you treat beta thalassaemia major?
Chronic transfusion support - 4-6 weeks
117
Why do you need iron chelation for beta thalassaemia major?
Prevents iron overloading and reduced life expectancy
118
Name a curative therapy for thalassaemia.
Bone marrow transplant
119
What factors can affect normal range of haemoglobin?
``` Age Sex Ethnic origin Time of day sample taken Time to analyse ```
120
What are the reference ranges for haemoglobin?
Male 12-70 (140-180) and >70 (116-156) Female aged 12-70 (120-160), >70 years (108-143)
121
List clinical features of anaemia.
``` Tiredness/pallor Breathlessness Swelling of ankles Dizziness Chest pain ```
122
What is MCH and MCV?
Mean cell haemoglobin | Mean cell volume
123
How can RBC be described morphologically?
Hypochromic, microcytic Normochromic normocytic Macrocytic
124
If macrocytic RBCs seen morphologically, what is this a sign of?
B12/folate deficient | or bone marrow problem
125
If hypochromic microcytic RBCs seen morphologically, what should you test for?
Serum ferritin
126
If normochromic normocytic RBCs seen morphologically, what should you test for?
Reticulocyte count - if low - may be aplastic anaemia, malignancy (i.e issue with bone marrow)
127
If you have hypo chromic microcytic anaemia and serum ferritin is normal or increased, what would this suggest?
Thalassaemia Secondary anaemia Sideroblastic anaemia
128
If you have hypo chromic microcytic anaemia and serum ferritin is low, what would this suggest?
Iron deficiency
129
What is the connection of ferritin and CRP?
Ferritin = acute phase reactant (goes up with CRP)
130
What is your total body iron?
approx 4g
131
Where is hepcidin synthesised?
Liver
132
What is the role of ferroportin?
Transports iron from enterocytes in gut and macrophages to iron stores by being bound to transferrin
133
What is the role of hepcidin?
When there is increased iron, blocks ferroportin so decreases intestinal iron absorption and mobilisation from reticuloendothelial cells
134
What is the commonest cause of anaemia?
Iron deficiency
135
What can cause iron deficiency anaemia?
``` Dyspepsia Menorrhagia Diet Increased requirement e.g pregnancy Malabsorption - gastrectomy, coeliac ```
136
List clinical signs of iron deficiency anaemia seen on examination.
Koilonychia Angular cheilitis Atrophic tongue
137
How do you manage iron deficiency anaemia?
Correct deficiency - oral iron, IV iron if intolerant, blood transfusion rare Correct cause - diet, ulcer therapy, gynaecology interventions, surgery
138
If normochromic normocytic anaemia with normal/low reticulocyte count what could be the cause?
Secondary anaemia
139
If normochromic normocytic anaemia with high reticulocyte count what could be the cause?
Acute blood loss | Haemolysis
140
What can cause haemolytic anaemia?
accelerated red cell destruction (reduce Hb) | Compensation by bone marrow (increased metic count)
141
What are the two different types of acquired haemolytic anaemia.
Immune - mostly extravascular Non-immune - mostly intravascular
142
What is the purpose of a direct anti globulin test?
Detects antibody or complement on red cell membrane immune haemolysis - autoimmune issue?
143
If DAGT positive in haemolytic anaemia, what does this suggest?
Immune mediated
144
How can you work out if patient is haemolysing?
``` FBC Reticulocyte count Blood film Serum bilirubin LDH ```
145
Should your reticulocyte count be high or low if haemolysing?
High
146
What tests are used in haemolytic anaemia?
Hx and Ex Blood film Direct antiglobulin test (Coomb's test)
147
How do you manage haemolytic anaemia?
Support marrow - folic acid Correct cause - immunosuppression if autoimmune, splenectomy, treat sepsis Consider transfusion
148
What is the commonest cause of microcytic anaemia?
B12 deficiency and folate deficiency
149
What is megaloblastic anaemia?
B12 and folate deficiency
150
What can cause B12 deficiency?
