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Haematology Flashcards

1
Q

What is haemopoiesis

A

The physiological developmental process that gives rise to the cellular components of blood

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2
Q

What are the characteristics of haemopoietic stem cells

A

Self renewal
High proliferative potential
Differentiation potential for all lineages
Long term activity throughout the lifespan of the individual

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3
Q

What are the two types of haemopoietic lineages

A

Myeloid

Lymphoid

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4
Q

When and where does haemopoiesis happen in utero

A

Day 27 in the aorta mesonephros region, expands rapidly at day 35, then disappear at day 40 when the haemopoietic stem cells migrate to the foetal liver which becomes the subsequent site of haemopoiesis

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5
Q

What are the functions of blood cells

A

Oxygen transport
Coagulation (haemostasis)
Immune response to infection
Immune response to abnormal cells (senescent, malignant etc)

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6
Q

What is anaemia

A

Reduced red cells

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7
Q

What is polycythaemia

A

Raised red blood cells

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8
Q

What are the functions of white blood cells (leukocytes)

A

Immunity and host defence

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9
Q

What are the types of white blood cells

A 
Granulocytes:
-Neutrophils
-Eosinophils
-Basophils
Moncytes
Lymphocytes
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10
Q

What is neutropenia

A

Decrease numbers of neutrophils

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11
Q

What is eosinophilia

A

Increased numbers of eosinophils

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12
Q

What is basophilia

A

Increased numbers of basophils

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13
Q

What is monocytosis

A

Increased numbers of monocytes

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14
Q

What is lymphocytosis

A

Increased numbers of lymphocytes

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15
Q

What is lymphopenia

A

Decreased numbers of lymphocytes

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16
Q

What are the four main subdivisions of haematology clinical practice

A

Coagulation
Malignant
Non-malignant
Transfusion

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17
Q

What are the diagnostic tests in haematology

A

Full blood count
Blood film (or smear)
Coagulation screen

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18
Q

What does the full blood count test

A 
Haemoglobin conc
Red cell parameters
-MCV (mean cell volume)
-MCH (mean cell Hb)
White cell count (WCC)
Platelet count
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19
Q

What does a coagulation screen do?

A

Tests measure the time taken for a clot to form when plasma is mixed with specified reagents

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20
Q

What parts of coagulation cascade can be assayed

A
  • Prothrombin time
  • Activated partial thromboplastin time
  • Thrombin time
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21
Q

How is bone marrow aspirated

A

Under local anaesthetic, liquid marrow is aspirated from the posterior iliac crest of the pelvis and a trephine core biopsy is then taken with a hollow needle

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22
Q

What is the sensitivity of a test

A

Defined as the proportion of abnormal results correctly classified by the test
Expresses the ability to detect a true abnormality

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23
Q

What is the specificity of a test

A

Defined as the proportion of normal results correctly classified by the test
Expresses the ability to exclude an abnormal result in a healthy person

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24
Q

How do the cells in iron deficiency anaemia present

A

Small, pale red cells
Low MCV and MCH
Variable size and shape- long thin pencil cells

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25
Q

What is leucodepletion

A

When whole blood is filtered before further processing to remove white cells

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26
Q

How is a unit of red blood cells kept healthy during storage

A

Plasma is replaced by a solution of electrolytes, glucose and adenine

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27
Q

What is the raise in a patients Hb expected to be after a unit of RBC transfusion

A

10g/L

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28
Q

How much iron is in one unit of packed RBC

A

200-250 mg of iron

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29
Q

How is a unit of RBC stored

A

At 4 degrees C for up to 35 days from collection

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30
Q

What is the therapeutic dose of RBC

A

10-20ml/kg of recipient

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31
Q

What is the usual transfusion time

A

1.5-3 hours

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32
Q

Why are red blood cells transfused

A

Significant bleeding
Acute anaemia
Chronic anaemia
To restore oxygen carrying capacity

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33
Q

What types of anaemia can be treated without blood transfusion

A 
Iron deficiency
B12 and folate deficiency
Renal disease (erythropoietin treatment)
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34
Q

How many units of pooled platelets are in a single pack

A

4-6 units which each come from different donors as one unit of platelet comes form one unit of whole blood

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35
Q

What are aphaeresis platelets

A

Platelets that have been removed through an apheresis machine that collects platelets and returns all other blood constituents to the donor
The amount of platelets collected this way is equivalent to 4-6 units of pooled platelets

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36
Q

How much will a therapeutic dose of platelets raise patients platelets

A

20-60x 10^9/L

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37
Q

What is the platelet count per therapeutic dose

A

3 x 10^11

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38
Q

How are platelets stored

A 
Room temperature (22 degrees C) on an agitator
Shelf-life 5 days from collection
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39
Q

What is the usual transfusion time

A

30 mins/unit

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40
Q

What is the limiting factor for the shelf life of platelets

A

The risk of contamination by bacteria from the donor’s arm that grow at the conditions of storage and can be transmitted to the recipient

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41
Q

Why are platelets transfused

A

To treat bleeding due to severe thrombocytopenia (low platelets) or platelet dysfunction

To prevent bleeding in patients with thrombocytopenia or platelet dysfunction

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42
Q

What is fresh frozen plasma

A

Contains all clotting factors at physiological levels

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43
Q

What is the therapeutic dose of fresh frozen plasma

A

12-15 ml/kg (4-6 units for average adults)

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44
Q

How is fresh frozen plasma stored

A

-30 degrees C for up to 36 months

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45
Q

What is the volume of 1 unit of fresh frozen plasma

A

300ml

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46
Q

What is the usual transfusion time of fresh frozen plasma

A

30 mins/unit

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47
Q

Why is fresh frozen plasma transfused

A

To replace clotting factors in patients with multiple factor deficiencies:

  • to treat significant bleeding in patients with abnormal clotting results
  • to correct abnormal clotting results prior to invasive procedures
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48
Q

When should you not transfuse FFP

A

To treat single factor deficiencies
To correct abnormal clotting results in patients that are not bleeding
To reverse warfarin

