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Haematology > Introduction: The Scope of Clinical Haematology > Flashcards

Introduction: The Scope of Clinical Haematology Flashcards

1
Q

What are the blood components?

A

Plasma - about 1/2

  • clotting/coagulation factors
  • albumin
  • antibodies

Buffy coat

  • platelets
  • white cells/leucocytes

Red blood cells

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2
Q

What are the functions of blood?

A

Transport

  • gases, oxygen and CO2 in red cells
  • nutrients, waste, communication in plasma

Maintenance of vascular integrity

  • prevention of leaks - platelets and clotting factors
  • prevention of blockages - anticoagulants and fibrinolytic agents

Protection from pathogens

  • phagocytosis and killing - granulocytes/monocytes
  • antigen recognition and antibody formation - lymphocytes
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3
Q

What is the pathogenesis of haematological abnormality?

A

High levels - increased rate of production, decreased rate of loss

Low levels (more common) - decreased rate of production, increased rate of loss

Altered function

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4
Q

Where are blood cells made? What are they derived from?

A

In the bone marrow
Derived from stem cells

Lymphocyte stem cells split off very early

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5
Q

What are the types of WBC?

A 
Neutrophils
Monocytes
Basophils
Eosinophils
Lymphocytes
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6
Q

What are the types of myeloid cells?

A 
Erythrocytes
Platelets
Neutrophils
Monocytes
Basophils 
Eosinophils
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7
Q

What are the features of stem cells?

A

Totipotent
Self-renewal
Home to marrow niche - CSCR4

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8
Q

As stem cells divide, what do they go through?

A

Binary fission and flux through differentiation pathways

Amplify numbers

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9
Q

What is flux regulated by?

A

Hormones, growth factors

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10
Q

What kind of cells make up most of the bone marrow?

A

Fat cells

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11
Q

Where in the bone marrow do stem cells usually lie?

A

Near the bone, moving closer to the centre as they differentiate
In most bones in children and axial bones in the elderly

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12
Q

What are the histological features of bone marrow?

A

Stroma and sinusoids

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13
Q

What is the normal RBC shape?

A

Bi-concave

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14
Q

What are erythroblasts known as when they are released into the circulation?

A

Reticulocytes (immature RBC)

These then become erythrocytes

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15
Q

What are reticulocytes useful for?

A

Can provide a good measurement of how many RBCs are being produced in around 24 hours

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16
Q

Where is erythropoietin made?

A

In the kidney in response to hypoxia

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17
Q

What haematological abnormality might develop in chronically hypoxic people?

A

Polycythaemia

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18
Q

What are the causes of anaemia?

A

Decreased production

  • deficiency in haematinics e.g. iron, B12, folate
  • thalassaemia

Increased loss

  • bleeding
  • haemolysis
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19
Q

How many RBCs can be made per day?

A

Around 10g/L/day

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20
Q

What are platelets formed from?

A

Megakaryocytes

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21
Q

What are the functions of platelets?

A

Haemostasis and immune functions

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22
Q

What regulates the production of platelets?

A

Thrombopoietin - produced in liver, regulation by platelet mass feedback

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23
Q

What is the lifespan of platelets?

A

7 days

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24
Q

What are the platelet pathologies?

A

Too many

  • thrombocytosis
  • myeloid malignancies, reactive

Thrombocytopenia
- marrow failure, immune destruction

Altered function
- aspirin, clopidogrel, abciximab etc.