Pernicious anaemia | Gastric/ileal disease
151
What can cause folate deficiency?
Dietary, increased requirements, GI pathology
152
What clinical signs would suggest megaloblastic anaemia?
"Lemon yellow" tinge
153
What is pernicious anaemia?
Autoimmune disease Develop antibodies against intrinsic factor Malabsorption of dietary B12
154
How do you manage megaloblastic anaemia?
Replace vitamin B12: intramuscular injection - loading dose then 3 monthly maintenance Oral folate replacement
155
What additional factors outwith anaemia, can cause macrocytosis (i.e differentials)?
``` Drugs Alcohol Disordered LFTs Hypothyroidism Myelodysplasia ```
156
What is involved in clot formation?
Platelets, vWF, coagulation factors
157
How is the clot confined to the site of injury?
Natural anticoagulants
158
What causes the clot to vanish after a week?
Fibrinolytic system
159
What is the function of nitric oxide and Prostsyacyclyn for endothelial cells in vessels?
Non-stick surface
160
What activates resting platelets and coagulation factors to form a clot?
Abnormal surface | Physiological activator
161
What is the role of platelets?
Adhere Activate Aggregation Provide phospholipid surface for coagulation
162
What are the 3 components of primary haemostats?
Vasoconstriction Platelet adhesion Platelet aggregation
163
What is a thrombus?
Clot in wrong place
164
What is a thromboembolism?
Movement of clot along vessel
165
What are the three components of Virchow's triad?
Stasis Vessel damage Hypercoagulation
166
What is the common components of arterial thrombus?
White clot - platelets and fibrin
167
What does arterial thrombi result in?
Ischameia and infarction
168
List examples of arterial thromboemolism
Coronary thrombosis - MI, unstable angina Cerebrovascular - stroke, TIA, Peripheral - limb ischaemia
169
List risk factors for arterial thrombosis.
``` Age Smoking Sedentary lifestyle HTN DM Obesity Hypercholesterolaemia ```
170
How do you manage arterial thrombosis?
Primary - lifestyle change, treat risk factors Acute presentation - thrombolysis, anti platelet/anticoagulant Secondary prevention
171
How are venous thrombi composed?
Fibrin and red cells
172
What does venous thrombi cause?
Back pressure --> valvular insufficiency --> post-thrombotic syndrome
173
What commonly causes venous thrombus?
Stasis and hyper coagulability
174
Give examples of venous thromboembolism.
DVT | PE
175
What are risk factors for venous thrombosis associated with stasis/hypercoagulability?
``` Age Pregnancy Surgery Obesity HRY Trauma Immobility FH Systemic disease ```
176
What systemic diseases
Cancer Myeloproliferative neoplasm (MPNs) Autoimmune disease e.g IBD, SLE, antiphospholipid syndrome
177
How do you diagnose venous thrombosis?
Pretest probability - Wells, Geneva scores Lab test - D-dimer Imaging - Doppler, V/Q scan, CTPA
178
What are the aims of management for venous thrombosis?
Prevent clot extension, embolisation and recurrence
179
How do you manage venous thrombosis with medication?
Anticoagulants (LMWH, Warfarin, DOACs) Thrombolysis only in selected cases e.g massive PE
180
What is heritable thrombophilia?
An inherited predisposition to venous thrombosis
181
What is the main scoring system for DVT?
Wells score
182
What should you do for a patient who has high pre-test probability for PE/DVT?
Straight to imaging
183
If there is compressibility of veins in ultrasound, what does this suggest?
No clot/occlusion
184
What is the treatment duration for clots?
Minimal of 3 months then after this, consider if it is long term.
185
List instances which DIC can occur in?
Sepsis Malignancy Eclampsia
186
What can DIC cause?
Gangrene | Organ failure
187
What confines clots? List 3 examples.
Natural anticoagulants 1. Tissue factor pathway Inhibitor 2. Activate protein C/S system 3. Antithrombin
188
What is the function of tissue factor and tissue factor pathway inhibitor?
