Haematology Flashcards

(125 cards)

1
Q

What is the definition of anaemia?

A

Low concentration of haemoglobin

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2
Q

Give two signs of anaemia

A

Tachycardia
Dyspnoea
Pallor
Angina

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3
Q

What is the structure of haemoglobin A (HbA)?

A

4 Globular subunits
2 Alpha + 2 Beta

An Fe is bound to each subunit

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4
Q

How does foetal Hb differ from adult Hb in structure?

A

Foetal Hb has 2 Alpha and 2 Gamma subunits.

[vs. 2 Alpha and 2 Beta for adult]

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5
Q

True or false, adults have no foetal haemoglobin?

A

False. Around 2-4% of adult Hb is foetal Hb.

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6
Q

Give two causes of micro, normo and macrocytic anaemia

A

Micro: Thalassaemia, Iron deficiency, Chronic disease

Normo: Blood loss, Haemolysis.

Macro: B12/Folate deficiency, Alcohol.

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7
Q

Hypothyroidism is always macrocytic, true or false?

A

False. It can be normocytic or macrocytic.

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8
Q

In iron deficiency what happens to ferritin and transferrin levels? Which is the gold standard test?

A

Ferritin - Low
Transferrin - High

Ferritin is the best diagnostic test

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9
Q

True or false, infection or inflammation can cause Ferritin levels to increase

A

True

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10
Q

What inheritance pattern does Sickle Cell Disease follow?

A

Autosomal Recessive

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11
Q

Which species of malaria does Sickle Cell make people resistant to?

A

P falciparum

P vivax

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12
Q

What is the amino acid change caused by the SCD mutation?

A

A –> T
Glutamine –> Valine

[End up with TV]

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13
Q

Give an investigation you would perform to diagnose sickle cell disease?

A

Blood film
Reticulocyte count
FBC

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14
Q

Give two managements for acute sickle cell crisis

A

Analgesia (paracetamol/ibuprofen)

Oxygen

Foetal Hb

Fluids

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15
Q

Give two signs of Sickle Cell Disease

A
Priapism
Frequent infection
Anaemia (normocytic)
Jaundice
Visual loss
Haematuria
Splenomegaly
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16
Q

What is TACO?

A

Transfusion Associated Circulatory Overload

Acute dyspnoea, hypertension and oedema caused by blood transfusion.

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17
Q

What is the long term treatment for sickle cell disease?

A

Regular blood transfusions to keep sickle Hb <30%.

Hydroxyurea (reduces the frequency of crises)

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18
Q

What kind of anaemia does thalassaemia cause?

A

Microcytic

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19
Q

What inheritance pattern does Thalassaemia follow?

A

Autosomal Recessive

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20
Q

What investigations are done to diagnose thalassaemia?

A

FBC

Electrophoresis

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21
Q

What is the treatment for thalassaemia?

A

Blood transfusions +iron chelation to prevent TACO

Bone marrow transplant

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22
Q

True or false, thalassaemia provides some protection against malaria?

A

True.

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23
Q

Name two cell types of myeloid origin and two of lymphoid

A

Myeloid

  • Basophils
  • Eosinophils
  • Monocytes
  • Neutrophils
  • Erythrocytes
  • Megakaryocytes

Lymphoid (learned immunity)

  • B/T lymphocytes
  • NK cells
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24
Q

How big is a red blood cell?