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49
Q

What are some acquired coagulopathies

A

Liver disease
Disseminated intravascular coagulation
Massive haemorrhage

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50
Q

How is warfarin anticoagulation reversed

A

Prothrombin complex concentrate (factor IX complex)

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51
Q

What is cryoprecipitate

A

Extracted from FFP during thawing
Contains fibrinogen, von Willebrand, factor VIII, factor XIII
Therapeutic dose: 10-15ml/kg (6-10 units)
Used as a concentrated source of fibrinogen in acquired coagulopathies

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52
Q

What are causes of anaemia

A 
Haematinic deficiencies
Secondary to chronic disease
Haemolysis
Alcohol, drugs, toxins
Renal impairment - EPO
Primary haematological/ marrow disease:
-Malignant
-Haemoglobin disorders (sickle etc)
-Aplasia
-Congenital
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53
Q

What are the classifications of anaemia based on MCV

A

Macrocytic
Normocytic
Microcytic

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54
Q

What types of anaemia fall in to Macrocytic

A
  • B12, Folate, metabolic (thyroid/liver disease)
  • Marrow damage (booze, drugs, marrow disease)
  • Haemolysis (due to reticulocytosis)
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55
Q

What types of anaemia fall in to normocytic

A

Anaemia of chronic disease/inflammatory

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56
Q

What types of anaemia fall in to microcytic

A

Iron deficiency
Haemoglobin disorders
Sometimes chronic disease

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57
Q

How is iron balanced

A 
No excretion - limited absorption
Controlled at the level of the gut mucosa
Most iron is thus re-cycled
Absorbed in duodenum
Transported by transferrin
Stored in ferritin/ haemosiderin
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58
Q

Where can iron be found in diet

A

Pretty much everything

Should not be iron deficiently anaemic if eating a balanced diet

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59
Q

What is the management of iron deficiency disorder

A

Establish that there is low iron
Establish the cause
Treat the iron and the cause

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60
Q

How is low iron established

A 
FBC, indices and film
Ferritin levels tell if iron deficient
% hypochromic cells
Serum iron/ TIBC
Marrow
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61
Q

What are the main causes of iron deficiency

A

Blood loss from anywhere (gut/ PV/ PU/ respiratory tract etc)
Malignancy
Increased demand (pregnancy/ growth)
Reduced intake (diet/ malabsorption)

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62
Q

How is iron deficiency treated

A

Oral iron
IM iron
IV iron

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63
Q

What is megaloblastic anaemia

A

A characteristic cell morphology caused by impaired DNA synthesis

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64
Q

What are the most common causes of thrombocytopenia

A 
Drugs, alcohol, toxins
ITP
Autoimmune disease
Liver disease and/or hyperslenism
Pregnancy
Haematological/ marrow disease
Infections acute or otherwise: 
-HIV
-Acute sepsis
-Viral infection (EBV)
Disseminated intravascular coagulation
Range of congenital conditions
Many others
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65
Q

What is ITP

A 
Common
Immune thrombocytopenic purport
Immune disorder
Occurs on its own or as part of:
-Other autoimmune disease
-Lymphomas/ CLL
-HIV
Can be acute/ chronic/ relapsing
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66
Q

How does ITP present

A 
Bruising or petechiae or bleeding
Platelet count:
<10 urgent/ might be bleeding
<20 a worry 
<30 need treatment especially if having surgery
No definitive test
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67
Q

How is ITP treated

A 
Steroid is first line
IV immunoglobulin
Immunosuppressives or splectomy
Thrombosis-mimetics:
-Eltrombopag
-Romiplostin
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68
Q

What is the outcome of ITP therapy

A 
Usually rapid response
Can relapse after therapy
Rarely life-threathening
Commonly recurrent
Some difficult refractory causes
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69
Q

What is TTP

A 
Thrombotic thrombocytopenia purpura
Rare but urgent diagnosis
Most are immune ADAMTS-13/VWD
Suspect if thrombocytopenia and:
-Fever
-Neurological symptoms 
-Haemolysis (retics/ LDH)
Seek evidence of microangiopathy:
-Blood film fragments
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70
Q

How is TTP treated

A

Plasma exchange with FFP/plasma
Steroids
(Vincristine)
(Rituximab)

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71
Q

What are the causes of megaloblastic change

A 
B12 and/ or folic acid deficiency
Alcohol
Drugs:
-cytotoxics
-folate antagonists
-N20
Haematological malignancy 
Congenital rarities:
-Transcobalamin deficiency
-Orotic aciduria
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72
Q

How do B12 and Folate cause anaemia

A

DNA consists of purine/pyrimidine bases
Folates are required for their synthesis
B12 is essential for cell folate generation
So low folate or B12 starves DNA of bases

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73
Q

Where can vitamin B12 be found in the diet and how is it absorbed

A 
Loads in most diets but only from animal sources
Absorption:
-gastric parietal cells
-intrinsic factor
-receptors in terminal ileum
Stores sufficient for some years
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74
Q

Who has b12 deficiency

A 
Nutritional: vegans
Gastric problems:
-pernicious anaemia (autoimmune)
-Gastrectomy
Small bowel problems
-terminal ileum resection/ Chrons
-Stagnant loops/ jejunal diverticulosis
-Tropical sprue/ Fish tapeworm
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75
Q

Where can folic acid be found and absorbed

A

Mainly in green vegetables, beans, peas, nuts and liver
Required intake needs decent daily diet
Absorbed in upper small bowel
4 months body stores

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76
Q

Why do patients have folic acid deficiency

A 
Mainly dietary/ malnutrition
Malabsorption/ small bowel disease
Increased usage:
-pregnancy
-haemolysis
-inflammatory disorders
Drugs/ alcohol/ ITU
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77
Q

What are the features common to B12 or folate deficiency

A 
Megaloblastic anaemia
Can have pancytopenia if more severe
Mild jaundice
Glossitis/ angular stomatitis
Anorexia/ weight loss
Sterility
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78
Q

What is haemolysis and the causes

A 
Shortened red cell life
Causes:
-Things wrong inside the red cell
-Things wrong with the red -cell membrane
-Things wrong external to the red cell
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79
Q

