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Phase 2a LM > Haematology Diseases > Flashcards

Haematology Diseases Flashcards

1
Q

What is anaemia

A

Reduced RBC mass +/- reduced Hb conc in plasma

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2
Q

What is the lifespan of RBCs

A

120 days

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3
Q

What are the 3 causes of microcytic anaemia

A

Iron deficiency
Thalassaemia
Anaemia of chronic disease

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4
Q

Why might someone be iron deficient

A

Not enough in diet
Haemorrhage - heavy periods
Breast milk iron def
Hookworm

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5
Q

What are the 3 causes of normocytic anaemia

A

Combined haematinic deficiency - B12 and iron
Acute blood loss
Chronic disease

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6
Q

What are the 4 causes of macrocytic anaemia

A

B12/folate def
Hypothyroidism
Excess alcohol consumption/liver disease
Haematological - cancer, bone marrow failure

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7
Q

What is polycythaemia

A

Too many RBCs

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8
Q

What is the main cause of polycythaemia (and other causes)

A

Smoking

Lung disease
Cyanotic heart disease
Polycythaemia rubra Vera (PRV)

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9
Q

What is neutrophilia

A

Too many neutrophils

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10
Q

What are the main causes of neutrophilia

A

Bacterial infection
Inflammation
Malignancies
Chronic myeloid leukaemia

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11
Q

What is the term for too many WBCs

A

Lymphocytosis

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12
Q

What are the causes of lymphocytosis

A

Infection inflammation malignancy

Chronic lymphocytic leukaemia

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13
Q

What is the term for high platelets

A

Thrombocytosis

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14
Q

What are the causes of thrombocytosis

A 
Infection inflammation malignancies 
Essential thrombocythaemia (over active bone marrow)
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15
Q

What is thrombocytopenia

A

Low platelets

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16
Q

What is the term for low neutrophils

A

Neutropaenia

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17
Q

What are the underproduction and increased removal causes of neutropaenia

A

Underproduction = marrow failure/infiltration/toxicity

Increased removal = autoimmune, felty’s syndrome, cyclical

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18
Q

When the endothelium is damaged, what do platelets adhere to it via

A

Collagen and vWF

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19
Q

What does the binding of platelets to collagen/vWF stimulate

A

Cytoskeleton shape change -> ‘spread out’

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20
Q

When platelets are activated by a change in shape, granules are released containing what?

A

ADP, fibrinogen, thrombin, calcium

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21
Q

Activated platelets are cross linked, and aggregation occurs by what molecule

A

Fibrin

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22
Q

What is the lifespan of platelets

A

7-10 days

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23
Q

What are the clinical features of platelet dysfunction

A

Mucosal bleeding - gums, nose, large menstruation
Easy bruising
Petechial, purpura
Traumatic haematomas

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24
Q

Give some causes of thrombocytopenia due to decreased production

A

Congenital = reduced/malfunctioning megakaryocytes
Infiltration of BM = leukaemia, myeloma, lymphoma, mets
Reduced platelet production by BM = low B12/folate, reduced TPO

