Haematology Flashcards
1
Q
What is the origin of platelets? What type of storage granules do they contain?
A
Megakaryocytes- come from the common myeloid precursor in haematopoesis.
3 types of storage granules:
- alpha: contain clotting factors
- dense: contain mediators of platelet activation eg. serotonin
- glycogen- provide energy
2
Q
Which factors are involved specifically in the extrinsic pathway of secondary haemostasis?
A
Tissue Factor
Factor 7
3
Q
Which factors are involved specifically in the intrinsic pathway of secondary hemostasis?
A
Factor 12
Factor 11
Factor 8
Factor 9
4
Q
Which clotting factor is deficient in:
A- Hamophilia A
B- Hamophilia B
C- Hamophilia C
A
A- Factor 8
B- Factor 9
C- Factor 11
5
Q
Which of the clotting factors are vitamin k dependant?
A
2 (prothrombin), 7 (extrinsic), 9 (intrinsic) and 10
6
Q
How does Warfarin work?
A
Prevents intrinsic and extrinsic clotting cascade by inhibiting the synthesis of Vitamin K dependent clotting factors (Prothrombin, 7, 9 and 10)
7
Q
How does Apixaban work?
A
Prevents intrinsic and extrinsic clotting cascades by inhibiting factor 10a.
8
Q
What is fibrinolysis?
A
The process by which the fibrin clot is degraded.
9
Q
How does the body prevent unwanted clots?
A
- Protein C in the venous circulation (activated by thrombin) degrades 5a and 8a.
- Natural heparins on the endothelial cell surfaces enhance the release of prostacyclin, and also the activity of anti-thrombin.
10
Q
What are the 2 main blood tests for clotting?
A
- Prothrombin time- tests the extrinsic pathway
2. Activated partial prothrombin time- tests the intrinsic pathway.
11
Q
What normally prevents platelets from adhering to endothelial cells?
A
- Nitrous oxide
- Prostacyclin
PREVENT platelets from adhering to endothelial cells.
12
Q
What are acanthocytes?
A
RBCs with spikes on surface
13
Q
In which conditions may acanthocytes be present in the blood?
A
Splenectomy
Alcoholic liver disease
Spherocytosis
14
Q
What are blasts?
A
Nucleated precursors
15
Q
In which conditions may blasts be present in the blood?
A
Leukaemia
Myelofibrosis
16
Q
What are Burr cells and In which conditions may they be present in the blood?
A
RBCs with surface projections, present in:
Renal failure
Liver failure
17
Q
What is a Cabot ring and in which conditions may they be present in the blood?
A
Thin red/ purple ring in a RBC, present in:
Pernicious anaemia
Lead poisoning
Bad infections
18
Q
Which condition may have cells with Auer rods present?
A
AML
19
Q
Which conditions have hypochromic RBCs?
A
Iron deficiency anaemia
Thalassaemia
20
Q
What are the blood film features in iron deficiency anaemia?
A
- Pencil cells
- Hypochromic RBCs
- Target cells
- Microcytic RBCs
- Decreased ferritin
21
Q
What are the blood film features in haemolytic anaemia?
A
- Spherocytes
- Reticulocytes
- Tear shaped RBCs
- Normocytic/ macrocytic
22
Q
What are the blood film features in B12/ folate deficiency?
A
- Macrocytic RBCs
2. Hypersegmented neutrophils (6-8 lobes)
23
Q
When are tear drop shape RBCs found?
A
Extra-medullary haematopoeisis eg. Myelofibrosis, haemolysis
24
Q
When are target cells seen?
A
- Iron deficiency anaemia
- Thalassaemia
- Liver disease
- Hyposplenism
25
What are spherocytes and in which conditions may they be present?
Spherical hyperchromic RBCs:
1. Hereditary spherocytosis
2. AIHA
26
What are schistocytes and in which conditions may they be present?
Fragmented RBCs which have been sliced by fibrin
DIC
Thrombotic thrombocytopenic purpura
27
What are reticulocytes and in which conditions may they be present?