Tissue factor = physiological activator, first coagulation factor released Tissue factor pathway inhibitor = flips and inactivates factors 7 and 10
189
What is the function of antithrombin?
Inactivates factors 8, 9, 10, 11 and thrombin
190
What is the function of activated protein C/S system?
Inactivates factor 5 and 8
191
How is fibrinolysis activated?
Endothelial cells release t-PA or u-PA which converts plasminogen to plasmin which breaks down the clot
192
Give an example of a fibrin degradation products.
D-Dimer
193
What does Warfarin inhibit?
Factors 4, 5, 7, 10 and prothrombin
194
What does Heparins inhibit?
10a and thrombin
195
What do DOACs inhibit?
10a
196
How does Dapigatran and Bivalirudin work?
Thrombin
197
List causes of hypercoaguable states.
``` Pregnancy Cancer Post-op HRT Combined pills ```
198
What does PT measure?
Prothombin time - measures integrity of extrinsic and common pathways How long it takes for blood to begin to form clots
199
What does PT help to diagnose?
Bleeding disorders and severity of liver disease
200
What factors are present in the primary haemostatic response?
Platelet plug vWF Wall
201
What factors are present in the secondary haemostatic response?
Fibrin plug formation
202
What is haemorrhage diathesis?
Any qualitative or qualitative abnormality
203
What can cause haemostatic defects?
Problems with platelets, vWF, coagulation factors
204
What points should you consider in a bleeding history?
``` Has patient got bleeding disorder? How sever is disorder? Pattern? Congenital or acquired? Mode of inheritance ```
205
What clinical signs might indicate history of bleeding?
``` Bruising Epistaxis Post-surgical bleeding Menorrhagia Post-trauma PPH ```
206
What surgeries are commonly associated with bleeding post-surgery.
Dental surgery Circumcision Tonsillectomy Appendicectomy
207
What is the best question to ask when taking a history of bleeding?
Post-surgical bleeding
208
Severe unprovoked internal bleeding is a sign of what?
Severe haemophilia
209
Where is bleeding seen if abnormalities in platelets/vWF?
``` Mucosal bleeding Epistaxis Purpura GI Menorhhage ```
210
If coagulation, factor deficiencies, what is the pattern of bleeding?
Articular Muscle haematoma CNS
211
Give an example of a condition which commonly presents with mucosal bleeding.
Severe thrombocytopenia
212
What pattern of inheritance is haemophilia A and B?
X-linked
213
In haemophilia A and B, what does severity of bleeding depend on?
Residual coagulation factor activity
214
What results from factor 8 deficiency?
Haemophilia A
215
What results from factor 9 deficiency?
Haemophilia B
216
What is the presentation of haemophilia?
``` Haemarthrosis Muscle haematoma CNS bleeding Retroperitoneal bleeding Post-surgical bleeding ```
217
What are common signs of haemarthrosis in haemophilia patients?
Key/worst joint = ankle Weight bearing joints Hinge joints have worst prognosis
218
Why does synovitis arise from joint bleeding?
macrophages eat blood --> inflammatory cytokines produced --> drives synovial hypertrophy --> lose joint cartilage --> endstage haemophilic arthropathy/OA
219
What are clinical complications of haemophilia?
Synovitis Chronic haemophilic arthropathy Neurovascular compression Stroke
220
How do you diagnose haemophilia?
Clinical Prolonged APTT Normal PT Reduced Factor VIII or Factor IX genetic Analysis - in utero if male or from cord blood at birth
221
How do you treat haemophilia, bleeding diathesis?
Coagulation factor replacement (factor 8 or 9) DDAVP - for mild haemophilia A and VW disease Tranexamic acid Gene therapy
222
Apart from medication, how is haemophilia managed?
``` Splints Physiotherapy Analgesia Synovectomy Joint replacement ```
223
What are complications of DDAVP use?
MI | Hyponatraemia
224
What viral infections are common complications haemophilia?