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25
What is the function of neutrophils?
To ingest and destroy pathogens, especially bacteria and fungi
26
What are neutrophils regulated by?
Immune responses - macrophage, IL-17
27
What is the lifespan of neutrophils?
1-2 days
28
What is the speed of response of neutrophil?
A few hours
29
When are neutrophils raised?
Infection - G-CSF - left shift, toxic granulation Inflammation - post-operative, MI, RA
30
What are the causes of neutropenia?
Lowered Racial - more common in certain populations Decreased production - drugs - marrow failure Increased consumption - sepsis - autoimmune Altered function e.g. chronic granulomatous disease
31
What is the function of the monocytes?
To ingest and destroy pathogens, especially bacteria and fungi Subset of monocytes migrate into tissues and become macrophages, main function is to migrate into tissues where they can differentiate into macrophages and survive for many months
32
What is the lifespan of macrophages?
Many months
33
What is monocytosis associated with?
Usually associated with neutrophilia | Mycobacterial inflections
34
What is monocytopenia associated with?
Mycobacteria in hairy cell leukaemia
35
What is the main role of eosinophils?
Historically main role was in fighting off parasitic infections, more commonly now in allergy
36
What is lymphocytosis associated with?
Infectious mononucleosis | Pertussis
37
What is lymphopenia associated with?
Usually post-viral | Lymphoma
38
What are the subtypes of lymphocytes?
B cells T cells Natural killer cells
39
Where are lymphocytes produced?
Produced in bone marrow
40
Where do B and T cells mature?
B cells mature in bone marrow | T cells mature in thymus
41
What do lymphocytes differentiate into in secondary lymphoid organs?
Effector cells
42
What are the regions of the B/T cell receptor?
Variable region at the top and constant region at the bottom
43
What are the roles of antibodies?
Adaptors between pathogens and clearance systems Opsonisation Fix complement Block binding IgG immunoglobulin most common, but many other types
44
What happens in the creation of a receptor chain gene?
Shuffling of exons at a time when lymphocytes are differentiating In gremlin DNA, 4 common enzymes are seen, joined up together in a random fashion (recombination)
45
What are the types of repertoire diversity and what do mistakes in these processes cause?
Combinatorial diversity Junctional diversity Combinational diversity Mistakes in processes cause lymphoid malignancy
46
What is the positive and negative selection in the bone marrow?
If the gene rearrangement results in a functional receptor, the cell is selected to survive - positive selection If the receptor recognises self-antigens, the cell is triggered to die - negative selection B cells that survive this selection are exported to the periphery
47
What are the classes of human leucocyte antigen (HLA)?
Class I - displays internal antigens on all nucleated cells | Class II - displays antigens eaten by professional antigen-presenting cells
48
What do HLA molecules help cells to identify?
Self vs non-self cells or uninfected vs infected cells
49
Give examples of blood disorders that occur due to systemic diseases
``` Anaemia of chronic diseases Iron deficiency Folate deficiency Immune haemolysis Neutrophilia Immune thrombocytopenia Cytopenia secondary to medication Felty syndrome ```
50
``` Give examples of blood disorders caused by; hepatic renal cardiovascular respiratory GI disease ```
Hepatic - anaemia, deficient clotting factors Renal - anaemia, haemolytic uraemic syndrome Cardiovascular - anaemia Respiratory - polycythaemia GI - anaemia
51
What are the components of the full blood count?
``` Haemglobin RBC Platelets WBC Neutrophils Lymphocytes ```
52
What is the normal Hb range?
Males - 135-170g/L | Females - 120-160g/L
53
What is the normal RBC range?
4-5 x 10^12/L
54
What is the normal platelet range?
150-400 x 10^9/L
55
What is the normal WBC range?
4-10 x 10^9/L
56
What is the normal neutrophil range?
1.5-7 x 10^9/L
57
What is the normal lymphocyte range?
1.5-4 x 10^9/L
58
What is affected by plasma disorders?
Too much - paraproteins Too little - clotting factors, haemophilia Abnormal function - clotting factors, haemophilia
59
What are the main haematological tests?
``` FBC Clotting times for clotting factors Bleeding time for platelets Platelet and leucocyte function tests Chemical assays - iron (ferritin), B12, folate Marrow aspirate and trephine biopsy Lymph node biopsy Imaging ```
60
What is the spleen composed of?
Red pulp and white pulp
61
What are the haematological effects of spleen disorders?
Hypersplenism - pancytopenia | Hyposplenism - infections with encapsulated bacteria, red cell changes
62
What are the causes of splenomegaly?
Infectious - acute e.g. EBV, CMV - chronic bacterial e.g. TB - chronic parasitic e.g. malaria Haematological - malignancy - leukaemias and lymphomas - myeloproliferative disorders Portal hypertension Haemolytic disorders - hereditary spherocytosis, thalassaemia, haemoglobinopathies - megaloblastic anaemia - autoimmune Connective tissue disorders - SLE - Felty syndrome Miscellaneous - sarcoid - malignancy - amyloid Storage pool disorders - Gaucher disease - Niemann Pick
63
What are the main haematology treatments?
Replacement - blood - haematinics - coagulation factors - plasma exchange Transplantation ``` Drugs - cytotoxics - monoclonal antibodies inhibitors of cellular proliferation - immunosuppressants - inhibitors of coagulation - inhibitors of fibrinolysis ```

Haematology (11 decks)

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