Why are anticoagulants used

A 
Prevention of venous thromboembolism
Prevention of stroke in AF
Treatment of DVT/PE etc
Mechanical valve patients
Arterial thrombosis/ limb ischaemia
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80
Q

What are the common anticoagulants

A 
Heparin
Fondaparinux
Vitamin K antagonists:
Warfarin
Sinthrome ( Acenocoumoral)
DOACs ( Direct Oral Anticoagulants):
-Rivaraoxaban
-Apixaban
-Edoxaban
-Dabigatran
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81
Q

What is heparin

A

Derived from pigs
Mode of action:
Increases ability of antithrombin to bind to and irreversibly switch off thrombin (IIa) and Factor Xa

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82
Q

What are the potential complications of heparin

A

Skin/allergic reactions
Osteoporosis
Heparin induced thrombocytopenia

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83
Q

How does heparin induced thrombocytopenia present

A 
drop in platelet count  
>50%  from baseline 
usually 5-10 days after starting heparin
can be associated with thrombosis
Calculate 4Ts score (timing, level, thrombosis, other causes) and if > 3 send HIT screen, stop heparin, start argatroban
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84
Q

What is fondaparinux

A

Synthetic pentasaccharide, given subcutaneously
Binds to antithrombin and inhibits Xa activity
Half life 17-20 hours if normal renal function
Bleeding- stop treatment and general haemostatic measures
No specific antidote
Critical bleeding- consider rFVIIa

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85
Q

How is warfarin monitored

A

By INR

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86
Q

What is INR

A

Prothrombin ratio

Prothrombin ratio = Patient’s prothrombin time/ mean normal prothrombin time

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87
Q

What are the problems with oral anticoagulants

A

React with many drugs
Food interactions- vitamin K containing
Alcohol

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88
Q

What are the characteristics of the ideal anticoagulant

A
  • Oral administration
  • No requirement for routine coagulation monitoring and dose adjustment
  • Wide therapeutic window: high efficacy in preventing thrombosis; low bleeding risk
  • Rapid onset of action
  • Predictable pharmacokinetics and pharmacodynamics
  • Minimal interactions with foods and other drugs
  • Ability to inhibit free and clot-bound coagulation factors
  • Low non-specific binding
  • Availability of an antidote
  • No unexpected toxicities
  • Acceptable costs
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89
Q

What are the benefits of DOACs

A

Uniform dose in most patients
No need for routine monitoring
Minimal interactions with drugs and foodstuffs

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90
Q

What are the cons of DOACs

A 
Contraindications:
-Renal impairment
-Women of child bearing age
-Extremes of body weight >120 kg
Management of bleeding
Lab issues
Adherence
Special populations:
-Mechanical valves
-antiphospholipid syndrome
Peri-operative management
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91
Q

What are the indications for measuring anticoagulant drug levels

A 
Bleeding
Need for emergency surgery/procedure
Question of adherence
Recurrent thrombosis
Renal impairment
Potential drug interactions
Extremes of weight
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92
Q

What is aspirin

A

Inactivates platelet cyclooxygenase reducing thromboxane A2
Irreversible effect, lasts 4-5 days
No reversal agents
Give 2-3 adult doses of platelets in critical bleeding

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93
Q

What are the acquired bleeding disorders

A 
Vitamin K deficiency 
Liver disease
Renal disease
Major haemorrhage
DIC
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94
Q

What are the causes of vitamin K deficiency

A

Obstructive jaundice
Prolonged nutritional deficiency
Broad spectrum antibiotics
Neonates (classical 1-7 days)

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95
Q

How is vitamin K deficiency treated

A

IV/Oral Vitamin K 10mg for 3-5 days

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96
Q

How is haemostasis impaired in liver disease

A
  • Thrombocytopenia (production or hypersplenism)
  • Platelet dysfunction (plasmin induced cleavage of surface glycoproteins)
  • Reduced plasma concentration of all coagulation factors (reduced synthesis) except FVIII
  • Delayed fibrin monomer polymerisation due to altered fibrinogen glycosylation (xs sialic acid)
  • Excessive plasmin activity
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97
Q

How is bleeding in liver disease treated

A

Platelet transfusions
FFP or prothrombin complex concentrate
Cryoprecipitate or fibrinogen concentrate
Endoscopy if GI bleed
NB above transfusions may not correct clotting tests completely.

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98
Q

How does bleeding in renal disease present

A

Symptoms: (30-50% of CRF patients)
Easy bruising, petechia, gum bleeding, nosebleeds, excessive bleeding from venepuncture/lines
ICH bleeds, retroperitoneal bleed, pericardial tamponade, GI bleeds
Patients with uncontrolled high BP and on dialysis Increased risk of ICH

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99
Q

What are the causes of bleeding in renal disease

A

Anaemia
Drugs accumulating in renal failure can bind to platelets and block their receptors
Uremia

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100
Q

How is bleeding prevented in renal disease

A

Correction of anaemia- EPO and transfusions
Avoidance of antiplatelet drugs for at least 7 days prior to procedures
Dialysis
DDAVP pre procedures
Tranexamic acid pre procedures (not if urinary tract and risk of haematuria)

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101
Q

How is bleeding treated in renal disease

A

DDAVP
Tranexamic acid
Cryoprecipitate used in cases not responsive to DDAVP ( rich in FVIII, VWF, fibrinogen, FXIII)

102
Q

What is the definition of a major haemorrhage

A 
HR >110, systolic BP <90 mmHg
And/ or
Transfusion of a volume equal to the patient’s total blood volume in less than 24 hours or
50% blood volume loss within 3 hours
Loss of > 150ml/min
103
Q

What is DIC

A

Disseminated intravascular Coagulation
It is characterised by systemic activation of pathways leading to and regulating coagulation, which can result in the generation of fibrin clots that may cause organ failure with concomitant consumption of platelets and coagulation factors that may result in clinical bleeding.