HIC HBV HCV vCJD
225
What type of bleeding is seen in von Willebrand disease?
Platelet type - mucosal
226
List the types of von Willebrand disease.
1. quantitative deficiency 2. qualitative, lots of antigens but under function 3. Complete deficiency
227
How do you manage vW disease?
vWF concentrate or DDAVP Tranexamic acid Topicals OCPs
228
List acquired bleeding disorders.
``` Thrombocytopenia Liver failure Renal failure DIC Drugs e.g Warfarin ```
229
What causes thrombocytopenia?
Under production or over consumptionof platelets
230
What can cause decreased production thrombocytopenia?
Marrow failure Aplasia Infiltration
231
What are clinical signs of thrombocytopenia?
Petechia Ecchymosis Mucosal bleeding Rare CNS bleeding
232
Where should you look at petechiae?
Ankles
233
What causes over consumption thrombocytopenia?
Immune ITP Non immune DIC Hypersplenism
234
What factors are produced in liver failure?
1, 2, 5, 7, 8, 9, 10, 11
235
What does prolonged PT and APTT signs of?
Liver failure
236
How do you prevent haemorrhage disease of the baby?
Injection of Vitamin K
237
What is pancytopenia?
Low WCC, Hb and platelets
238
What do spherocytes suggest?
Haemolysis
239
What does aPTT measure?
Intrinsic and common coagulation pathways Evaluates risk of bleeding before surgery For unexplained bleeding and clotting
240
What is required to convert fibrinogen to fibrin?
Thrombin
241
List roles of platelets.
Produce factors that will form a clot and provide phospholipid surface for end-stage coagulation Adhere Activate Aggregate
242
List the 3 receptors found on platelets.
ADP Thrombin adrenaline
243
List Glycoproteins on platelets.
GPIIb/IIIc GP Ib/V.IX GP Ia/IIb GP VI
244
What is the function of the open canalicular system?
Secrete granules (alpha and dense) from inside platelet surface which contain thrombin
245
What is the function of platelet receptors?
Released at areas of damage allowing platelets to activate and become sticky
246
What activates platelets and coagulation factors/
Abnormal epithelium surface and physiological activator
247
What is the first physiological activator released with tissue damage?
Tissue factor
248
What enzyme flips the platelet membrane so the phospholipid layer is on the outside?
Scramblase
249
Where does DOACs act?
GPIIb/IIIa pathway
250
Where does Aspirin act?
blocks COX pathway --> preventing conversion of arachidonic acid to thromboxane A2 therefore preventing platelet aggregation
251
Which drugs block the ADP pathway?
Clopidogrel Ticagrelor Prasegrel
252
What is the function of von Willebrand factor?
Allows platelets to adhere to damaged endothelium sites
253
What does von Willebrand Factor bind to?
Factor 8
254
What is the most common type of lymphoma.
Non-Hodgkin's
255
What is the pathogenesis of haematological malignancy?
Multi-step process Acquired genetic alterations in a long lived cell Proliferative/survival advantage to the mutated cell --> malignant clone --> grows to dominant
256
In what lineages to myeloid malignancies occur?
Down myeloid line from myeloid progenitor line
257
In what lineages to lymphoid malignancies occur?
Lymphoid line (lymphocytes)
258
What is the difference between leukaemia and lymphoma?
Leukaemia
259
what is the difference between acute and chronic leukaemia?
Acute - cells don't differentiate, bone marrow failure, rapidly fatal if untreated Chronic - cells retain ability to differentiate, proliferative bone marrow failure, survival for a few years
260
Is chronic leukaemia curable?
Potentially - Tyrosine kinase inhibitors
261
Why are germinal centres important in lymph nodes?
Location of immature B cells are programmed to respond to antigen and if they emerge --> they form memory cells
262
If lymph node localised and painful. What do you think?
Bacterial infection in draining site
263
If lymph node localised and painless. What do you think?