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25
Give some causes of thrombocytopenia due to increased destruction
Autoimmune = immune thrombocytopenia Hypersplenism = portal HTN, splenomegaly Drug related = heparin induced thrombocytopenia Consumption of platelets = DIC, HUS, major haemorrhage
26
Name 3 types of medication affecting platelets
``` Aspirin = inhibit COX 1 Clopidogrel = blocks P2Y12 Tirofiban = blocks Gp IIb/IIIa ```
27
How many globin chains does a normal Hb molecule have
4: 2 alpha and 2 beta
28
What is the amino acid substitution in sickle cell
Valine -> glutamine
29
What is a carrier of sickle cell protected against
Falciparum malaria
30
Where is the DNA is the mutation in sickle cell
Beta globin gene on chromosome 11
31
What are the disease modifying Tx for sickle cell
Transfusion Hydroxycarbamide Stem cell transport
32
What is thalassaemia
Reduced production of alpha or beta globin chains
33
What are the main deletions and mutations in thalassaemia
Deletions - alpha thalassaemia Mutations - beta thalassaemia (Mainly)
34
What are the 3 categories of thalassaemia
Thalassaemia major - require transfusions to survive Thalassaemia intermedia - can survive without transfusion Thalassaemia carrier - asymptomatic
35
Are membranopathies autosomal recessive or dominant
Dominant
36
Give one example of an enzymopathy
Glucose-6-phosphate dehydrogenase deficiency
37
What is leukaemia
Cancer of the bone marrow with abnormally high levels of white blood cells
38
What cell/cells are affected in acute myeloid leukaemia
Myeloblasts
39
What do myeloblasts differentiate into
Neutrophils, eosinophils, basophils, monocytes
40
What are the cell/cells affected in chronic myeloid leukaemia
Any of: basophils, eosinophils, neutrophils
41
What are the cell/cells affected in acute lymphoblastic leukaemia
Lymphoblasts
42
What do lymphoblasts differentiate into
T lymphocyte | B lymphocyte
43
What is the cell/cells affected in chronic lymphocytic leukaemia
B lymphocytes
44
What are the 3 signs/presentation of bone marrow problems
Anaemia - fatigue, SOB Thrombocytopaenia - rash, bruises, spots Infections - fever, rigors
45
What is the sign of an acute leukaemia
>20% of bone marrow is blast cells
46
What is the age, the cause and the diagnosing detail of acute myeloid leukaemia
Age: 70 yrs (rare in children) Cause: complication of chemo Diagnosis: Auer rods in myeloblasts
47
What is the most common paediatric cancer
Acute lymphoblastic leukaemia
48
What are some of the signs/symptoms of acute lymphoblastic leukaemia
Marrow failure - infection, anaemia, bleeding | Tissue infiltration - lymphadenopathy
49
What is the cause of chronic myeloid leukaemia
Philadelphia chromosome
50
What is the Philadelphia chromosome
A translocation of chromosome 9 ->22
51
What does the Philadelphia chromosome cause
Increase in tyrosine kinase that then increases the number of affected WBCs
52
What age group does chronic myeloid leukaemia typically affect
40-60 yr olds
53
What is the most common leukaemia
Chronic lymphocytic leukaemia
54
What are the Tx for chronic lymphocytic leukaemia
Blood transfusions Chemo Rituximab (anti CD20 - found on B lymphocyte surface)
55
What is lymphoma
Malignant growth of WBCs - predominantly in the lymph nodes
56
What are most caused by
Unknown! Immunodeficiency - HIV, transplant recipients Infection AI
57
What is the difference between Hodgkin's lymphoma and non-hodgkins lymphoma
Hodgkin's lymphoma has the presence of Reed-Sternberg cells in the lymph node biopsy, but NHL doesnt
58
What is the presentation of Hodgkin's lymphoma
Enlarged and painless nodes
59
What are B symptoms
Sweats, weight loss, fever, pruritus and lethargy | Systemic features of cancer
60
What are the 4 clinical stages of Hodgkin's lymphoma
``` 1= single lymph node region 2= two or more regions on the same side of the diaphragm 3= nodes on both sides of diaphragm 4= spread beyond the lymph nodes A= absence of B symptoms B= presence of B symptoms ```
61
What is the Tx for Hodgkin's lymphoma
Stage 1-2A: chemo then radio | Stage 2-4B: combined chemo
62
What are the two types of NHL
Low grade or high grade
63
What is an example of low grade NHL
Follicular lymphoma
64
What is an example of a high grade NHL
Diffuse large B cell
65
What are the Tx for the high grade NHL
Short course chemo and radio | Combination chemo and monoclonal Abs e.g. Rituximab
66
What is myeloma
Cancer of differentiated B lymphocytes known as plasma cells
67
What's the peak age for myeloma
70
68
What are the features of myeloma
Malignant plasma cells accumulate in the BM- BM failure Abnormal Ab and short chain (paraproteins) produced by the plasma cells Renal failure Osteoporosis
69
How do you diagnose myeloma
1. Monoclonal plasma cells in BM >10% 2. Monoclonal Ab (paraprotein) in serum or urine (bence jones) 3. One or more of: C- calcium - hypercalcaemia R- renal failure A- anaemia B- bone - lytic lesions, osteoporosis, spinal cord compression
70
Name some chemicals the malignant plasma cells produce in the bone
IL3 (stops osteoblasts progenitors developing) DKK1 (inhibits OPG from stopping RANK-L binding to RANK) MIP1-alpha (increase osteoclasts activity) RANK-L (stimulates osteoclasts activity)
71
What is a common source of neutropaenia sepsis
Chemo (recently had)
72
What are the symptoms of neutropenic sepsis
Fever Rigors Generally 'off' Low BP
73
What to do is suspect neutropaenic sepsis
1. See patient ASAP 2. Put cannula in before history 3. ANTIBIOTICS before history
74
Sickle cell disease crisis patient in agony, what do you do
1. See patient 2. Control pain 3. Put cannula in, start fluids and oxygen
75
If sickle cell leads to acute chest syndrome (lung infarction and hypoxia then worse sickle) what do you do
1. See patient 2. give fluids/Abx and oxygen 3. Call haem (need RBC exchange)
76
What are the symptoms of the myeloma complication of spinal cord compression
Back pain Neuropathic pain Leg weakness/numbness Saddle anaesthesia
77
What to do if suspect spinal cord compression
1. Keep in bed 2. Large dose of steroid - dexamethasone 3. MRI
78
Complication of myeloma: hyperviscosity syndrome, symptoms
``` Non-specific Headaches Blurred visions SOB Mucosal bleeding ```
79
What to do if suspect hyperviscosity syndrome
1. Put cannular in 2. Fluids 3. Call haem
80
What is tumour lysis syndrome
Where chemo has killed cancer cells, but they release all their intracellular contents and the kidneys become overwhelmed
81
What are the 4 common signs of tumour lysis syndrome
Increased potassium Increased phosphate Increased Uric acid Decreased calcium
82
What's the Tx for tumour lysis syndrome
LOTS OF FLUID | Manage electrolytes

Phase 2a LM (7 decks)

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