104
Q

What is the pathogenesis of DIC

A

Excess thrombin generation
Reduced natural anticoagulant activity
Decreased fibrinolysis

105
Q

What are the causes of DIC

A

Acute DIC:
Sepsis (any organism)
Obstetric complications- amniotic fluid embolism, abruption
Trauma/Tissue necrosis/Fat embolism
Acute intravascular haemolysis eg ABO incompatible blood transfusion
Fulminant liver disease
Organ destruction ( e.g. severe pancreatitis)
Massive blood loss
Severe toxic or immunological reactions (e.g. recreational drugs, transfusion reactions, transplant rejection, snake bites)

Chronic DIC
Malignancy
End stage liver Disease
Vascular abnormalities (e.g. Kassbach-Merrit syndrome)

106
Q

What are the clinical features of DIC

A

Mucosal oozing, bleeding from surgical wounds or indwelling canulae
Multi organ failure secondary to microthrombi (and hypovolaemia)
Skin necrosis
Thrombus

107
Q

What are the common causes of Haemolysis

A

Inside:

  • Haemoglobinopathy (sickle)
  • Enzyme defects (G6PD)

Membrane:
-Hereditary spherocytosis/ elliptocytosis

External:

  • Antibodies (warm/cold)
  • Drugs, toxins
  • Heart valves
  • Vascular/ vasculitis/ microangiopathy
108
Q

How can the presence of Haemolysis be tested

A 
Anaemia
High MCV, microcytic
High reticulocytes
Blood fil (fragments/ spherocytes)
Raised bilirubin, LDH
Low haptoglobins
Urinary haemosiderin
109
Q

When do acute and delayed reactions to transfusion occur

A

Acute: <24 hours post transfusion
Delayed: >24 hours post transfusion

110
Q

What are the complications of transfusion which are non immunological

A

Transfusion transmitted infections
Transfusion associated circulatory overload (TACO)
Febrile non-haemolytic transfusion reaction (FNHTR)
Iron overload

111
Q

How are viral transfusion transmitted infections prevented

A 
Donor questionnaire
Mandatory testing:
-Hep B
-HIV
-Hep C
-Hep E
-Human T-cell lymphotropic virus
-Syphilis
112
Q

What are the symptoms of a transfusion of bacterial contaminated components

A 
Rigors
High fever
Severe chills
Hypotension
Nausea
Vomiting
Dyspnoea
Circulatory collapse
113
Q

How may blood components for transfusion become contaminated

A

By bacteria from the donor’s skin during collection
By unrecognised bacteraemia in the donor
Contamination from the environment
Increase risk with storage after donation

114
Q

What is FNHTR

A

Febrile non-haemolytic transfusion reaction

Due to cytokines or other biologically active molecules accumulating during storage of blood components

115
Q

What are the clinical features of FNHTR

A 
Rise of temp >1 degree C from baseline
±rigors
±tachycardia
Unpleasant but not life-threatening
Resolves after discontinuation of transfusion
116
Q

What are the symptoms of transfusion associated circulatory overload

A 
Starts up to 24 hours after transfusion
Sudden dyspnoea
Orthopnoea
Tachycardia
Hypertension
Hypoxemia
Raised BP
Elevated JVP
117
Q

What are the risk factors for transfusion associated circulatory overload

A 
Elderly patients
Small children
Patients with compromised left ventricular function
Large transfusion volume
Increased rate of transfusion
118
Q

How is transfusion associated circulatory overload prevented

A

Follow guidance on volume and rate of transfusion for each component

119
Q

What are the immunological complications related to transfusions

A

Acute haemolytic transfusion reaction due to incompatibility
Delayed haemolytic reaction
Post transfusion purpura
Allergic/ anaphylactic reaction
Transfusion related acute lung injury (TRALI)
Transfusion-associated graft-versus-host disease (TA-GvHD)

120
Q

What causes acute haemolytic reaction

A

Transfusion of red blood cells to a recipient that has preformed antibodies against antigens that are expressed on the transfused red blood cell causing free haemoglobin to be released into the circulation

121
Q

What are the possible results of acute haemolytic reaction due to free haemoglobin

A

Vasoconstriction, hypertension, angina

Fever, riggers, hypotension

Bleeding

Acute kidney injury

Death

122
Q

What are the signs and symptoms of acute haemolytic reaction due to incompatibility

A 
Fever and chills 
Back pain
Infusion pain
Hypotension/shock
Haemoglobinuria
Increased bleeding (DIC)
Chest pain
Sense of impending death
123
Q

What is a group and screen

A

Determination of ABO and Rh(D) group
Test patient’s plasma to screen for antibodies against other clinically significant blood group antigens:
-Positive: antibody identification by testing the patient’s plasma against a panel of red cells containing all the clinically significant blood groups
-Negative: no further testing

124
Q

What is crossmatching testing

A

Final test before transfusion of RBC
Donor RBC of correct ABO and Rh group and antigen negative for the antibodies detected in the green are selected from blood bank
Crossmatching is when patient’s plasma is mixed with aliquots of donor RBC to see if there is a reaction
-No: RBS units compatible, no risk of acute haemolysis
Yes: RBC incompatible, risk of acute haemolysis

125
Q

What causes delayed haemolytic reaction to transfusion

A

Post transfusion formation of new immune IgG antibodies against RBC antigens other than ABO

126
Q

What are the clinical features of delayed haemolytic reaction

A 
Onset 3-14 days after transfusion of RBC
Fatigue
Jaundice
± Fever
Lab findings:
-Drop in Hb
-Increased LDH
-Increased indirect bilirubin
-Direct and indirect antiglobulin test positive
127
Q

What are the other names given to direct anti-globulin test

A

Coomb’s test
Anti-human globulin test (AHG)
Direct anti-globulin test (DAT)

128
Q

What causes allergic reactions to transfusions

A

Hypersensitivity of recipient to transfused random proteins

Usually after transfusion of components that contain plasma (FFP, Cryoprecipitate, platelets)

129
Q

What are the clinical features of allergic reactions to transfusions

A 
Rash 
Urticaria
Pruritus
±fever
±rigors
Periorbital oedema
130
Q

What are the symptoms of anaphylactic reaction to transfusions and who is at higher risk

A

Laryngeal oedema
Bronchospasm
Hypotension
Swelling

Risk:
Patients with IgA deficiency and anti-IgA antibodies

131
Q

How are newborns screened for haemoglobinopathy and why

A

At 5 days
Mid wife, heel prick test
Analysis of dried blood spot (multiple conditions tested for)
Early detection of sickle cell disease (parent education, starting antibiotic prophylaxis