Rare infections e.g TB Metastatic carcinoma from drainage site (HARD) Lymphoma (rubbery) Reactive, no cause identified
264
If lymph node generalised and painful/tender. What do you think?
Viral infections e.g EBV, CMV, hepatitis, HIV
265
If lymph node generalised and painless. What do you think?
``` Lymphoma Leukaemia CT diseases e.g sarcoidosis Reactive, no cause identified Drugs ```
266
What lymphoma most commonly presents with nodal disease?
Hodgkin's lymphoma
267
What lymphoma most commonly presents with nodal disease?
Extranodal disease
268
List systemic symptoms of lymphoma.
``` Fever Drenching sweats Loss of weight Pruritis Fatigue ```
269
When do you prescribe cryoprecipitate?
Low fibrinogen
270
When do you prescribe FFP?
Low coagulation factors
271
When do you prescribe red cells?
Anaemia
272
When do you prescribe platelets?
Thrombocytopenia
273
72 yo lady, haematemesis, Hb 106g/L. Transfusion?
Not until you know BP and HR (Assess harm-dynamics)
274
76yo, Hb 54 and blood film consistent with megaloblastic anaemia. Transfusion?
No --> give B12 and folate
275
48yo lady admitted for a planned hysterectomy, Hb 95g/L. Transfusion?
No likely cause iron deficiency from dysmenorrhoea or malignancy
276
What is the new recommended baseline levels for transfusion?
Transfuse so haemoglobin kepyt about 70, except if pre-existing cardiac impairment (use 90 or 100 as baseline)
277
List indications for platelet transfusion.
``` Prophylactically or therapeutically to stop bleeding Dilutional thrombocytopenia Cardiopulmonary bypass surgery DIC if bleeding Abnormalities of platelet function ```
278
List indications for FFP.
Replace coat factors DIC Major haemorrhage Thrombotic Thrombocytopenia purpura
279
Do you transfuse a bleeding patient with factor VIII deficiency?
No - use factor concentrate
280
Do you transfuse a bleeding patient with factor V deficiency?
Yes - there isn't a factor 5 concentrate
281
Do you give bleeding patient on DOAC FFP transfusion?
No - use a direct inhibitor
282
Do you give a patient FFP who has a massive haemorrhage but no coagulation results?
Yes
283
Do you give FFP to a bleeding patients with DIC?
Yes
284
Do you give FFP to a patients with DIC?
No
285
What are indications for cryoprecipitate prescription?
Hypofibrinogenaemia DIC with bleeding and low fibrinogen Renal or liver failure and abnormal bleeding
286
Which samples do you send to the lab for blood?
Group and screen/save Cross match 'Group specific' blood Two sample policy
287
If patient is at risk of circulatory overload, what should you do when prescribing red cells?
Give 20mg oral furosemide
288
How do you prevent graft vs host disease when transfusing red cells to immunosuppressed patients?
Irradiate blood components (done for all platelets)
289
What are risks of transfusion?
``` Transfusion of ABO incompatibility TACO TRALI Hypotension nvCJD Hypotension Allergy ```
290
How do you treat urticaria from transfusion reaction?
Antihistamine
291
How do you treat SOB from TACO, TRALI and anaphylaxis from transfusion reaction?
Oxygen Diuretic Ventilation Adrenaline
292
How do you treat shock from transfusion reaction?
``` Adrenaline IV IV fluid ITU admission Abx FFP/Platelets if DIC ```
293
What are clinical features (triad) of AML?
Anaemia Thrombocytopenic bleeding (purport and mucosal membrane bleeding) Infection because of neutropenia
294
What are symptoms of AML associated with?
Bone marrow failure
295
What are essential investigations for AML?
FBC Blood film Bone marrow aspirate/trephine (>20% blasts in marrow) Cytogenetics from leukaemia blasts Immunophenotyping of leukaemia blasts CSF examination if symptoms (paediatrics) Targeted molecular genetics for associated acquired gene mutations Extended NGS myeloid gene panels
296
How do you treat AML?
