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Emily Phase 2a > Haematology > Flashcards

Haematology Flashcards

1
Q

What is myeloma?

A

A blood cancer arising from plasma cells in the bone marrow.
- accumulation of malignant plasma cells in the bone marrow leading to progressive bone marrow failure

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2
Q

What protein is a myeloma associated with?

A

Paraprotein
- In myeloma, plasma cells produce a large amount of a single type of antibody, paraprotein, that has no useful function

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3
Q

What complications can myelomas cause?

A
  • kidney failure
  • destructive bone disease and hypercalcaemia
    (stimulate osteoclasts and inhibits osteoblasts - pathological fracture)

CRAB

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4
Q

What are the key characteristics of a myeloma?

A

Spikey old CRAB
C - elevated Calcium >0.25mmol/l
R - renal impairment
A - anaemia
B - lytic Bone lesion, osteoporosis

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5
Q

What is the epidemiology of myelomas?

A
  • 2nd MC haematological cancer
  • median age at 70 yrs
  • survival ranges from a few months to 20 yrs
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6
Q

What are the classifications of myelomas?

A
  1. Monoclonal gammopathy of undetermined significance = no evidence of impairment
  2. Smouldering myeloma = more than 10% plasma cells in bone marrow, but no related organ impairment (doesn’t have symptoms)
  3. Symptomatic myeloma = evidence of related organ/tissue impairment
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7
Q

What is systemic AL amyloidosis?

A

Build up of an abnormal protein (amyloid) in tissues
‘L’ = light chains that form amyloid proteins

(amyloid protein is broken down very slowly by the body and so builds up in tissues/organs, gradually damaging them)

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8
Q

What is the treatment for AL amyloidosis?

A

Not curative so aims to kill the abnormal plasma cells that produce the amyloid protein and prevent new deposits forming

  1. high dose therapy
  2. stem cell transplantation
    (go into remission but then will relapse)
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9
Q

How do patients present with amyloidosis?

A

Presents with
- nephrotic syndrome
- +/- renal impairment (33%), congestive
- cardiomyopathy (20%),
- sensorimotor
- and/or autonomic neuropathy (20%)
- often with GI disturbance
- and hepatomegaly; rarely, bleeding and Raccoon eyes and macroglossia

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10
Q

What are the initial investigations for patients with myelomas?

A
  1. FBC
  2. Serum + urinary electrophoresis
  3. imaging = whole body MRI
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11
Q

What is serum electrophoresis?

A
  • helps identify presence of abnormal proteins
    by looking at charge of molecules
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12
Q

What is the process of measuring the monoclone?

A
  1. Electrophoresis - If a discrete additional band seen or bands fuzzy or suppressed, proceed to immunofixation
  2. Immunofixation repeats the electrophoresis plus identifies monoclones via the addition of specific antibodies. A second immunofixation is sometimes required if a rare band expected eg IgD or IgE; performed at diagnosis and at other key intervals eg relapse
  3. Densitometry scan performed on 1. or 2. to quantify the monoclone
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13
Q

What are the complications of myeloma bone disease (MBD)?

A

Cause abnormalities in bone leading to:
- disability = walking impairment, kyphosis, permanent deformity and paralysis
- bone pain
- decreased quality of life
- increased hospital visits

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14
Q

What is the main treatment for myeloma diseases?

A

Bisphosphonates
e.g. clodronate, zoledronic acid or pamidronate

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15
Q

What secondary treatments are used to treat myelomas?

A

Bisphosphonate to protect bones
Blood transfusions/EPO injections
Antibiotics as needed
Pain-killers as required
Radiotherapy
Kyphoplasty occasionally indicated (inject cement to improve bone strength)
Psychological suppor

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16
Q

What are the different types of anti-myeloma chemotherapies?

A
  1. Monoclonal antibodies – daratumumab (anti CD38)
  2. Proteasome inhibitors – bortezomib (sc), carfilzomib (iv), ixazomib (po)
  3. Immunomodulatory drugs – thalidomide and analogues lenalidomide (Revlimid) and pomalidomide
  4. Corticosteroids – dexamethasone, prednisolone
  5. Alkylating agents – cyclophosphamide, melphalan
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17
Q

What are the 2 types of stem cell transplantation?

A
  1. allogenic = stem cell donation
  2. autologous = stem cells from your own bone marrow
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18
Q

What is the process of stem cell transplantation?

A
  1. Stem cell mobilisation
  2. stem cell collection
  3. stem cell transplant
  4. high-dose melphalan (chemo drug)
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19
Q

What are the 3 things that can be wrong in RBCs to cause haemoglobinopathies?