132
Q

What is a normal red blood cell

A

Bi-concave disks
No nucleus
Function is to transport oxygen bound to haemoglobin
Production controlled by erythropoietin produced in kidneys in response to tissue oxygen concentration

133
Q

What is haemoglobin

A

Tetramer of globin chains, each non-covalently bound to a haem
2 alpha and 2 non-alpha chains

134
Q

What is the function of globin

A

Protects haem from oxidation
Renders the molecule soluble
Permits variation in oxygen affinity

135
Q

What is a haemoglobinopathy

A

Changes in globing genes or their expression Leeds to disease

136
Q

What is thalassaemia

A

Change in globing gene expression leading to reduced rate of synthesis of normal globing chains. Pathology is due to imbalance of alpha and beta chain production (free globing chains damage red cell membrane)

137
Q

What are structural Hb variant haemoglobinopathies

A

Usually a single base substitution in globin gene causes altered structure/ function
Eg sickle cell

138
Q

How are Haemoglobinopathies inherited

A

Autosomal recessive

139
Q

What is the clinical picture for sickle cell trait (Heterozygous)

A

Blood count normal

No problems except when extreme hypoxia/ dehydration

140
Q

What happens in sickle cell disease

A

Sickle Hb polymerises to form long liberals which distort the red cell membrane and produce the classic sickle shape

141
Q

What is the clinical picture for sickle cell disease (homozygote)

A

Blood count: anaemia

Blood film: sickle cells

142
Q

What are acute complications of sickle cell disease

A

Vaso-occlusive crisis
Septicaemia
Aplastic crisis
Sequestration crisis

143
Q

What are chronic complications of sickle cell disease

A

Hyposplenism: due to infarction and atrophy of spleen
Renal disease: medullary infarction with papillary necrosis.
- tubular damage then can’t concentrate urine resulting in bed-wedding
- Glomerular damage results in chronic renal failure/ dialysis
Avascular necrosis: femoral/humeral heads
Leg ulcers, osteomyelitis, gall stones, retinopathy, cardiac, respiratory

144
Q

How is sickle cell disease treated in neonatal

A

Penicillin from 6 months (neonatal screening)

145
Q

How is sickle cell disease treated in acute crisis

A

Vaso-occlusive - analgesia (opiates), hydration (to maintain red cell water), treatment of precipitants
-Priapism- education

146
Q

How are thalassaemias categorised

A

α, β, δβ and γδβ
according to which globing chain is reduced
In some, no globing chain is produced and in others they are produced at reduced rate

147
Q

What is the blood picture in β-Thalassaemia and how does it present clinically

A

resembles iron deficiency by being small pale red cells
Total Hb level normal or slightly reduced
No clinical problems

148
Q

How do those with β-Thalassaemia major present

A

Short stature and distorted limb growth due to premature closure of epiphyses in long bones
Enlarged liver and spleen

149
Q

What are the three top causes of death in patients with thalassaemia

150
Q

How is iron overload prevented in patients with β-Thalassaemia major

A

To prevent death patients are started on Iron chelation therapy from 2nd year of life to promote excretion of iron in urine and faeces

151
Q

How is desferrioxamine given

A

8-12 hourly subcutaneous infusion via a syringe-pump as home-treatment on at least 5 nights a week to prevent the accumulation of iron in β-Thalassaemia major patients

152
Q

What are new oral iron chelators

A

Deferiprone

Deferasirox

153
Q

What is the target Ferritin

A

~1000-1500µg/L

154
Q

What are the physiological changes which may occur in pregnancy

A

Anaemia (macrocytosis), thrombocytopenia
Neutrophilia (and left shift)
Increased pro-coagulant factor and decrease in fibrinolysis

155
Q

What are the fundamental factors which contribute to thrombogenesis

A

Alterations in blood flow producing stasis, damage to the vascular endothelium and changes in blood constituents resulting in hypercoagulability

156
Q

What is arterial thrombosis

A

Cause by atherosclerosis of vessel wall.
Rupture of atheromatous plaque
Endothelial injury
Platelet aggregation and platelet thrombi play an important role in final vessel occlusion

157
Q

What are the risk factors for arterial thrombosis

A

Smoking, hypertension, hypercholesterolaemia, diabetes, family history, obesity, physical inactivity, age, male sex

158
Q

What is venous thrombosis

A

Pathogenesis mainly involves:

  • venous stasis
  • hypercoagulable states
159
Q

What makes up venous thrombi

A

Predominantly composed of fibrin with a lesser role for platelet accumulation and aggregation

160
Q

What is the care pathway for hospital admission in relation to VTE

A

Patient admitted to hospital
Assess VTE risk
Assess bleeding risk
Balance risks of VTE and bleeding
Offer VTE prophylaxis if appropriate
Do not offer pharmacological VTE prophylaxis if patient has any risk factor for bleeding and risk of bleeding outweighs risk of VTE
Reassess risks of VTE and bleeding within 24 hours of admission and whenever clinical situation changes

161
Q

What are the risk factors for VTE

A
  • Active cancer or cancer treatment
  • > 60 yo
  • Critical care admission
  • Dehydration
  • Known thrombophilias
  • One or more significant medical comorbidities
  • Surgery
  • Major trauma
  • Personal history of VTE
  • Use of hormone replacement therapy
  • Use of oestrogen-containing contraceptive therapy
  • Varicose veins with phlebitis
  • Obesity (BMI over 30)
  • Pregnancy and postnatal period
  • Immobility
  • First degree relative with VTE
162
Q

What is the general advice for those at risk of VTE in hospital

A

Do not allow patients to become dehydrated unless clinically indicated
Encourage patients to mobilise as soon as possible
Do not regard aspirin or other anti-platelet drugs as adequate prophylaxis for VTE

163
Q

What meds are used in pharmacological prophylaxis

A 
Low dose low molecular weight heparin
Fondaparinux (synthetic pentasaccharide)
Newer anticoagulants:
-direct inhibitors of factor Xa:
-- rivaroxaban
-- apixaban
-direct thrombin inhibitors:
-- dabigatran
164
Q