A

5-10 microns in diameter

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25
True or false, reticulocytes are smaller than mature red blood cells?
False. They are larger.
26
What is the life span of a red blood cell?
120 days | 4 months.
27
What hormone stimulates the production of red blood cells? Where is it produced?
Erythropoietin (EPO) | Kidney in response to low O2 levels.
28
What deficiency is present in pernicious anaemia?
B12 + Folate (B9) deficiency caused by autoimmune destruction of parietal cells in the stomach which reduces levels of intrinsic factor, thereby reducing absorption of B12/9.
29
How do you treat pernicious anaemia?
B12 and B9 supplementation [Must give them together as giving folate without B12 can cause degeneration of the spinal cord]
30
Where is unconjugated bilirubin conjugated and by what enzyme? What is the purpose of conjugation?
Liver (in the hepatocytes) Uridine-diphosphoglucuronic glucuronosyltransferase (UDPGT aka UGT) Converts it to glucoronic acid. Purpose is to make bilirubin soluble in water so it can pass into bile.
31
Where is conjugated bilirubin converted into urobilinogen? What causes this conversion?
The small bowel. | Gut bacteria.
32
What does urobilinogen (produced in the gut) become?
80% --> Stercobilin (faeces) 2% --> Urobilin (urine) 18% --> Enterohepatic circulation
33
What is unconjugated bilirubin bound to in circulation?
Albumin When unconjugated it is only lipid soluble not aqueous soluble.
34
What is Gilbert's sydrome and what blood result would you expect?
Liver disorder affecting 5% of people. Involves dysfunction of UGT enzyme in hepatocytes. This means bilirubin cannot be conjugated. So there is a high level of unconjugated bilirubin in the blood.
35
What are the terms for high and low platelets?
High: - Thrombocytosis - Thrombocythemia Low: - Thrombocytopenia
36
From which progenitor are platelets formed?
Megakaryocytes
37
What hormone stimulates the production of platelets? Where is it produced?
Thrombopoietin Liver [Liver damage can cause thrombocytopenia]
38
Give a cause of underproduction and of destruction of platelets
Underproduction: - B12/Folate deficiency - Liver failure (low thrombopoietin) Destruction: - Idiopathic thrombocytopenic purpura (ITP) - Thrombotic Thrombocytopenic Purpura (TTP) - Haemolytic Uraemic Syndrome (HUS) - Systemic Lupus Erythemaotosus (SLE) [Low platelets can also result from sequestration in the spleen]
39
What test is used to diagnose autoimmune haemolytic anaemia?
Coomb's Test IgG/M against RBC
40
In haemolytic anaemia what would you expect to happen to the levels of reticulocytes, spleen size, unconjugated bilirubin, and MCV?
Reticulocytes: Raised Spleen: Enlarged Unconj: Raised MCV: Macrocytic
41
What is the treatment for immune/idiopathic thrombocytopenic purpura?
Prednisolone (1st line) | Rituximab (2nd line)
42
What is the pathophysiological cause of Thrombotic Thrombocytopenic Purpura?
Autoimmune inhibition of enzyme ADAMTS13 --> excessive Von Willebrand Factor (not broken down) which results in excessive clotting.
43
What is the treatment for Thrombotic Thrombocytopenic Purpura?
Plasma exchange | Immune Suppression
44
Which is more common type A or B haemophilia?
Type A is 5x more common.
45
What inheritance pattern does haemophilia follow?
X-linked recessive | Males affected, females carry
46
Which factors are affected in type A and B haemophilia?
``` A = 8 B = 9 ```
47
Give two signs of haemophilia?
Bleeding/Bruising Arthralgia/Arthropathy Palsies (haematomas)
48
What is the treatment for haemophilia?
Blood transfusions Clotting factors Analgesia (not NSAIDs!)
49
Which clotting pathway is affected in haemophilia? Consequently what would you expect to happen to PT and aPTT and platelets?
Intrinsic pathway (12, 11, 9, 8) aPTT prolonged PT normal Platelets normal
50
Which clotting factors are in the intrinsic clotting pathway? Which are in the extrinsic?
INT: 12, 11, 9, 8 EXT: 7
51
What causes Fibrinogen to form Fibrin?
Thrombin [Produced from Prothrombin (II) by activated factor X]
52
Which clotting factors are affected by vitamin K deficiency?
10 9 7 2 [1972]
53
How many vitamins are there? Which are fat soluble?
13 (A, C, D, E, K) + 8 B vitamins Fat soluble = A D E K
54
True or false, the body can synthesise both vitamin D and K?
True. D via skin K via bacteria in intestines
55
What are the four types of laukaemia? Which is most common in adults and which is most common in children?
Acute Lymphoblastic (Kids) Chronic Lymphoblastic Acute Myeloid (Adults) Chronic Myeloid
56
Give two risk factors for luekaemia
``` Previous cancer Chemical exposure e.g. benzenes Smoking Down's syndrome FHx ```
57
What is the typical blood result indicative of leukaemia?
Pancytopenia + raised WCC [Raised blast cells (immature cells) indicates AML]