Supportive care Anti-leukaemia chemotherapy - Danorubicin and cytosine arabinoside, high dose arabinoside, gemtuzumab, CPX-351 Allogenic stem cell transplant all-trans retinoid acid and arsenic trioxide in low risk acute prolyelocytic leukaemia
297
What are new developments for AML treatment/
Targeted Abs Targeted small molecules New chemotherapy delivery systems - CPX-351
298
What indicates remission achieved?
Blast count <5% | Blood cell count normal
299
What are signs of CML?
``` Anaemia Splenomegaly Weight loss Hyperleukostasis --> fundal haemorrhage and venous congestion, altered consciousness, respiratory failure Gout ```
300
What lab features will suggest CML?
Bone marrow and blood cells contain Philadelphia chromosome - t(9;22) Bone marrow hyper cellular Blood film shows all stages of WCC differentiation Increased basophils Anaemia High platelet count High WCC
301
How do you treat CML?
Tyrosine kinase inhibitors: Imatinib, Dasatinib, Nilotinib Busitinib Ponatinib Direct inhibitors of BCR-ABL protein
302
How can lymphoma present?
Enlarged lymph nodes Extranodal involvement Bone marrow involvement
303
What are systemic (B) symptoms of lymphoma?
Weight loss (>10% in 6 months), fever, night sweats, pruritis, fatigue
304
How is the diagnosis for lymphoma made?
Biopsy
305
How is lymphoma staged?
Clinical examination and imaging
306
Where do you classically find acute leukaemia?
Bone marrow, blood
307
Where do you classically find lymphomas and CLL?
Lymph nodes
308
Where do you classically find malignant melanoma?
Plasma cells in bone marrow
309
What type of lymphoma is a broad term which has high-grade and low-grade and around 70 subtypes?
Non-hodgkin lymphoma
310
Name a type of lymphoma that is a medical emergency and can double in size in 24 hours?
Burkitts
311
List lymphoproliferative disorders.
ALL CLL Hodgkin lymphoma Non-hodgkin lymphoma
312
What type of lymphoma is diffuse large B-cell lymphoma?
High-grade NHL
313
What is the most common lymphoproliferative disorders?
NHL (DLBCL)
314
17 year old male, 1 month impaired vision in both eyes, 1/2 stone weight loss, breathless on minimal exertion. Retinal haemorrhages, marked leukocytosis, thrombocytopenia, low platelets. Bone marrow (0% B lymphocytes. Diagnosis?
ALL
315
What are characteristic cells of ALL?
Large Express CD19 - all B cells have this CD34 TDT - markers of early immature cells
316
What is the standard treatment for ALL?
Combination chemo | Stem cells transplantation of high risk
317
Why do patients with ALL require CNS direct treatments - intrathecal methotrexate?
High risk of CNS relapse
318
List new therapies for ALL?
- Bi-specific T cell engagers e.g Blinatunumab | - CAR-T
319
How does CAR-T therapy work?
T-cells harvested, transfected to express a specific T cell receptor on leukaemia cells (CD19), expanded in vitro, re-infused into patient
320
What are key side effects of T cell immunotherapy?
Cytokine release syndrome Neurotoxicity
321
List poor risk factors for ALL.
Increasing age Increasing WCC Cytogenetics/molecular genetics (e.g philadelphia chromosome) Slow-poor response to treatment
322
What is the typical presentation of ALL?
Bone marrow failure +/- raised white cell count | Bone pain, infection, sweats
323
How are CLL cells different?
Abnormal cells are mature | Grow slowly
324
What is the most common leukaemia worldwide?
CLL
325
What is the lymphocyte count required to diagnose CLL?
>5
326
How does CLL present?
Often asymptomatic
327
What clinical findings may be found in CLL?
``` Bone marrow failure Lymphadenopathy Splenomegaly Fever and sweats Hepatomegaly Infections Weight loss ```
328
What staging system is used for CLL?
Binet (A,B,C)
329
What are indications of treatment fro CLL?