What is the Wells score

A

Reflects the risk of developing DVT
Validated numerical clinical probability score
Sensitive quantitative D-dimer with high negative predictive value
Used in an agreed algorithm

165
Q

How can DVTs be diagnosed with imaging

A

Ultrasound
Duplex scanning with compression will aid to detect any thrombus
Highly sensitive and specific
Look for loss of flow signal, intravascular defects or non collapsing vessels in the venous system

166
Q

How is low molecular weight heparin used in VTE treatment

A

Doses are fixed by body weight
Once daily by sub cut injection
Treat for at least 5 days
Overlap with warfarin until INR >2.0 for two consecutive days

167
Q

How are VTEs managed

A

First episode of proximal vein DVT or PE:

  • treat for 3-6 months
  • warfarin target INR= 2.5

Recurrent VTE:
- treat with long term anticoagulation

Proximal DVT or PE which has occurred in absence of reversible risk factor:
-consider long term anticoagulation

Recurrent VTE on therapeutic anticoagulation:
-increase target INR to 3.5 for warfarin

168
Q

What are the definitions of thrombophilia

A

Familial or acquired disorders of the haemostat mechanism which are likely to predispose to thrombosis

Patients who develop VTE:

  • spontaneously
  • of disproportionate severity
  • recurrently
  • at an early age
169
Q

What are heritable thrombophilias

A 
Antithrombin deficiency
Protein C deficiency
Protein S deficiency
Activated Protein C resistance/ Factor V Leiden
Dysfibrinogenaemia
Prothrombin 20210A
170
Q

What is the acquired thrombophilia

A

Antiphospholipid syndrome

171
Q

What are the clinical features of thrombophilia

A

DVT
PE
Superficial thrombophlebitis
Thrombosis of cerebral, axillary, portal, mesenteric veins
Arterial thrombosis |(APS, only)
Coumarin induced skin necrosis (PC deficiency)
Obstetric complication: foetal wastage (APS)

172
Q

What is antiphospholipid syndrome

A

Antiphospholipid antibodies on at least 2 occasions, 8 weeks apart in association with venous thrombosis or arterial thrombosis or >2 foetal loss
May be primary or secondary

173
Q

When should a patient be tested for thrombophilia

A

Not unselected patient who just have a VTE
After counselling re pros and cons and discussion of management
Asymptomatic relatives not usually tested

174
Q

What are some haematological emergencies on a haematology ward

A
  • Neutropenic sepsis
  • Pneumonia
  • Hypercalcaemia and hyper-viscosity
  • Spinal cord compression
  • Acute kidney failure
  • Tumour lysis syndrome
  • Sickle cell crisis
  • Acute haemolysis
  • Acute haemorrhage in haemophilia A or B
  • New acute leukaemia
  • Mediastinal mass
175
Q

What are some haematological emergencies on a general ward

A
  • PE
  • DVT
  • Transfusion reactions (haemolysis, febrile non-haemolytic, TRALI, Massive transfusion)
  • Heparin induced thrombocytopenia
  • Immune thrombocytopenia purpura
  • Disseminated intravascular coagulation (DIC)
  • Over anti-coagulated patient
  • Management of the anti-coagulated patient undergoing acute surgery
  • Thrombotic thrombocytopenia purpura
  • HELLP syndrome
176
Q

What is HELLP syndrome

A

Haemolysis
Elevated liver enzymes
Low platelet count

177
Q

What are chronic myeloproliferative disorders

A

CMPD
Clonal stem cell disorders of the bone marrow
Malignant

178
Q

What are the three types of CMPD

A

Polycythaemia Vera
Essential thrombocytosis
Idiopathic Myelofibrosis

179
Q

What is polycythaemia vera

A

Increased red cells
±neutrophils
±platelets
Distinguish from secondary polycythaemias and relative polycythaemia

180
Q

What is essential thrombocythaemia

A

Increased platelets

Distinguish from reactive thrombocytosis

181
Q

What is myelofibrosis

A

Variable cytopenias with a large spleen

Distinguish from other causes of splenomegaly

182
Q

What are the signs and symptoms of polycythaemia vera

A 
Symptoms:
Insidious itching
Plethoric face (red)
Headache
Muzziness
General malaise
Tinnitus
Peptic ulcer
Gout
Gangrene of the toes

Signs:
Plethora
Engorged retinal veins
Splenomegaly

183
Q

How is PV diagnosed

A 
Persistent increased Hb/hct > 0.5 
Relative vs absolute
Primary vs secondary 
History and exam
FBC
Ferritn
Epo level
U and E/ LFT
184
Q

What are causes of secondary polycthaemia

A 
Central hypoxic process
Renal disease
EPO producing tumours
Drug associated
Congenital 
Idiopathic erythrocytosis
185
Q

What does the presence of JAK2 V617F mutation show

A

In peripheral blood DNA it is diagnostic of a myeloproliferative disorder

186
Q

How is PV treated

A

Aspirin 75mg daily

Aim for HCT<0.45

187
Q

What is the prognosis for PV

A

Good- 15 year median survival

Risk of developing AML and/or myelofibrosis

188
Q

How is thrombocytosis investigated

A 
History and exam
Recent normal count prior to surgery
Persistent platelets >450 x 109/L
1st line:
-FBC and film
-Ferritin
-CRP
-CXR
-ESR
2nd line:
-JAK2
-CALR
-Bone marrow biopsy
-Extensive search for secondary cause
189
Q

What is CALR mutation

A

Calreticulin mutation
Cell signalling protein produced in endoplasmic reticulin
Mutation in EXON 9 of gene
Found in Myeloid progenitors in essential thrombocythaemia

190
Q

How is ET diagnosed

A

JAK2 mutation- approx 50%
CALR mutation- approx 45%
Bone marrow

191
Q

How is ET treated

A

Assess thrombotic risk
Antiplatelet treatment (aspirin 75mg daily)
Cytoreduction (if high risk)

192
Q

What performs cytoreduction

A

Hydroxycarbamide
Interferon
Anagrelide
P32

193
Q

What is the prognosis of ET

A

Excellent- 20 year median survival
Risk of AML or myelofibrosis
CALR mutated have lower thrombosis risk