A
  1. membrane
  2. haemoglobin
  3. enzymes
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20
Q

What is the structure of foetal haemoglobin?

A

2 alpha and 2 gamma chains

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21
Q

What is the structure of adult haemoglobin?

A

2 alpha and 2 beta chains

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22
Q

What is sickle cell disease?

A

Abnormal Hb
- single point mutation in beta globin gene, HbS
- autosomal recessive

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23
Q

What is the pathophysiology of sickle cell disease?

A

HbS polymerises when deoxygenated (form rigid sickle shape)
- blocks blood vessels > ischaemia, sequestration
- chronic haemolysis > pulmonary hypertension, leg ulcers etc.

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24
Q

What is the epidemiology of sickle cell disease?

A

Black Asian prevalence
- sickle cell allele gives increased protection against malaria

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25
Q

What is the effect on HbS carrier?

A
  • healthy
  • avoid severe hypoxia (as can then become sickled)
  • inheritance risk
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26
Q

What are the investigation tests for sickle cell?

A
  • Sickle solubility test (positive result could also be a carrier)
  • Hb separation = capillary/ gel electrophoresis, isoelectric focusing
  • Antenatal: molecular genetics
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27
Q

What are the complications of Sickle cell disease?

A
  • Acute painful crisis (cut off blood supply to organ/tissue)
  • stroke
  • acute chest syndrome
  • sequestration = blood pools in liver/spleen
  • gallstones
  • priapism = painful prolonged erection
  • leg ulcers
  • retinal bleed
  • cardiac failure
  • liver/kidney failure
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28
Q

What is acute chest syndrome?

A

lung damage (chest infection, post op, covid etc.) -> hypoxia -> HbS polymerisation -> reduced blood flow -> lung damage worsens

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29
Q

How do you prevent sickle cells disease complications?

A
  • supportive = stay warm, vaccinations
  • regular blood transfusion
  • hydroxycarbamide = increase HbF
  • new drugs = crizanlizumab, voxelotor
  • donor bone marrow transplant = only curative option
  • genetic therapies
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30
Q

Does sickle cell disease affect newborns?

A

No its only when they try to switch from foetal to adult haemoglobin

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31
Q

What is thalassaemia?

A

abnormality of alpha or beta global gene in Hb
- autosomal recessive
- carriers = hypothermic, microcytic (blood count may look like Fe deficiency)
- imbalance of alpha and beta is bad

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32
Q

What is the treatment and complications for thalassemia?

A

transfusion only curative option
- can cause iron overload and chelation

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33
Q

What is lymphoproliferative disease?

A
  • neoplastic, clonal proliferation of lymphoid cells
    (cancer of WBCs)
  • typically affects lymph nodes
  • can be extranodal (bone marrow/liver/spleen/anywhere)
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34
Q

What are the types of lymphomas?

A
  1. Hodgkin lymphoma
  2. Non-Hodgkin lymphoma = aggressive or indolent
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35
Q

What is the difference between Hodgkin and non-Hodgkin lymphomas?

A

Hodgkin
- B lymphocytes characterised by the Reed-Sternberg cell
- progresses slower
- progresses in a orderly way from lymph node region to the next

Non-Hodgkin’s
- B or T Lymphocytes or Natural Killer cells
- less predictable in their course
- less likely to have systemic ‘B” symptoms at diagnosis

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36
Q

What is an indolent lymphoma?

A
  • slow growing and advanced at presentation
  • generally incurable
  • many different sub-types
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37
Q

What cells can cause lymphomas?

A
  1. B cells 90% = opened up when differentiating so most likely to have wrong gene inserted
  2. T cells 10%
  3. NK-cell (natural killer cell) <1%
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38
Q

What is the aetiology/risk factors of indolent lymphomas?

A

Mostly cause is unknown

Risk Factors include:
1. Primary Immunodeficiency e.g. Wiscott-Aldrich Syndrome; Common Variable Immunodeficiency
2. Secondary Immunodeficiency e.g. HIV; Recipients of Transplant
3. Infection e.g. EBV; HTLV-1; Helicobacter Pylori
4. Autoimmune Disorders (e.g. on LT treatment for rheumatoid arthritis)

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39
Q

What is the B symptom triad for lymphoma diagnosis?

A
  1. Fever
  2. Night sweats
  3. weight loss
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40
Q

What is the clinical presentation for indolent lymphomas?

A

Majority of Indolent Lymphomas present with painless lymphadenopathy (lump)

Association with B-Symptoms – Fevers, Night Sweats and Weight Loss

Bone Marrow involvement – ?Leukaemic component

Autoimmune Phenomena

Compression Syndromes

Organ Involvement e.g. Skin = rash

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41
Q

What are the investigations for indolent lymphomas?