``` Progressive bone marrow failure Massive lymphadenopathy Progressive splenomegaly Systemic symptoms Autoimmune cytopenias Lymphocyte doubling ```
330
How do you treat CLL?
Often watch and wait Cytotoxic chemo Monoclonal antibodies Novel agents
331
What are poor prognostic markers of CLL?
``` Binet stage B or C CD38+ expression Atypical lymphocyte morphology Rapid lymphocyte doubling time p53 loss/mutation ```
332
How do patients with lymphoma present?
Lymphadenopathy Extranodal disease B symptoms Bone marrow involvement
333
Describe the staging system for lymphoma?
Ann Arbour staging 1 - localised, single lymph nodes 2 - 2 or more lymph node regions on same side 3 - 2 or more lymph node regions above and below diaphragm 4 - widespread, multiple organs, with or without lymph node involvement A = absence of B symptoms B = fever, night sweats, weight loss
334
What is the origin of most NHL?
B cell
335
What is the difference between low and high grade lymphoma?
Low = incurable, responds to chemo, often asymptomatic high = aggressive, fast-growing, combination chemo, can be cured
336
What are the two most common types of NHL?
Diffuse large B cell (high grade) | Follicular (low grade)
337
How are DLBCL and follicular lymphoma treated?
Combination chemo and antibody (rituximab)
338
List risk factors for Hodgkin Lymphoma?
EBV FH Geographiocal clustering
339
How is Hodgkin's lymphoma treated?
Combination chemo +/- monoclonal antibodies (anti-CD30) | Immunotherapy
340
How do you assess response to treatment of Hodgkin's lymphoma?
PET scan
341
What type of lymphomas are incurable?
Low grade NHL
342
What is CD5 a marker for?
T cell
343
List conditions associated with paraproteins.
Amyloidosis Waldenstroms macroglobulinaemia MGUS
344
What is a paraprotein?
A monoclonal immunoglobulin present in blood or urine
345
What does the presence of paraproteins indicate?
Indicates that somewhere in the body, a B cell or plasma cel is proliferating
346
What qualitative test identifies presence of paraproteins?
Serum protein electrophoresis (separates protein according to size and charge)
347
Summarise the tests for paraproteins.
Total immunoglobulin levels Electrophoresis (key to assess antibody diversity and show paraprotein) Immunofixation (identifies subclass of paraprotein) Light chains (assess imbalance)
348
What is the purpose of immunofixation?
Identifies class of paraprotein
349
What do IgM paraproteins indicate?
Lymphoma (since maturing B lymphocytes produce IgM antibodies)
350
What do IgG and IgA paraproteins indicate?
Myeloma (mature plasma cells generate these)
351
What is myeloma?
Neoplastic disorder of plasma cells resulting in excess production of a single type of immunoglobulin (paraprotein)
352
At what age does myeloma usually affect?
Elderly - over 70
353
What are clinical manifestations of myeloma?
CRAB hyperCalcaemia Renal failure Anaemia Bone disease
354
What bone disease features are associated with myeloma?
``` Lytic bone lesions Pathological fractures Cord compression Hypercalcaemia Infections Bone marrow failure ```
355
List effects of paraproteins.
Renal failure (cast nephropathy) Hyperviscosity Hypogammaglobulinaemia
356
What are clinical features of hyperviscosity?
Retinal, oral, nasal or cutaneous bleeding Cardiac failure, pulmonary congestion, confusion, renal failure
357
What causes amyloidosis?
Characterised by abnormal deposition of fibrillar protein
358
How can amyloidosis manifest/
``` Nephrotic syndrome LVH Carpal tunnel syndrome Autonomic neuropathy Cutaneous infiltration ```
359
What is MGUS?
Monoclonal gammopathy of uncertain significance --> paraproteins present but no effect
360
What is the most common type of paraprotein associated with myeloma?
IgG
361
How do you treat myeloma?