Large young RBCs
| Haemolytic anaemia
28
How are blood tests affected in DIC?
```
APTT increased
PT (INR) increased
TT increased
D-dimer increased
Platelet count decreased
Presence of schistocytes
```
29
How are blood tests affected in Haemophilia?
APTT increased
30
How are blood tests affected in VWD?
APTT increased
31
How are blood tests affected in Vitamin K deficiency?
PT and APTT increased
32
How are blood tests affected in liver disease?
PT increased
APTT increased
Platelet count may decrease
33
What are the characteristics of Haemophilia?
Bleeding into joints
| Bleeding into muscles
34
What are the characteristics of Von Willebrand disease?
Bleeding
Epistaxis
Usually less severe than Haemophilia
35
What is desmopressin used for?
Managing haemophilia and Von Willebrands disease
36
Which triad may be present in Haemochromatosis?
1. Tanned skin
2. Cirrhosis
3. Diabetes
37
What is haemochromatosis?
Excessive intestinal absorption of iron
| Iron overload and accumulation in tissues (liver, adrenals, heart, skin, pancreas)
38
Which gene is usually mutated in hereditary haemochromatosis and how is this inherited?
Autosomal recessive inheritance- mutations in HFE gene
39
Which factors promote fibrinolysis?
1. Thrombin induces the release of TPA which converts plasminogen to plasmin. Plasmin helps to break the fibrin mesh
2. Thrombin also stimulates the production of anti-thrombin: which inhibits the production of thrombin and factor 10a, 9a and 11a.
40
Which myeloproliferative neoplasm affects WBCs?
CML
41
Which myeloproliferative neoplasm affects RBCs?
Polycythaemia vera
42
Which myeloproliferative neoplasm affects platelets?
Essential thrombocythaemia
43
Which myeloproliferative neoplasm affects fibroblasts?
Myelofibrosis
44
Most myeloproliferative neoplasms have an abnormality in which gene?
JAK2
45
Which myeloproliferative neoplasm may present with headaches, facial plethora, burning sensation in fingers and toes, and tinnitus?
Polycythaemia vera
46
In polycythaemia vera, there is excess production of ______
RBCs
WBCs
Platelets
47
In polycythaemia vera there is increased risk of __________
```
Hyperviscosity
Venous thrombi (DVT, PE), arterial thrombi (MI)
```
48
Other than polycythaemia vera, what are the other causes of polycythaemia (increased RBC production)?
1. Hypoxia
| 2. Excess EPO hormone
49
How can polycythaemia vera be treated?
1. Venesection
2. Aspirin
3. Hydroxycarbamide for thrombocytosis
50
What are the 3 main consequences of essential thrombocythaemia?
1. Bleeding
2. Thrombi (Arterial or venous)
3. Microvascular occlusion
51
Microvascular occlusion is a consequence of thrombocythaemia and can cause symptoms such as.....
```
Headache
Blurred vision
Chest pain
Digital ischemia
Burning in fingers/ toes (erythromelalgia)
```
52
What are the differentials for essential thrombocythaemia?
Cancer
Chronic inflammation
Past surgery
53
The BCSH criteria are used for diagnosing.....
Essential thrombocythaemia
54
Essential thrombocythaemia can be treated using ..........
1. Aspirin
| 2. Hydroxycarbamide
55
In __________ there is clonal proliferation of megakaryocytes causing platelet counts of >1000 with abnormal functioning platelets.
Essential thrombocythaemia
56
What is myelofibrosis?
Clonal proliferation of fibroblasts
Bone marrow fibrosis
Leads to extramedullary haematopoiesis (spleen and liver)
57
What are the consequences of myelofibrosis?
1. Anaemia
2. Infection
3. Extramedullary haematopoeisis
58
If a patient presented with the following symptoms what may the diagnosis be?
