Haematology Flashcards

1
Q

Heinz Bodies

A

G6PD deficiency

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2
Q

Mechanism of action: Dabigatran

A

Direct thrombin inhibitor

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3
Q

Mechanism of action: Heparin

A

Antithrombin III

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4
Q

Mechanism of action: Rivaroxiban/Apixaban

A

Direct factor Xa inhibitor

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5
Q

Mechanism of action: Warfarin

A

Inhibits clotting factors II, VII, IX and X

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6
Q

In which 2 conditions is spherocytes found

A

autoimmune haemolytic anaemia as well as hereditary spherocytosis

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7
Q

EMA blood testing

A

hereditary spherocytosis

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8
Q

direct Coombs test

A

autoimmune haemolytic anaemia

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9
Q

Indirect Coombs test

A

Rhesus haemolytic disease of the newborn

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10
Q

‘Tear-drop’ poikilocytes

A

Myelofibrosis

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11
Q

Howell-Jolly bodies

A

hyposplenism

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12
Q

Anaemia caused by cytoplasmic defects. MCV. Causes.

A

Microcytic Anaemia - caused by a shortage of Hb components

  • Iron deficiency
  • Porphyrin deficiency
  • Congenital Sideroblastic Anaemia
  • Thalassaemia
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13
Q

Anaemia caused by nuclear defects. MCV. Causes.

A

Macrocytic Anaemia

Megaloblastic - large nucleated RBCs
- B12 and folate deficiency

Non-megaloblastic - alcoholic, liver disease hypothyroidism, marrow failure

Spurious - macrocytosis

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14
Q

Alpha thalassaemia with 3 defected genes

A

HbH disease (Hb with 4x B globin chains –> golf ball appearance)

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15
Q

Alpha thalassaemia with 4 defected genes

A

Hb barts hydrops fetalis

  • Hb barts (4x gamma chains), and
  • HbH (4x B globin chains –> golf ball appearance)

incompatible with life

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16
Q

High HbA2; low MCV

A

Beta Thalassaemia trait

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17
Q

Presentation of Major Beta Thalassaemia

A

Presents at 6-24 months (as HbF falls) with pallor and failure to thrive

requires transfusions

Skull X-ray –> “hair on end” appearance

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18
Q

Skull X-ray –> “hair on end” appearance

A

Major Beta Thalassaemia

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19
Q

What is HPLC used for?

A

Thalassaemia diagnosis

B trait –> excess HbA2
B major –> Some HbF, Some HbA2, No HbS, No HbA
A trait –> normal (use DNA testing)
Sickle Cell –> HbS

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20
Q

Aetiology, Features and Management of sickle cell crisis

A

Caused by hypoxia, infection or stress

features incl.

  • hyposplenism caused by infarcts
  • chronic haemolysis
  • widespread infarcts

Manage with oxygen, analgesia, antibiotics (if there is an infection), transfusion

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21
Q

Diagnosis of autoimmune haemolysis

A

Coomb’s test

  • Warm (IgG) e.g., SLE, Penicicllins, infection, CLL
  • Cold (IgM)e.g., TB, EBV
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22
Q

Features of haemochromatosis

A

Weakness, Arthritis, Arthralgia, Fatigue, Impotence, Cirrhosis, Diabetes, Cardiomyopathy

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23
Q

Causes of prolonged Prothrombin Time

A

Mild Liver disease
Vitamin K deficiency
Factor VII deficiency
Anticoagulation therapy with warfarin