194
Q

How does myelofibrosis present

A

Pancytopenia
B symptoms
Massive splenomegaly

195
Q

How is myelofibrosis investigated

A

FBC and film

Haematinics

196
Q

How is myelofibrosis diagnosed

A

Blood film
Bone marrow results
JAK2 mutation 50%
CALR mutation 30%

197
Q

What are the causes of splenomegaly

A 
CHICAGO
Cancer
Haematological (myelofibrosis, CML, CLL, hairy cell leukaemia)
Infection (schistosomiasis, malaria, leishmaniasis, EBV)
Congestion (liver disease/portal)
Autoimmune (haemolysis, SLE)
Glycogen storage disorders
Other - Amyloid, sarcoid
198
Q

What are the treatments of splenomegaly

A

Supportive care
JAK2 inhibitors
Bone marrow transplant

199
Q

What is the prognosis of splenomegaly

A

Poor with median survival of 5 years

200
Q

How is chronic myeloid leukaemia characterised

A

Leucocytosis
Leucoerythroblastic blood picture
Anaemia
Splenomegaly

201
Q

What are the symptoms of chronic myeloid leukaemia

A 
Abdominal discomfort (splenomegaly)
Abdominal pain (splenic infarction)
Fatigue (anaemia, catabolic state)
Venous occlusion (retinal vein, DVT, priapism)
Gout (hyperuricaemia)
202
Q

What are the causes of acute leukaemia

A

Result of accumulation of early myeloid (AML) or lymphoid (ALL) precursors in bone marrow, blood and other tissues
Probably occurs by somatic mutation in a single cell within a population of early progenitor cells
May arise de novo or secondary to prior chemotherapy/radiotherapy or develop from another haematological condition

203
Q

What is the median age at presentation of AML

204
Q

What are the clinical features of AML

A 
Presents with features of bone marrow failure:
-anaemia
-infections
-early bruising and haemorrhage
Organ infiltration by leukaemia cells may occur in:
-spleen
-liver
-meninges
-testes
-skin
205
Q

What does AML mean

A

Acute monocytic leukaemia

206
Q

What are the haematological features of AML

A
  • Anaemia
  • Low or high white cell count with circulating leukaemia cells
  • Low platelets
207
Q

How is AML diagnosed

A

Morphology
Immunological markers
Cytogenetics (chromosomes) (certain abnormalities correlate with prognosis eg t(8;21) inv(16) and t(15:17))

208
Q

What is important for prognosis in AML

A 
Age 
Chromosomes
Molecular features (NPM1 and FLT3-ITD)
Extramedullary disease
Disease that doesn't respond to treatment
209
Q

Up to what age should AML patients be considered for intensive treatment

A

Up to age 80 yo

210
Q

What is the intensive chemotherapy regime for AML and the risks

A 
3-4 cycles of intravenous cytotoxic drugs given centrally
80-85% complete remission after cycle 1
Disease assessment after 1st cycle
High risk patients go on to have a bone marrow transplant 
Risk:
-death
-sepsis
-alopecia
-infertility
-tumor lysis
211
Q

How factors determine who to target with intensive vs non-intensive chemo

A 
Age
Co-morbidity
Body habits
Lifestyle decisions
Cytogenetics
Molecular information
212
Q

When should patients be given immediate intensive chemotherapy treatment

A

Critically ill patients with rapidly progressive disease (such as WCC>100 x 10^9/L) with respiratory/ neurological/ other organ compromise

All other patients: no proven benefit to early initiation of treatment, wait for cytogenetics and mutational status prior to deciding on definitive therapy

213
Q

What are the options for non-intensive treatment in AML

A

Low dose chemotherapy (cytarabine)
Hypomethylating agents
New treatments

214
Q

What do the newer treatments available to patients with AML do

A

Target specific abnormalities expressed on leukaemia cells
Provide individualised treatments
Used in combination with chemo or on their own

215
Q

What does ALL mean

A

Acute lymphoblastic leukaemia

216
Q

How does ALL present

A 
Fatigue
Bruising/bleeding
Weight loss
Weight sweats
Hepatosplenomegaly
Lymphadenopathy
Mediastinal mass
217
Q

What is the general course of treatment for ALL patients

A

4 components:

  • Induction (8 weeks)
  • Intensification/ CNS prophylaxis (4 weeks)
  • Consolidation (20 weeks)
  • Maintenance (2 years)

If high risk proceed to bone marrow transplant after intensification

218
Q

What are the treatment options for relapse disease of ALL

A 
Further intensive chemo
Blinatumomab
Inotuzumab
CAR-T cells
BMT- sib/MUD/Cord/Haplo
219
Q

What are immunotoxins/ immunoconjugates

A

Monoclonal antibodies/ cell antigen binding fragment and a toxin moiety which induces cell death
Markedly increases activity of the antibody
CD22 most attractive target

220
Q

What is neutropenic sepsis and how is it treated

A

Life threatening complication of chemotherapy
Time critical medical emergency
Symptoms:
-fever
-hypotension
-organ impairment
Treat with broad spectrum IV antibiotics as soon as suspected

221
Q

What is MDS

A

Myelodysplasia
Represents several related disorders with common features
A heterogeneous group of clonal bone marrow stem cell disorders that result in ineffective haematopoiesis with reduced production of one or more of the peripheral blood cell lineages
Incidence of MDS increases with age

222
Q

What are the features of MDS

A

Dysplasia
Inefficient haematopoiesis
Cytopenias
Increased risk of transformation to AML

223
Q

What should be the procedure for low risk MDS

A

May only need to monitor the patient
Only treat if symptomatic
Erythropoietin for anaemia
Blood product support as necessary

224
Q

What should be the procedure for high risk MDS

A

Treatment aimed at altering natural history of the disease
If fit enough treat as per AML with intensive chemotherapy and bone marrow transplant
If not fit or complex cytogenetics consider azacytidine

225
Q

What are immunoglobulins

A

Glycoprotein molecules
Produced by plasma cells in response to an immunogen
Composed of two light chains and two heavy chains held together by covalent disulphide bonds
Each chain has one variable and one constant region