Chemotherapy (proteosome inhibitors,monoclonal antibodies) Bisphosphonate therapy Radiotherapy Steroids Surgery - pinning of long bones, decompression of spinal cord Autologous stem cell transplant
362
What are clinical presentations of IgM paraproteins?
Lymphoma - bone marrow failure - lymphadenopathy - heptosplenomegaly - B symptoms
363
What are B symptoms?
Fever, night sweats, weight loss
364
Patients with prolonged episodes of neutropenia get treated with what?
Antibiotics - ciprofloxacin (gram negative protection) Anti-fungals - fluconazole Anti-virals - acyclovir PJP - co-trimoxazole
365
What are supportive measures aimed at reducing sepsis in haematological malignancies?
``` G-CSF Stem cell resuce/transplant Protective environment IV immunoglobulin replacement Prophylaxis - antibiotics, anti-fungals, anti-virals ```
366
What causes neutropenia?
marrow failure (higher risk) or immune destruction
367
What are levels of neutropenia?
``` <0.5 = sig risk <0.2 = high risk ```
368
How long is high risk neutropenia?
> 7 days
369
What can result from disrupted skin/mucosal surfaces in neutropenic patients?
Mucositis | GVHD
370
What is the most common bacterial cause of febrile neutropenia?
Gram positive bacteria e.g MSSA, MRSA, strep viridian's, enterococcus faecalis, corynebacterium, bacillus
371
Name gram negative bacteria that cause 30-40% febrile neutropenia.
E.coli Klebseilla Pseudomonas aeruginosa
372
What are common fungal infections seen in immunocompromised patients?
Candida, aspergillus
373
What contributes to increased fungal infection risk in immunocompromised patients?
Monocytopenia and monocyte dysfunction
374
How does neutropenic sepsis present?
Fever with no localising signs >38.5 (1 reading) or >38 (2 readings) ``` Rigors Pneumonia Cellulitis UTI Septic shock ```
375
How do you investigate neutropenic fever?
Hx Ex Blood cultures - Hickman line and peripheral CXR Throat swab and other clinical sites of infections Sputum if productive FBC, U&Es, LFTs, Coag screen
376
How do you manage neutropenic sepsis?
Resuscitation Braod spectrum IV Abx - Tazocin, Gentamicin If gram +ve - vancomycin or teicoplanin If no response in 72 hours, add IV anti fungal e.g caspofungin CT chest, abdo, pelvis
377
What infections are commonly seen in severely lymphopenic patients?
Atypical pneumonia e.g PJP, CMV, RSV Viral e.g shingles, Herpes, adenovirus, EBV Fungal e.g candida, aspergillus
378
What is the best treatment regimen for boy with haemophilia A who develops inhibitory antibodies to factor 8?
Give factor 8 prophylaxis - small amounts every day or at least 3 times a day
379
What are future treatments for haemophilia?
``` Gene therapy Monoclonal Ab (Emicizumab) ```
380
What are petechiae, menorrhagia, buccal bleeding and epistaxis suggestive of?
Mucosal pattern of bleeding --> Thrombocytopenia
381
List causes of thrombocytopenia.
AML Aplastic anaemia (typically pancytopenia) IPT
382
How does ITP cause thrombocytopenia?
Normal production with increased consumption of platelets
383
What is the likely platelet count for spontaneous bruising and petechiae formation?
Less than 10
384
What illnesses are associated with immune thrombocytopenia?
HIV SLE Glandular fever
385
What increases VTE risk in combination with the COCP?
Factor V Leiden
386
List causes of thrombocytosis
``` Infection Post surgery/trauma Malignancy Iron deficiency Inflammation - IBD, RA Primary myeloproliferative disease ```
387
What are causes of lymphocytosis?
``` Viral infections Other infections - TB, Brucellosis, syphilis, vasculitis ALL CLL Lymphoma ```

Haematology and Immunology (2 decks)

Brainscape helps you reach your goals faster, through stronger study habits.
© 2025 Bold Learning Solutions. Terms and Conditions