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24
Q

Causes of prolonged APTT

A
Factor VIII/XI deficiency 
Haemophilia A (VIII deficiency) or B (IX deficiency)
vWF deficiency 
Anti-phospholipid syndrome
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25
Causes of both prolonged PT and APTT
Severe liver disease FI/II/V/X deficiency DIC
26
What does vitamin K do?
Carboxylates FII(prothrombin), VII, IX, X Remember... Factors 2, 7, 9, 10
27
Management of DIC
Platelets, FFP (clotting factors), Cryoprecipitate (fibrinogen and factor VIII)
28
Inheritance of Haemophilia
X-linked
29
Knee Haemarthrosis with no Hx of trauma - investigate for ...
Haemophilia
30
Management of Haemophilia
Lifelong IV factor concentrates
31
When would you screen for thrombophilia?
VTE in <45yo
32
Monitoring for LMWH
Anti-Xa Assay
33
Monitoring for Unfractionated Heparin
APTT
34
Monitoring for Warfarin
INR and PT
35
Mechanism of Aspirin
COX-1 inhibitor
36
Mechanism of Clopidogrel
ADP receptor (P2Y12) antagonist
37
Mechanism of Dipyridamole
Phosphodiesterase inhibitor
38
Mechanism of Abciximab
GPIIb/IIIa inhibitor - used in cardiac surgery
39
Macro-ovalocytes
Megaloblastic Anaemia (B12/Folate deficiency)
40
Hyper-segmented Neutrophils
Megaloblastic Anaemia (B12/Folate deficiency)
41
Cigar or Pencil Cells
Iron Deficiency
42
Blood film. 'rouleaux formation'
Myeloma
43
Features of Myeloma (CRAB)
C - calcium raised R - renal failure A - anaemia (technically a pancytopenia) B - bone pain
44
When should lithium levels be checked after a dose change?
7 days; 12 hours since last dose
45
Reed-Sternberg cell AKA 'mirror image nuclei'
Hodgkin's Lymphoma
46
Features of histological aggression
``` increased N:C open chromatin more primitive cells larger cells prominent nucleoli rapid proliferation ```
47
itch without a rash and alcohol induced pain
Hodgkin's disease
48
Hard tender lymphadenopathy with skin inflammation
bacterial
49
Hard tender lymphadenopathy with no skin inflammation
viral
50
Hard non-tender irregular lymphadenopathy
Metastatic carcinoma
51
Soft rubbery non-tender lymphadenopathy
Lymphoma
52
What is the difference in immunohistochemistry and immunophenotyping with respect to investigating haematological malignancies?
Immunohistochemistry - analysis of surface proteins to classify lymphoma Immunophenotyping - used for cells in liquid phase e.g., leukaemia, Burkitt's Lymphoma
53
CD30+ Lymphoma
Hodgkin's Disease
54
CD20+ Lymphoma
Non-Hodgkin's Lymphoma
55
Biological treatment for NHL
Ibrutinib
56
Subtypes of Non-Hodgkin's Lymphoma
T-cell (10%) | B-cell (90%)
57
Features of Pancytopaenia
Anaemia - fatigue, shortness of breath Neutropaenia - infections (particularly Gram -'ve) Thrombocytopenia (bleeding, purpura, petechiae)
58
Fanconi's Anaemia. features
Short stature, skin pigmentation, GI/Renal/CV and haematological abnormalities presents around 7 years old with bone marrow failure high risk of leukaemia
59
What does myelodysplastic syndrome put you at risk of?
AML
60
Causes of hypercellular marrow?
Myelodysplastic Syndrome B12/Folate deficiency Hypersplenism
61
Causes of hypocellular marrow?
Aplastic anaemia
62
What gives rise to immunoglobulin variability?
VDJ recombination
63
Features of Myeloma (CCRABI)
Use the mnemonicCRABBI: Calcium Hypercalcaemia occurs as a result of increased osteoclast activity within the bones This leads to constipation, nausea, anorexia and confusion Renal Monoclonal production of immunoglobulins results in light chain deposition within the renal tubules This causes renal damage which presents as dehydration and increasing thirst Other causes of renal impairment in myeloma include amyloidosis, nephrocalcinosis, nephrolithiasis Anaemia Bone marrow crowding suppresses erythropoiesis leading to anaemia This causes fatigue and pallor Bleeding bone marrow crowding also results in thrombocytopenia which puts patients at increased risk of bleeding and bruising Bones Bone marrow infiltration by plasma cells and cytokine-mediated osteoclast overactivity creates lytic bone lesions This may present as pain (especially in the back) and increases the risk of fragility fractures Infection a reduction in the production of normal immunoglobulins results in increased susceptibility to infection
