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Haematology Flashcards

1
Q

How are haematological malignancies classified?

A

By disease behaviour and lineage:
Acute/chronic
Myeloid/lymphoid

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2
Q

What type of malignancy can occur before monoclonal selection of B lymphocytes in the lymph node?

A

Mantle cell lymphoma

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3
Q

What is the aetiology of Burkitt’s lymphoma?

A

Diffuse large B-cell lymphoma arising in the lymph node following clonal expansion

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4
Q

What type of haematological malignancy is formed from plasma cells?

A

Myeloma

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5
Q

What is multiple myeloma?

A

An accumulation of malignant differentiated B lymphocytes (plasma cells) in the bone marrow, which leads to progressive bone marrow failure.

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6
Q

How is multiple myeloma diagnosed?

A 
Spikey old CRAB:
Spikey - Malignant B cells characteristic 'M spike' paraprotein (useless antibody)
Old - average age 70
CRAB:
Calcium (hypercalcaemia)
Renal impairment
Anaemia
B-lymphocytes
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7
Q

How does myeloma usually progress?

A

Starts with asymptomatic phase (MGUS/smouldering myeloma), progresses to symptomatic phase (active myeloma), then a pattern of remission and relapse with remission periods getting shorter until refractory relapse occurs.

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8
Q

What is the most common haematological cancer?

A

Non-Hodgkin lymphoma

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9
Q

What is MGUS?

A

Monoclonal Gammopathy of Undetermined Significance - <30g/l paraprotein, <10% of plasma cells in the bone marrow with no evidence of amyloid/lymphoproliferative disorder. May or may not progress, requires no treatment.

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10
Q

What is smouldering myeloma?

A

> 30g/l paraprotein and/or >10% plasma cells in bone marrow, but no organ or tissue impairment. May progress to symptomatic myeloma. No treatment.

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11
Q

How is symptomatic myeloma diagnosed?

A

Paraprotein >30g/l and/or >10% of plasma cells in bone marrow
+ evidence of tissue/organ impairment or presence of amyloid

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12
Q

What is systemic AL amyloidosis?

A

Occurs when light chains from immunoglobulins are converted into amyloid due to a protein conformation disorder. Deposition of amyloid leads to multiple organ disease.

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13
Q

How can systemic AL amyloidosis present?

A 
Nephrotic syndrome +/- renal impairment
Congestive cardiomyopathy
Sensorimotor and/or autonomic neuropathy
GI disturbance
Hepatomegaly
In rare cases - bleeding leads to racoon eyes and macroglossia
Fatal within 2 years if untreated
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14
Q

What tests would you use to establish a diagnosis of myeloma?

A

Bone marrow aspirate with trephine biopsy, immunofixation of serum and urine, whole body MRI/low dose CT/PET CT

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15
Q

What is ‘free light chain myeloma’?

A

When the myeloma cells produce only free light chains instead of producing an intact immunoglobulin product as well.

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16
Q

Myeloma bone disease is a potential complication of myeloma. How does it present?

A

Lytic lesions, other skeletal complications, bone pain, hypercalcaemia

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17
Q

What drugs can be used to decrease osteoclast activity in myeloma bone disease?

A

Zoledronic acid and denosumab

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18
Q

What supportive treatments are given to myeloma patients?

A

Bisphosphonate, blood transfusions/EPO injections, antibiotics (as required), analgesia (as required), radiotherapy, kyphoplasty if indicated, psychological support.

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19
Q

Name some chemo drugs that inhibit microtubules during G2 phase

A

vinblastine, vincristine, vinorelbine, docetaxel, paclitaxel

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20
Q

Name some antimetabolite drugs that stop cells producing building blocks of DNA during the G1 phase

A

methotrexate, azathioprine

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21
Q

How does sickle cell disease cause hypoxia?

A

HbS polymerises when deoxygenated, change in shape causes blockage of blood vessels, leading to ischaemia and sequestration. This also leads to chronic haemolysis, which results in low baseline haemoglobin.

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22
Q

How is sickle cell disease diagnosed?