58
What is the difference between autographic and allographic stem cells?
Autographic are taken from the patient prior to treatment. Allographic are taken from someone else.
59
Which leukaemia is the philadelphia chromosome mutation associated with?
AML
60
In Disseminated Intravascular Coagulopathy (DIC) what happens to PT, APTT, Fibrin?
PT: Prolonged APTT: Prolonged Fibrin: Reduced This is because the excessive clotting uses it all up the clotting factors.
61
What happens to phosphate, potassium and calcium levels in tumour lysis syndrome?
Phosphate and Potassium are released from the lysed cancer cells and so are raised. Calcium gets bound to phosphate and deposits in tissues. Therefore calcium in the blood decreases! Often deposits in kidneys causing renal failure.
62
What is the treatment for Tumour Lysis Syndrome?
Allopurinol (prophylaxis) as it inhibits xanthine oxidase (thus reducing uric acid levels). Rasburicase in acute TLS, which breaks down uric acid.
63
Which clotting pathway does warfarin affect?
Extrinsic | Prolongs PT
64
True or false, warfarin is contraindicated in pregnancy?
True. It is teratogenic
65
Based on INR when do you stop warfarin/use Vit K, and when do you restart it?
1) Major Bleeds = stop! Give Vit K (+Factors 2, 7, 9, 10) 2) INR >8 = Stop + Vit K. Restart when <5. 3) INR 5-8 = Stop and restart when <5.
66
Name two thrombolytic drugs. How do they work?
Alteplase Streptokinase Increase plasminogen which converts to plasmin (breaks down clots).
67
Name a genetically caused thrombophylic condition
Factor V Leiden Factor V enhanced which results in FX activation and clotting.
68
How much of blood is fluid?
55% (Fluid component / plasma) Cellular component = 44% RBC and 1% WBC/platelets.
69
What is normal haematocrit?
44% Volume of RBC in blood
70
If you have blood loss, what will happen to the haematocrit?
Remain the same. It is a proportional measure. i.e. the % of blood that is RBC.
71
Which leukocyte has multi-lobed nucleus, phagocytoses bacteria, is granular and represents around 70% of WBCs?
Neutrophils
72
Which leukocyte has bilobed nucleus, is granular, is active in parasitic infections, makes up 5% of WBCs, and neutralises the effects of histamine?
Eosinophils
73
Which leukocyte makes up 0.5% of WBCs, has a multilobed nucleus and acts with mast cells to release histamine?
Basophils
74
Which leukocyte has a kidney shaped nucleus?
Monocytes
75
Where do B and T lymphocytes mature?
``` B = Bone marrow T = Thymus ```
76
What is the difference between CD4+ and CD8+ lymphocytes?
``` CD4 = Helper CD8 = Cytotoxic ```
77
What is the most common plasma protein in blood?
Albumin (54% of plasma protein)
78
Apart from albumin, name another plasma protein
Globins (A, B, and G) - Iron transport - Fat soluble vitamins - Immunoglobulins (G) Fibrinogen - Clot formation
79
What is the mechanism of action of aspirin?
Blocks Cyclo-oxygenase 1 enzyme which suppresses prostaglandins and thromboxane A2 (preventing platelet aggregation).
80
What does clopidogrel/prasugrel act on?
Blocks ADP P2Y12 receptors which stops platelet activation
81
What does ABCiximab act on?
Blocks glycoprotein IIb / IIIa receptors which stops platelet aggregation.
82
Give an example of where the 5 types of collagen are used?
1) Skin/Bone 2) Cartilage 3) Reticulin (organ structure) 4) Basement membrane 5) Placenta
83
Basement membranes are made of what type of collagen?
Type 4
84
What is the predominant type of collagen in skin?
``` Type 1 (90%) Type 3 (10%) ```
85
What type of muscle contains multiple nuclei, is striated and had fused myoblasts (syncitium)?
Skeletal muscle
86
Which tool/score is used to assess the bleeding risk in patients taking anticoagulants?
HAS-BLED ``` Hypertension Abnormal liver/renal function Stroke Bleeding Labile INR Elderly >65 Drugs/Alcohol ```
87
What score is used to assess the risk of VTE?
Well's score ``` Active cancer Calf swelling Previous DVT Localised tenderness Bedridden 3+ days Paralysis/Immobilisation No alternative Dx. more likely ``` {3+ = significant. 5+ = CTPA!]
88
If a patient scores 5 on the Well's score what do you need to do?
CTPA LMWH or Fondaparinux if CTPA is delayed.
89
Is Fondaparinux a direct or indirect inhibitor of Factor X?
Indirect (just like LMWH) Potentiates Antithrombin III This inhibits Factor X
90
Is D-Dimer a test of inclusion or exclusion?
Exclusion. If its -ve you can rule out a VTE. If its +ve you need to do more tests. It is sensitive but not specific.
91
What is virchow's triad?
Major factors contributing to thrombus formation. - Stasis - Hypercoagulability - Endothelial injury
92
Give two risk factors for varicose veins
``` Age Female Pregnancy FHx Long time standing/sitting ```
93