A
  1. Lymph node biopsy = core needle biopsy/excision node biopsy (can’t do fine needle biopsy as just gets liquid out)
  2. bone marrow biopsy
  3. PET-CT scan
  4. bloods
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42
Q

What is the treatment for indolent lymphomas?

A
  1. Watch + Wait = asymptomatic, follow up regularly, no compromise in survival
  2. Radiotherapy = local control, can be curative for stage 1/2 follicular lymphomas
  3. Chemoimmunotherapy +/- maintenance
  4. small molecules inhibitors/ Novel therapies
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43
Q

What are myelodysplastic syndromes?

A

Bone marrow abnormalities
- bone marrow cells fail to make adequate numbers of healthy blood cells (both quantity + quality)
- abnormal cells crowd out remaining normal cells

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44
Q

What is acute myeloid leukaemia?

A

heterogenous clonal malignancy characterised by:
- immature myeloid cell proliferation
AND
- bone marrow failure (abnormal cells crowd out normal ones)

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45
Q

What are the laboratory features of MDS?

A
  1. low blood counts (of RBCs, WBCs, platelets)
  2. peripheral blood film demonstrates dysplastic features (e.g. hypogranular neutrophils)

(MDS = don’t have enough healthy blood cells)

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46
Q

What are auer rods?

A

crystallised aggregates of the myeloperoxidase enzyme
(seen in AML blood film)

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47
Q

What is the symptoms for low RBC count?

A
  • fatigue
  • SOB
  • Lightheadeness
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48
Q

What are the symptoms for low WBC count?

A
  • increased risk of frequent and/or severe infections
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49
Q

What are the symptoms of low platelet count?

A

Bleeding/bruising

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50
Q

What are the laboratory features of AML?

A
  1. WBC count can be low, normal or high
  2. RBCs and platelet are usually low
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51
Q

What are some key indications a patient needs to be referred to a haematologist?

A
  • there are blasts on peripheral blood
  • deterioration in FBC parameters is very rapid
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52
Q

What investigations are done for MDS and AML diagnosis?

A
  1. blood tests = full blood count + blood film
  2. bone marrow aspirate and trephine biopsy
  3. morphology (appearance of cells on slides)
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53
Q

What other conditions can present with dysplasia?

A

B12/ folate or mixed haematinic deficiency
Infection (e.g. retroviral disease, herpesvirus)
Medications
Autoimmune
Liver disease (e.g. cirrhosis)

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54
Q

What hormone regulates RBC production?

A

Erythropoietin

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55
Q

What is polycythaemia vera?

A

a myeloproliferative disorder characterised by neoplasia of mature myeloid cells, in particular those involved in the red cell lineage, within the bone marrow.

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56
Q

What are the 3 requirements for a diagnosis of polycythemia vera?

A
  1. Hb >16.5
  2. Bone marrow tai-lineage proliferation with pleomorphic mature megakaryocytes
  3. Presence of JAK2 mutation
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57
Q

What is the treatment for polycythaemia?

A
  1. aspirin 75mg daily
  2. Venesection on 2 occasions
  3. Hydroxycarbamide 500mg daily
  4. Aim to keep haematocrit <0.45
  5. annual cardiovascular risk assessment
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58
Q

What investigations should you do for someone with a high platelet count?

A

Erythrocyte Sedimentation Rate (ESR) = rate at which RBCs in anticoagulated whole blood descend in a standardised tube over a period of one hour (measure of inflammation if RBCs settle relatively quickly )

C-reactive protein (CRP) = level of CPR increases when there’s inflammation

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59
Q

What is thrombocytosis?

A

Excess production of platelets

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60
Q

What would you see on the blood count in a splenectomy?

A

High WBC
High platelet
High Jolly bodies (RBC with nucleus as no spleen to remove deranged RBCs)

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61
Q

What would you see on the blood count in anaemia?

A

Pencil cells
small red blood cells

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62
Q

What is JAK2 positive essential thrombocythaemia?

A

A rare chronic blood cancer characterised by the overproduction of platelets by megakaryocytes in the bone marrow.

High Platelet count
presence of a JAK2 mutation

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63
Q

What is the treatment for thrombocythemia?

A
  • aspirin
  • hydroxycarbamide
    (aim to keep platelet count between 150-400)
  • treat cardiovascular risk factors = smoking, obesity, diabetes, hypertension, high cholesterol
64
Q

What are the 3 main symptoms of an enlarged spleen?

A
  1. fever
  2. night sweats
  3. weight loss
65
Q

What are the causes of an enlarged spleen?