64
Features of MGUS
benign paraproteinaemia , precursor to myeloma, asymptomatic but may have demyelinating neuropathy, protein <30g/L
65
Most common myeloma subtype
IgG
66
Congo red staining -> apple-green birefringence
Amyloidosis
67
Most common adult leukaemia
CLL
68
Smudge cells on blood film
CLL
69
Richter's transformation
CLL->NHL high grade
70
BCR-ABL1+ (Philadelphia Chromosome)
CML
71
Biological treatment of CML
TK inhibitors e.g., Imatinib
72
Myeloproliferative disorders
BCR-ABL1 -ve - Myelofibrosis - Polycythaemia Rubra Vera - Essential Thrombocytopaenia BCR-ABL1 +ve - CML
73
Pruritis after hot bath
Polycythaemia Rubra Vera
74
JAK2 positive in 95%
PRV
75
Management of polycythaemia rubra vera
venesection (until Hct <0.45) is first line could add aspirin could add hydroxyurea(hydroxycarbamide)
76
burning sensation in the hands + thrombosis + haemorrhages
Essential Thrombocytosis
77
Teardrop poikilocytes
Myelofibrosis
78
Management of Myelofibrosis
Supportive - JAK 2 inhibitor e.g. ruloxitinib | Definitive - stem cell transplantation
79
Investigations show a prolonged bleeding time and increased APTT. Normal PT and platelet count.
vWF disease
80
Management of acquired haemophilia
Steroids
81
Main difference between TRALI and TACO?
TACO - hypertensive | TRALI - hypotensive
82
Management of TACO
Slow or stop transfusion Consider intravenous loop diuretic (e.g. furosemide) and oxygen
83
Which haematological condition occurs in 30% of individuals with coeliac disease?
Acquired Hyposplenism
84
Blood smear results associated with hyposplenism
target cells and howell-jolly bodies
85
Causes of a Acidosis with a normal anion gap
Remember: H.A.R.D Hyperchloraemia Addison's disease Renal tubular acidosis Diarrhoea
86
Biochemistry seen in early myeloma
High calcium, normal phosphate, normal ALP
87
Management of Warfarin: Major Bleeding
Stop warfarin Give intravenous vitamin K 5mg Prothrombin complex concentrate - if not available then FFP*
88
Management of Warfarin: INR > 8 with minor bleeding
Stop warfarin Give intravenous vitamin K 1-3mg Repeat dose of vitamin K if INR still too high after 24 hours Restart warfarin when INR < 5.0
89
Management of Warfarin: INR > 8 with NO bleeding
Stop warfarin Give vitamin K 1-5mg by mouth, using the intravenous preparation orally Repeat dose of vitamin K if INR still too high after 24 hours Restart when INR < 5.0
90
Management of Warfarin: INR 5.0-8.0 | Minor bleeding
Stop warfarin Give intravenous vitamin K 1-3mg Restart when INR < 5.0
91
Management of Warfarin: INR 5.0-8.0 | with NO bleeding
Withhold 1 or 2 doses of warfarin | Reduce subsequent maintenance dose
92
Management of VWF disease
tranexamic acid for mild bleeding desmopressin (DDAVP): raises levels of vWF by inducing release of vWF from Weibel-Palade bodies in endothelial cells factor VIII concentrate
93
Prolonged bleeding time, normal platelets, prolonged APTT
VWF disease
94
Fatigue, prolonged bleeding and thrombocytopaenia
Immune thrombocytopenic purpura (ITP)
95
Management. Immune thrombocytopenic purpura (ITP)
Oral Prednisolone | pooled normal human immunoglobulin (IVIG) - for active bleeding
96
Monitoring for LMWH e.g. Dalteparin
Anti Factor Xa
97
Adverse effects of Heparin
bleeding thrombocytopenia osteoporosis and an increased risk of fractures hyperkalaemia - this is thought to be caused by inhibition of aldosterone secretion
98
Which blood product is most likely to result in septicaemia?
Platelets - stored at room temperature
99
Features of Factor V Leiden
Most common thrombophilia (5% in UK) | Protein C resistance
100
Characteristic iron study profile in haemochromatosis
Raised transferrin saturation Raised ferritin Low TIBC