B symptoms (fever, night sweats, weight loss)
Anaemia
Infections
Enlarged spleen
Gout
Myelofibrosis
59
How does myelofibrosis appear on blood films?
1. Tear drop shape RBCs
| 2. Nucleated RBCs (leukoerythroblastic cells)
60
Ruxolitinib is an oral JAK2 inhibitor, used for managing ___________. It causes decreased nightsweats and reduction in splenomegaly.
Myelofibrosis
61
The BCR:ABL translocation is most commonly present in which condition?
CML
62
How can AML and CML be distinguished on blood film?
AML:
1. Auer rods
2. Myeloblasts
3. Monoblasts
CML:
1. High cell count (hypercellular marrow)
2. Raised eosinophils
3. Raised basophils
63
Which type of leukaemia is most common in children?
ALL
64
What are the 3 phases of CML?
1. Chronic phase
2. Accelerated phase
3. Blast transformation
65
What are the myelodysplastic syndromes?
Group of disorders which present as bone marrow failure (anaemia, neutropenia and thrombocytopenia)
66
What is the pathogenesis of myelodysplastic syndromes?
MDS arises in haematopoietic stem cells
Abnormal cells produced due to a partial block in differentiation
Blasts may accumulate
67
How can myelodysplasia be diagnosed on blood film?
Pancytopenia
Increased marrow cellularity
Ring sideroblasts
68
How may myelodysplasia be treated?
1. Transfusion of RBCs or platelets
2. EPO
3. G-CSF
4. Allogenic stem cell transplant
69
If a patient presented with the following 3 symptoms what may be the diagnosis?
1. Jaundice
2. Hepatosplenomegaly
3. Pigment gallstones
Haemolytic anaemia
70
What is the direct Coombs test used for?
Autoimmune haemolytic anaemia
detects antibodies for RBCs. Agglutination = positive test result
71
When is the indirect Coombs test used?
Prenatal testing
Before blood transfusion
Detects anti RBC antibodies free in serum
72
Warm AIHA is mediated by ____
IgG
73
Cold AIHA is mediated by ____
IgM
74
How is warm autoimmune haemolytic anaemia treated?
1. Steroids
2. Rituximab
3. Potential splenectomy
75
How is cold autoimmune haemolytic anaemia treated?
1. Keep warm
2. Blood transfusions
3. Chlorambucil
76
What are the ACQUIRED types of haemolytic anaemia?
1. Autoimmmune (warm or cold)
2. Drug induced
3. Infection
4. Microangiopathic haemolytic anaemia
77
What are the HEREDITARY types of haemolytic anaemia?
1. Enzyme defects eg. GP6D deficiency, pyruvate kinase deficiency
2. Membrane defects eg. Hereditary spherocytosis
3. Haemogobinopathys eg. Thalassaemia, Sickle cell
78
Which type of haemolytic anaemia will be Coombs test POSITIVE?
Autoimmune haemolytic anaemia (acquired)
79
What happens to iron, ferritin and total binding capacity in IDA?
Iron decreases
Ferritin decreases
TIBC increases
80
What happens to iron, ferritin and total binding capacity in anaemia of chronic disease?
Iron decreases
Ferritin increases
TIBC decreases
81
What happens to iron, ferritin and total binding capacity in haemolytic anaemia?
Iron increases
Ferritin increases
TIBC decreases
82
_______ inhibits iron absorption by binding ferroportin, causing it to become internalised.
Hepcidin
83
In inflammation/ infection, Il-6 is released, causing Hepcidin to be switched ___
ON
84
Sickle cell anaemia is caused by a genetic polymorphism in the _______ gene, causing Glu -> Val translocation, leading to HbA -> HbS.
Beta globin
85
What happens to HbS when it is deoxygenated?
It polymerises, causing RBCs to deform and block small vessels, leading to ischemic pain.
86
What are the potential complications of sickle cell anaemia?
1. Vaso-occlusive crises
2. Infections
3, Hyposplenism- pneumococcus, meningococcus
4. Acute chest syndrome
5. End organ damage
87
Which type of Thalassaemia is more common?