A

Infection (MC) =
liver disease = liver damage increases intrahepatic pressures, increasing pressure in hepatic vein

rheumatoid arthritis
SLE systemic lupus erythematous
Cancer (once all other things are excluded)

66
Q

What is shown on a leucoerythroblastic blood film?

A

Nucleated RBCs
(proliferation of immature RBCs, erythroblasts)

erythroblast –> reticulocyte –> erythrocyte

67
Q

What are tear drop poikilocytes and what do they signify?

A

Oval, teardrop shaped RBCs
Myelofibrosis

68
Q

What is myelofibrosis?

A

Scar tissue builds up inside the bone marrow and RBCs are not made properly

69
Q

What is the treatment of myelofibrosis?

A
  • started on Ruxolitinib (JAK2 inhibitor)
  • erythropoietin injections
  • analgesia for splenic discomfort
  • blood transfusion support if required
70
Q

What causes infectious mononucleosis?

A

aka Glandular fever
Epstein-Barr virus (EBV)

71
Q

What is chronic myeloid leukaemia?

A

involves the proliferation of partially mature myeloid cells, in particular granulocytes, within the bone marrow and blood.

72
Q

What is the treatment for CML?

A

Baseline quantitative PCR for BCR::ABL1
Start Tyrosine Kinase inhibitor (Imatinib 400mg daily)
Monitor FBC for haematological remission
Reassess spleen size at 3 months
Monitor BCR::ABL1 every 3 months

73
Q

What are the main causes of high white blood cell count?

A

(in order of MC)
1. Infection
2. medication
3. tissue ischemia
4. paraneoplastic
5. haematological

74
Q

What is the lifecycle of a RBC?

A
  1. made in bone marrow
  2. live for 120days
  3. destroyed in the spleen
  4. Haem is broken down into bilirubin
  5. iron is recycled
75
Q

What are the 2 main causes of reduced RBC count?

A

Decreased production:
- iron deficiency
- folate deficiency
- B12 deficiency
- bone marrow failure

Increased Loss:
- BLEEDING
- Haemolysis

76
Q

What are normal Hb levels?

A

Female normal = 110-147g/l
Male normal = 131-166g/l

77
Q

What is anaemia?

A

Haemoglobin below lower limit of normal

78
Q

What are the 3 types of MCV?

A

MCV : mean cell volume (cell size)

Macrocytic = cells larger than normal
Microcytic = cell smaller than normal
Normocytic = normal size

79
Q

What conditions have microcytic anaemia?

A

TAILS

  • Thalassaemias
    (hypochromic, pale cells, less Hb)
  • Anemia of chronic disease
  • Iron deficiency
  • Lead poisoning
  • Sideroblastic anaemia
80
Q

What anaemias have normocytic RBCs?

A

3 As and H
Low reticulocyte count (bone marrow producing less)
- Aplastic anemia
- anemia of chronic disease

High reticulocyte count (being destroyed so bone marrow producing more)
- acute blood loss
- haemolytic = hereditary sherocytosis, G6PD deficiency, sickle cell anemia, autoimmune haemolytic anemia

81
Q

Which anaemias have macrocytic RBCs?

A

Megaloblastic:
- folate deficiency
- B12 deficiency

Non-megaloblastic:
- alcohol
- reticulocytosis
- liver disease
- drugs
- hypothyroidism

82
Q

What conditions other than anaemia can cause macrocytosis?

A
  • reticulocytosis
    -raised immunoglobins
  • hypothyroidism
  • alcohol
83
Q

What is the Coombes test?

A

Shows if antibodies are stuck to RBCs to see if someone has an autoimmune disease destroying the RBCs

84
Q

Where is iron absorbed?

A

Duodenum and upper jejunum

85
Q

How does pregnancy affect iron deficiencies?

A

pregnancy can unmask iron deficiency
- if before on the lower end of iron, now giving a proportionate of that to the foetus so become fully deficient

86
Q

What is ferritin? And what can ferritin levels tell you?

A

Helps to store iron in cells
- tells you how much iron is the body and if you have iron deficiency

87
Q

What does an iron study tell you?

A

Transferrin saturation = transports iron around the body so how much transferrin has iron bound to it (low Fe -> causes increased production of transferrin)

High total iron binding capacity = more trandferrin free to have iron bind to it

88
Q

What results would you get from an iron study in iron deficiency?

A

Low transferrin saturation
High total iron binding capacity (has higher capacity to bound as less iron is bound to it)

89
Q

How much folate do you need a day? and where is it absorbed?