A

Sickle solubility test. Haemaglobin can be separated using techniques such as electrophoresis. Can also be diagnosed antenatally using molecular genetics techniques.

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23
Q

Why are patients with sickle cell disease at increased risk of sepsis?

A

Because hyposplenism is a complication of sickle cell disease.

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24
Q

How might a patient suffering with a sickle cell disease acute crisis present?

A

Severe pain, can also lead to acute chest syndrome with chest pain, cough, fever and hypoxia

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25
How is an acute sickle cell crisis treated initially?
Analgesia (subcutaneous oxycodone, paracetamol, NSAIDs) and fluids
26
Why might an episode of acute chest syndrome require an exchange blood transfusion?
The lung damage leads to hypoxia, which causes HbS polymerisation, which reduces blood flow and causes further lung damage --> cycle repeats
27
What is thalassaemia?
Reduced haemoglobin production
28
What is the difference between alpha and beta thalassaemia?
Alpha thalassaemia is caused by an abnormality of the alpha globin gene and beta thalassaemia is caused by an abnormality of the beta globin gene.
29
What is the inheritance pattern of alpha/beta thalassaemia?
Autosomal recessive
30
What are the characteristic features of a thalassaemia carrier's blood?
Hypochromia, microcytosis. May be mildly anaemic.
31
What are three types of Non-transfusion-dependent thalassaemias?
Beta thalassaemia intermedia Mild/moderate HbE/beta-thalassaemia Alpha thalassaemia intermedia (HbH disease)
32
Transfusion-dependent beta thalassaemia patients require regular blood transfusions for survival. What are the potential complications of this?
Iron overload, which can damage the heart, liver and endocrine organs. Deaths are usually due to sepsis or cardiac iron overload.
33
What diseases are tested for on a newborn Guthrie (heel prick) test?
``` Sickle cell disease Cystic fibrosis Congenital hypothyroidism Severe combined immunodeficiency (SCID) + inherited metabolic diseases (phenylketonuria, MCADD, maple syrup urine disease MSUD, isovaleric acidaemia IVA, glutaric aciduria type 1 GA1 and homocystinuriea HCU) ```
34
Name two upcoming therapies that may be used to treat haemoglobinopathies in the future
Lentiviral gene therapy | CRISPR/Cas9 genome editing
35
What is the most common malignancy in children?
Acute lymphoblastic leukaemia
36
The cause of ALL is not normally known. Name 5 agents that can cause ALL.
1. Radiation 2. Benzene 3. Smoking 4. Down's syndrome 5. Immunodeficiency
37
How does ALL usually present clinically?
Extreme tiredness, which leads to a FBC, leading to diagnosis. Also bone pain, infection, bleeding, testicular pain.
38
How is ALL treated?
Over many weeks with many chemo cycles with different drugs initially, then consider stem cell transplant/bone marrow transplant.
39
Why might chemotherapy for ALL need to be delivered intrathecally?
Chemotherapy doesn't get through to the brain easily because of the blood brain barrier
40
What are immuno-conjugates?
Chemo drugs used to treat ALL - monoclonal antibodies attached to cytotoxic drug via linker molecule. Allows targeted therapy.
41
Inotuzumab ozogamicin is an immuno-conjugate drug used to treat ALL. What does it target?
CD22, which is expressed on the surface of ALL cells.
42
How can blinatumomab be used to treat ALL?
Blinatumomab is a bi-specific antibody. One end targets CD29, the other end targets CD3. This effectively links a T cell to the ALL cell so the T cell can kill it.
43
What upcoming treatment may become the standard treatment for ALL in the future?
Autologous CAR-T production, which harnesses the patient's own immune system to target cancer by genetically engineering the patient's own T cells to kill ALL cells and then infusing them back into the patient.
44
What differentiates Hodgkin's lymphoma from non-Hodgkin's lymphoma?
Reed-Sternberg cells are present in Hodgkin's lymphoma.
45
What causes white blood cells to become vulnerable to malignant change?