Beta thalassaemia
88
When does Beta Thalassaemia usually present?
Aged under 1 year
Newborn has HbF but this declines in levels
Present with severe anaemia and failure to thrive
89
Alpha thalassaemia major/ Hb Barts usually results in _________
Death in utero
90
What are some inherited causes of thrombophilia?
1. Protein C & S deficiency
2. Factor V Leiden
3. Anti thrombin deficiency
91
What are some acquired causes of thrombophilia?
1. Oral contraceptive pill
| 2. Antiphospholipid syndrome
92
What is myeloma?
Monoclonal proliferation of plasma cells in bone marrow
| Leads to IgG/ IgA secretion (paraprotein) which can't function properly
93
What are the clinical features of myeloma? (CRABI)
```
C- Hypercalcaemia
R- Renal impairment
A- Anaemia, neutropenia and thrombocytopenia
B- Bone pain and destruction
I- Infection
```
94
How can myeloma be diagnosed?
1. Monoclonal protein band on electrophoresis of blood or urine
2. Plasma cells >10% in marrow
3. Organ damage: anaemia, hyperkalaemia, renal failure
4. Xrays: bone lesions
95
What are the complications of myeloma?
1. Hypercalcaemia: renal stones, brittle bones
2. Anaemia
3. Spinal cord compression
4. Hyperviscosity
5. AKI
96
Which bones does myeloma often affect?
Pelvis
Spine
Ribs
Shoulders
97
How can myeloma be treated?
1. Analgesia
2. Bisphosphonates (Zoledronate) slow down osteoporosis
3. Blood transfusions
4. Iv immunoglobulins
5. Chemotherapy (Lenalidomide, Dexamethosone, Velcade)
6. Autologous stem cell transplant
98
Give examples of microcytic anaemia.
Iron deficiency anaemia
Haematinic deficiencies- sickle cell, thalassaemia
Lead poisoning
Anaemia of chronic disease
99
Give examples of normocytic anaemia
```
Anaemia of chronic disease
Bone marrow failure
Acute blood loss
Renal failure
Aplastic anaemia
```
100
Give examples of macrocytic anaemia.
```
Megaloblastic: B12 or folate deficiency
Alcohol
Hypothyroidism
Haemolytic anaemia
Liver disease
Myelodysplasia
Serum paraprotein
```
101
Monoclonal gammopathy of unknown significance significance usually preceeds _________.
Myeloma
102
What is the lower limit of the Hb reference range for women?
120g/L
103
What is the lower limit of the Hb reference range for men?
130g/L
104
Which protein effluxes iron into the blood from enterocytes?
Ferroportin
105
What are the causes of iron deficiency anaemia?
Poor dietary intake
Poor absorption: Crohn's, coeliac
Iron loss: GI bleeding, menorrhagia
Increased requirement: pregnancy
106
Which drugs may cause folate deficiency?
Methotrexate
| Phenytoin
107
The majority of B12 is absorbed by binding ________ and being absorbed in the terminal ileum.
Intrinsic factor
108
What is pernicious anaemia?
Autoimmune gastritis meaning a lack of intrinsic factor secreted from parietal cells
Poor absorption of Vitamin B12
109
Which blood group is the universal donor of RBCs?
Group O neg
110
Which blood group is the universal recipient of RBCs?
Group AB positive
111
What is the most common blood group in Caucasians?
Group O
112
Which Ig antibodies against RBCs cause extravascular haemolysis slowly?
IgG
113
Which Ig antibodies against RBCs cause acute and dramatic intravascular haemolysis?
IgM
114
How does forward blood typing work?
Patients RBCs added to antibodies
If agglutination appears = patients RBCs has those antigens
ANTIGEN SCREENING
115
How does reverse blood typing work?
Patients plasma is added to RBCs
Used to detect which antibodies are in the patients serum
ANTIBODY SCREENING
116
Which type of blood product is stored for <35days at 4degrees?