What 2 investigations must you do before giving a patient compression stockings?
Duplex US to ensure good venous circulation and Ankle Brachial Pressure Index (arterial supply).
94
How are varicose veins treated?
- Compression stockings - Endothermal ablation - Surgical ligation - Foam sclerotherapy (irritant foam causes local inflammation which closes the veins).
95
What is the 1st line treatment for a continuous nosebleed?
Nasal Packing (cotton wool + vasoconstrictor + local anaesthetic) Once stopped cauterise with silver nitrate stick.
96
What is the first line treatment for haemochromatosis?
Blood letting Deferoxamine (2nd line)
97
What mutation is responsible for 95% of cases of primary polycythaemia?
JAK2 Results in the proliferation of erythroid progenitor cells
98
Give two causes of secondary polycythaemia
Smoking Cyanosis COPD Polycystic Kidney Disease
99
True or false, in polycythemia you find macrocytosis of RBCs?
False. You find microcytosis due to iron depletion resulting from overproduction.
100
Give two signs of multiple myeloma
[CRAB] ``` HyperCalcaemia Renal impairment Anaemia Bone lesions (pepper potting) + Hyperviscosity ```
101
What would you expect to see on the bone marrow aspirate of a patient with multiple myeloma?
Monoclonal bands of IgG
102
Give two key investigations you would do in a patient suspected of multiple myeloma
Bone marrow aspirate Skeletal survey MRI spine (if compression) Serum electrophoresis for para protein (light chains). (electrophoresis is preferred to Bence Jones screening now).,
103
If a patient with microcytic anaemia is not responding to iron treatment, what do you suspect?
Sideroblastic anaemia (Microcytic) Caused by ineffective erythropoesis and iron loading in bone marrow.
104
In sideroblastic anaemia is ferritin increased or decreased?
Increased. Poor use of iron results in accumulation of iron in the blood stream as wellas in the bone marrow.
105
What is koilonychia? Give a cause
Spooning of the nails | Iron deficiency
106
What are acanthocytes?
RBCs with spike projections due to unstable membranes. Caused by splenomegaly, alcoholic liver disease and spherocytosis.
107
In microcytic anaemia e.g. thalassaemia, iron deficiency and sideroblastic anaemia, do you find hyper or hypochromic RBCs?
Hypochromic
108
True or false, steroids can increase the neutrophil count?
True
109
Name a vitamin deficiency that can cause glossitis?
B12 B3 (Pellagra) Iron Zinc
110
What would you expect to happen to the MCV in pernicious anaemia?
Increased (macrocytic) | Lack of B12
111
What is the treatment for pernicious anaemia?
Hydroxocobalamin (B12) IM for 2 weeks
112
What is Coomb's test for?
Autoimmune haemolysis
113
Give two indications for fresh frozen plasma
Urgent correction of clotting defect - DIC - Warfarin OD - TTP - Liver disease - When Vit K too slow
114
What is TRALI?
Transfusion related acute lung injury (TRALI) Acute reaction to a blood transfusion (within 24hrs): - Dyspnoea - Cough - White out on CXR Tx: Stop infusion! 100% 02 treat ARDS.
115
What legal statutes prevent medics from treating a patient against their consent?
Battery - Common Law 'Degrading act/torture' - European Convention on Human Rights
116
What diuretic would you give, if a patient was fluid overloaded (tachycardic, dysphnoea, raised JVP etc) following a blood transfusion?
100% O2 | Furosemide 40mg IV ('Loop for lungs')
117
How does LMWH work?
Inactivates Factor Xa [Fondaparinux does the same].
118
How do you bridge a patient onto anticoagulation therapy?
Give both warfarin + LMWH | Continue until INR is within therapeutic range (2-3)
119
What causes Hodgkin's Lymphoma? Which has a worse prognosis, HL or NHL?
Malignant proliferation of lymphocytes in the lymph nodes. HL = Reed Sternberg cells = 15% cases = More treatable
120
What staging system is used to assess Hodgkin Lymphoma?
Ann Arbor 1) Single lymph node 2) 2+ lymph nodes (same side of diaphragm) 3) Nodes both sides of diaphragm 4) Beyond lymph nodes e.g. bone marrow
121
What is the RCHOP regimen?
``` Rituximab Cyclophosphamide Hydroxydaunorubicin Vincristine (Oncovin) Prednisolone ``` Used for high grade lymphoma. Low grade may only need radiotherapy.
122
What is agranulocytosis? Give a drug which can cause it
When granulocytes e.g. neutrophils, basophils or eosinophils stop being produced. This leaves the patient vulnerable to infection. Carbimazole Procainamide Clozapine
123
Give two treatments for myeloma
Analgesia Bisphosphonates Chemotherapy Stem cell transplantation
124
If a patient with myeloma gets spinal cord compression, what is the best investigation and treatment?
MRI spine | Dexamethasone + Radiotherapy
125
What condition is considered a precursor to myeloma?
Waldenstrom macroglobulinaemia IgM paraprotein overproduction --> CNS disorder and hyperviscosity.