Genetic changes that occur during somatic hypermutation and class switching
46
What is indolent lymphoma?
A slow growing lymphoma that is advanced at presentation. Incurable with a median survival of 9-12 years.
47
Name three subtypes of indolent lymphoma
1. Follicular lymphoma 2. Mantle cell lymphoma 3. Chronic lymphocytic leukaemia (CLL)
48
The cause of indolent lymphoma is not usually know. Give three risk factors.
1. Immunodeficiency 2. Infection e.g. EBV, H-pylori, HTLV-1 3. Autoimmune disorders and associated treatments
49
What investigations are needed to diagnose lymphoma?
Lymph node biopsy (core needle biopsy or excision node biopsy, NOT FNA) Bone marrow biopsy if evidence of bone marrow involvement (e.g. low Hb, low platelets, high WBC)
50
What investigations are required for staging lymphoma?
Depends on spread - imaging with CT neck/thorax/abdomen/pelvis or PET-CT
51
What treatment options are available for indolent lymphomas?
Treatment depends on subtype but possible treatment strategies: 1. Active surveillance (for asymptomatic, advanced stage patients) 2. Radiotherapy (usually for local control) 3. Chemotherapy (alkylating agents, anthracyclines, corticosteroids, monoclonal antibodies)
52
What is anaemia?
A decrease in the number of red blood cells in the body caused by RBC loss exceeding production
53
Name 4 possible causes of anaemia
1. Iron deficiency 2. Folate deficiency 3. B12 deficiency 4. Bone marrow failure
54
What blood tests are relevant to anaemia?
1. Haemoglobin 2. Mean cell volume 3. Mean cell haemoglobin 4. Reticulocyte count (has to be requested separately, not part of standard FBC)
55
How is mean cell volume relevant to diagnosis of anaemia?
Used to classify anaemia in terms of microcytic, normocytic or macrocytic
56
How is mean cell haemoglobin relevant to diagnosis of anaemia?
Anaemia can be described as hypochromic or normochromic.
57
What are reticulocytes and how is reticulocyte count useful in diagnosing anaemia?
Reticulocytes are 'young' red blood cells, which still contain RNA and can be used as an indicator of RBC production. A high reticulocyte count indicates the bone marrow is working overtime to try and compensate for haemolysis. A low reticulocyte count could indicate the patient is not getting enough nutrients to produce red blood cells.
58
What types of anaemia are microcytic?
Iron deficiency anaemia | Thalassaemias
59
What types of anaemia are normocytic?
``` Chronic anaemia Renal disease (acute bleeding) ```
60
What types of anaemia are macrocytic?
Folate deficiency, B12 deficiency, haemolysis, bone marrow disorders
61
How is iron deficiency anaemia diagnosed?
Microcytic hypochromic anaemia noted on FBC Check ferritin levels - low ferritin diagnostic (but ferritin not always low) Check transferrin - normally high
62
How is iron deficiency anaemia managed?
``` Investigate/treat any potential blood loss Replace iron (preferably orally) Continue iron supplementation for 3 months after Hb and MCV return to normal to replenish stores ```
63
What iron studies are used to aid in the diagnosis of anaemia?
1. Ferritin (measure of iron stores) - low levels are diagnostic of anaemia but not always low in anaemic patients. 2. Serum iron (day to day and circadian variation) 3. Transferrin saturation (main protein that iron binds to). Transferrin increased in iron deficiency anaemia as lower proportion occupied.
64
What are the possible causes of folate deficiency?
Poor nutrition, malabsorption (e.g. due to coeliac disease/Crohn's), pregnancy, haemolysis
65
How is folate deficiency treated?
Oral replacement. However, B12 levels must be checked first - replacing folate empirically risks triggering irreversible neurological damage.
66
What is vitamin B12 used for?
DNA and fatty acid synthesis
67
How is vitamin B12 absorbed?
Binds to intrinsic factor (produced by parietal cells) and absorbed in the terminal ileum.
68
What causes vitamin B12 deficiency?
``` Pernicious anaemia Gastrectomy/ileal resection Vegan diet Bacterial overgrowth Oral contraceptives Hypochloridia Nitric oxide ```
69
How is vitamin B12 deficiency treated?