RBCs
117
Which type of blood product is stored for <7days at 22degrees?
Platelets
118
Which type of blood product is stored frozen?
Plasma
119
What is the volume of the average bag of donor blood?
274ml
120
What are the 4 types of blood product that can be transfused?
1. RBCs
2. Platelets
3. Plasma (FFP)
4. Cryoprecipitate (frozen fibrinogen replacement)
121
How long should a bag of RBCs be transfused over?
2-3 hours
122
How long should a bag of platelets be transfused over?
30 mins
123
What is the definition of major haemorrhage?
1. Loss of >50% total blood in <3 hours
2. Loss of whole blood volume over 24 hours
3. Loss of >150ml/minute
124
What are the 3 most SERIOUS risks of blood transfusion?
1. ABO incompatibility
2. Bacterial contamination
3. Anaphylaxis
125
What are the 3 most COMMON risks of blood transfusion?
1. Transfusion associated circulatory overload (TACO)
2. Allergy
3. Febrile non haemolytic transfusion reaction (delayed)
126
How many pooled donations are used in a bag of platelets?
4
or 1 apheresis
127
How many pooled donations are used in a bag of cryoprecipitate?
2
128
Which haematological cancer presents with B symptoms, cervical/axillary lymphadenopathy and alcohol related lymph node pain?
Hodgkin's lymphoma
129
The Ann Arbor system is used to stage which haematological cancer?
Hodgkin's lymphoma
130
Which chemotherapys are used for Hodgkin's lymphoma?
A- Adriamycin
B- Bleomycin
V- Vinblastine
D- Dacarbazine
131
Which drugs make up the RCHOP regimen for high grade NHL lymphoma treatment?
```
R- Rituximab
C- Cyclophosphamide
H- Hydroxydaunomycin
O- Oncovin (Vincristine)
P- Prednisolone
```
132
Rituximbab and Chlorambucil are used to treat low grade ________
Non Hodgkin's Lymphoma
133
Thrombopoeitin usually stimulates __________
Platelet production
134
Which clotting factor does von willebrand bind?
Factor 8
135
What is aplastic anaemia?
Bone marrow failure
| Bone marrow stem cells disappear leaving fat spaces
136
Serum LDH is raised in which type of anaemia?
Haemolytic anaemia
137
Which antibody is secreted in breast milk?
IgA
138
IgA deficiency leads to an increased susceptibility to which type of bacteria?
Giardia lamblia
139
Bernard-Soulier syndrome is an autosomal recessive bleeding disorder caused by a deficiency in the platelet glycoprotein complex Ib-IX-V, which would normally bind to __________________________ to allow platelet adherence.
von Willebrand factor (vWF)
140
Thrombocytopenia is ______
Abnormally low numbers of platelets
141
DiGeorge syndrome involves failure of the thymus and can be rememberd by the mnemonic CATCH:
```
C-Cardiac abnormalities
A- Abnormal facial expression
T- Thymus failure
C- Cleft palate
H- Hypoparathyroidism
```
142
Ring Sideroblasts are a feature of which condition?
Myelodysplastic syndromes
143
What is the chance of contracting HIV from a needle stick injury?
0.3%
144
What is the chance of contracting HepC from a needle stick injury?
3%
145
What condition presents with:
1. Neurological symptoms (headache, confusion)
2. AKI
3. Fever
4. Low platelets
5. Microangiopathic haemolytic anaemia
Thrombotic thrombocytopaenic purpura
146
Smear cells on blood film are characteristic of which haematological cancer?
CLL
147
CD19, CD10 and CD5 are markers of which type of cell?
Lymphocytes
148
Pale proliferation centres and an increase in lymphocytes is associated with which haematological cancer?
CLL
149
Lenalidomide, Dexamethosone and Velcade are used for which haematological disorder?
Myeloma
150
ADAMTS13 is attacked, preventing its maintenance breakage of VWF in which condition?
TTP