226
Q

How are immunoglobulins classified

A

According to amino acid sequences in the constant region of the:
Heavy chains: IgG, IgM, IgA, IgD, IgE
Light chains: kappa or lambda

227
Q

What is protein electrophoresis

A

The lab technique whereby serum is placed in a gel and exposed to an electric current
Five major fractions are normally identified:
-Serum albumin
-Alpha-1 globulins
-Alpha-2 globulins
-Beta globulins
-Gamma globulins

228
Q

What is immunofixation

A

Enables the detection and identification of monoclonal immunoglobulins
Performed when M spike seen on electrophoresis
Serum or urine is placed on a gel and electric current applied to separate the proteins
Anti-immunoglobulin antisera is added to each migration lane
If the immunoglobulin is present, a complex precipitates

229
Q

What is myeloma

A

An incurable malignant disorder of clonal plasma cells

230
Q

What is diagnostic of myeloma

A

IMWG diagnostic criteria:
Clonal BM plasma cells >/= 10% or biopsy-proven boney or extra medullary plasmacytoma and any one or more of:
-CRAB features
-MDEs

231
Q

What are the CRAB features

A

C- hypercalcamia (>2.75mmol/L)
R- renal insufficiency (creat clearance <40ml/min or serum creat >177micromol/L)
A- anaemia (Hb<100g/L)
B- bone lesions (one or more osteolytic lesions on skeletal radiography, CT, or PET/CT

232
Q

What are MDEs

A

Myeloma-defining events:

  • > /= 60% clonal plasma cells on bone marrow biopsy
  • SFLC ratio >100mg/L provided the absolute level of the involved LC is >100mg/L
  • > 1 focal lesion on MRI measuring >5mm
233
Q

What are the clinical features of myeloma

A 
Confusion
Poor appetite
Thirst 
Chest infections
Breathlessness
Polyuria or oliguria/ anuria
Peripheral oedema
Constipation
Pathological fractures
Nausea
Bone pains
234
Q

What is the normal range of Hb

A

130-180g/dL

235
Q

What is the normal range of creatine

A

40-90mmol/L

236
Q

What is the normal range of Ca2+

A

2.2-2.6mmol/L

237
Q

What is MGUS

A

Serum M-protein <30g/L
<10% clonal plasma cells in the bone marrow
Absence of end organ damage (CRAB)
Majority progress to myeloma

238
Q

What is the normal range for albumin

239
Q

What are lymphomas

A

Caused by malignant proliferation of lymphocytes
Lymph nodes are predominantly affected though in advanced stages, there may be bone marrow involvement and other organ involvement
Classified according to the presence of Redd-Sternberg cells

240
Q

What are important factors to consider is a patient presents with a neck lump

A 
Nature of lump (size, rate of change, tenderness, skin changes, history of trauma)
Additional lumps elsewhere
Weight loss
Night sweats
Breathlessness, cough, haemoptysis
PMH (malignancies)
SH (smoking)
FH (bone marrow disorders or malignancies)
241
Q

What will clinical examination of patient with neck lump focus on

A

Nature of the lump – size, location, skin changes, contour, whether fixed to underlying structures
Evidence of additional neck masses
Presence of palpable lymphadenopathy
Presence of hepatosplenomegaly
Presence of breast lumps
Chest examination – insection, auscultation and percussion

242
Q

What are the possible causes of a neck mass

A

Malignant:

  • lymphoma
  • chronic lymphocytic leukaemia
  • metastatic cancer of the lung/ breast/ cervix

Non-malignant:

  • Infection (bacterial, viral, mycobacterial)
  • Inflammation (sarcoidosis)
  • Lipoma
  • Fibroma
  • Haemangioma
243
Q

What investigations should be done on a patient with neck lump

A

Bloods:

  • FBC
  • U&Es
  • LFTs
  • Ca2+
  • LDH
  • Immunoglobulins and protein electrophoresis

Imaging:

  • Chest X-ray
  • Ultrasound scan of the neck lump
  • Fine needle aspirate and/or core needle biopsy
244
Q

What is follicular lymphoma

A

Neoplastic disorder of lymphoid tissue

Type of non-Hodgkin lymphoma characterised by slowly enlarging lymph nodes

245
Q

What are the important factors in the history of a patient presenting with breathlessness

A

Nature of the breathlessness – rate and duration of onset, variability with activities, exacerbating and relieving factors
Additional symptoms – cough, sputum production, ankle swelling, orthopnoea, PND, weight loss, night sweats
Past medical history – childhood illnesses
Social history – smoking, occupational and animal exposure
Family history – any history of respiratory/cardiac problems

246
Q

What are the important factors clinical examination features of a patient presenting with breathlessness

A

Chest and cardiovascular examination

Lymphadenopathy

247
Q

What are the investigations done on a patient presenting with breathlessness

A

Bloods:

  • FBC
  • U&Es
  • LFTs
  • LDH
  • ACE level
  • ESR

Imaging:

  • Chest -ray
  • PET-CT
248
Q

What are the characteristic of Hodgkin lymphoma

A

Presence of Hodgkin Reed-Sternberg (HRS) cells within a cellular infiltrate of non-malignant inflammatory cells eg: eosinophils

HRS fail to express surface immunoglobulin and evade apoptosis through several mechanisms – eg: activation of NFkB, incorporation of EBV and latent membrane proteins (LMP1 and LMP2)

249
Q

How is Hodgkin lymphoma managed

A

Chemotherapy
Radiotherapy
Doses/number of courses depends on stage

250
Q

What is the prognosis of Hodgkin lymphoma

A

High proportion are cured – 86% 5 year survival

Long-term effects of therapy are important:

  • Increased mortality is still seen at >20 years post therapy
  • Pulmonary toxicity
  • Cardiovascular disease
  • Secondary malignancies
251
Q

What is CLL

A

Chronic lymphocytic leukaemia
A malignant disorder of mature B cells
Most common type of leukaemia in UK

252
Q

What is the treatment for CLL

A

Chemo-immunotherapy (bendamustine and rituximab)

Bone marrow treatment

Y2 Clinical Pathology (11 decks)

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