Intramuscular replacement. Consider oral replacement long term if dietary deficiency suspected.
70
What is pernicious anaemia?
An autoimmune condition which leads to the destruction of parietal cells. No intrinsic factor is produced and therefore the body is unable to absorb B12.
71
What is haemolysis?
Reduction in red blood cell lifespan due to increased RBC destruction. If rate of destruction exceeds the rate of synthesis, this causes anaemia.
72
How is haemolysis diagnosed?
``` Blood film - might show spherocytes, polychromasia, red cell fragments Reticulocyte counts Bilirubin Lactate dehydrogenase Haptoglobin Direct antiglobulin test ```
73
What causes haemolysis?
RBC membrane disorders (e.g. hereditary spherocytosis), abnormal haemoglobins (e.g. sickle cell disease), microangiopathic haemolytic anaemias, prosthetic heart valves (can mash up RBCs), autoimmune haemolytic anaemias.
74
Why do testosterone therapy, smoking and lung disease lead to polycythaemia?
Due to raised erythropoietin
75
What is erythropoietin?
A growth factor produced by the kidneys which stimulates the bone marrow to produce red blood cells
76
When should erythropoietin levels be checked?
When investigating the cause of elevated haemoglobin and haematocrit
77
What is haematocrit?
The proportion of blood made up of cells (also known as PCV - packed cell volume)
78
What are the clinical features of polycythaemia vera?
High RBC count High haematocrit Low erythropoietin
79
What is polycythaemia vera?
A rare blood cancer that affects the bone marrow and causes abnormal red blood cell proliferation
80
What is JAK2?
A protein involved in controlling production of blood cells from haematopoietic stem cells
81
How is polycythaemia vera treated?
aspirin +/- statin and hydroxycarbamide (if over 60)
82
What is hydroxycarbamide?
A chemo drug used to treat polycythaemia vera and chronic myeloid leukaemia (and other myeloproliferative disorders)
83
What are the clinical features of essential thrombocythaemia?
- High platelet count - Possible JAK2 mutation - No iron deficiency - ESR and CRP normal (no inflammatory process) - Platelet anisocytosis (variable size) on blood film
84
How is essential thrombocythaemia treated?
<60 aspirin only | >60 hydroxycarbamide
85
What is myelofibrosis?
A rare blood cancer that causes scarring of the bone marrow and makes it more difficult to produce blood cells.
86
What are the features of myelofibrosis?
``` Normal B12/folate/ferritin High LDH (lactate dehydrogenase) Splenomegaly Bone marrow scarring JAK2 mutation ```
87
How is myelofibrosis treated?
``` Depends on risk May require ruxolitinib (JAK2 inhibitor) Erythropoietin injections Analgesia for splenic discomfort Blood transfusion if required ```
88
Besides JAK2, which other mutations are commonly associated with myeloproliferative disorders?
CALR and MPL
89
What are the features of chronic myeloid leukaemia?
B symptoms (lethargy, weight loss, night sweats) Splenomegaly Blood film: lots of mature leukocytes Bone marrow biopsy: WBCs at all stages
90
What drug is normally used to keep CML under control?
Imatinib
91
What are the two types of stem cell transplants?
Autologous | Allogenic
92
What needs to be given to mobilise the stem cells before they can be collected from the peripheral blood via leukapheresis?
G-CSF (granulocyte colony stimulating factor)
93
For which disorders is autologous stem cell transplant sometimes indicated?
``` Myeloma Mantle cell lymphoma T-cell non-Hodgkin lymphoma Multiple sclerosis Rheumatic disorders Solid cancers e.g. germ cell tumours ```
94
For which disorders is allogenic stem cell transplant sometimes indicated?
Acute leukaemia Chronic leukaemia (if refractory) Aplastic anaemia
95
Which organs tend to be involved in acute GvHD?
Skin, liver and GI tract
96
What is the definition of acute GVHD with respect to stem cell transplant?
Occurring within the first 100 days of stem cell transplant
97
Which organs tend to be involved in chronic GvHD?
Any tissue can be involved
98
What sort of rash is characteristic of GvHD?
A macropapular rash

Phase 2a Medicine (13 decks)

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