A

0.1-0.2 mg (can’t be stored)
absorbed in proximal jejunum

90
Q

What are the causes of folate deficiency?

A
  • poor nutrition (found in green veg, nuts)
  • malabsorption = coeliac, crohns, pregnancy, haemolysis
91
Q

What is folate needed for?

A

needed for DNA replication

92
Q

Why should B12 levels be checked when treating folate deficiency?

A

if give more folate causes bone marrow to make more RBCs causing B12 to become even lower
- so treat B12 deficiency first

93
Q

What is B12 required for?

A
  • DNA synthesis
  • fatty acid synthesis
94
Q

Where is B12 absorbed?

A

terminal ileum - must bind to intrinsic factor produced by gastric parietal cells

95
Q

What are the causes of B12 deficiency?

A
  • pernicious anemia (MC cause)= autoimmune gastric atrophy, loss of intrinsic factor production
  • gastrectomy/ileal resection
  • vegan diet
  • bacterial overgrowth
  • oral contraceptive
  • hypochloridia
  • nitric oxide
96
Q

How do you test for B12 deficiency?

A
  • intrinsic factor antibodies
97
Q

How do you treat B12 deficiency?

A
  • intramuscular replacement = initially frequent, then maintenance
98
Q

What are the 2 types of hameolysis?

A

Compensated haemolysis = increased destruction matched by increased synthesis

Decompensated haemolysis = rate of destruction exceeds rate of synthesis, causing anemia

99
Q

What is haemolysis?

A

Reduction in red cell lifespan due to increased red blood cells destruction

100
Q

What are the different bleeding disorders?

A

haemophilia
Von Willebrands disease
Idiopathic thrombocytopaenic purpura
Functional platelet disorders
Disseminated intravascular coagulation

101
Q

What is haemophilia A?

A

X-linked recessive disorder causing mutation that leads to increased bleeding and bruising
- deficiency in clotting factor VIII

102
Q

How is haemophilia categorised?

A

Severe = <1% frequent spontaneous bleeds, joint deformity
Moderate = 1-5% occasional spontaneous bleeds, post traumatic bleeding
Mild = 5-20% post traumatic bleeding

103
Q

What is Von Willebrand’s disease?

A
  • autosomal bleeding disorder causing mucocutaneous bleeding
104
Q

Where is von Willebrand factor synthesised and stored?

A

Synthesised in endothelial cells and megakaryocytes
Stored in Weibel-Palade bodies and alpha granules in platelets

105
Q

What is the role of von Willebrand factor?

A
  1. circulating vWF adheres to damaged, exposed sub endothelium
  2. undergoes a structural modification
  3. now spontaneously binds to circulating platelets and initiates platelet adhesion
  4. FVIII is released and binds to activated platelets to serve a primary regulator of coagulation
  5. FVIII (anti-haemophilic factor) bound to vWF is protected form activated protein C destruction
106
Q

What are the types of von Willebrand’s disease?

A

Type 1 = MC, partial quantitative deficiency (AD), variable phenotype
Type 2 = qualitative deficiency
Type 3 = rare, Complete quantitative deficiency (AR), concordant extreme reductions

107
Q

What are the features of Type 2A vWD?

A

vWF can attach to collagen and factor VIII, but unable to bind platelets

108
Q

What are the features of Type 2B vWD?

A

vWF binds to platelets in the bloodstream, without injury. These platelets are cleared by the liver and spleen causing thrombocytopenia

109
Q

What are the features of Type 2N vWD?

A

vWF binds to collagen and platelets, but not to factor VIII

110
Q

When are the steps in a FVIII binding assay test?

A

1.Coat a micro-titre plate with human anti vWF
2. Add test samples including positive control, negative control and samples to be tested
3. Cleave off endogenous factor VIII
4. Add exogenous rFVIII
5. Perform a chromogenic VIII assay to see how much rVIII has bound

111
Q

What are the results of a FVIII binding assay test?

A

Normal binding – normal
Reduced binding – heterozygous for 2N
Absent binding – homozygous for 2N

112
Q

What are the features of Type 2M vWD?

A

vWF can attach to collagen and factor VIII, but unable to bind platelets

113
Q

Explain the role of platelets in endothelial damage?

A

1) Platelets adhere to vascular endothelium via collagen & vWF (von Willebrand factor)

2) Binding of platelets to collagen stimulates cytoskeleton shape change within the platelets, and they ‘spread’ out

3) This increases their surface area and results in their activation, leading to the release of platelet granule contents including ADP, fibrinogen, thrombin and calcium. These components facilitate the clotting cascade ending with the production of fibrin.

4) Aggregation of platelets then occurs, which involves the cross-linking of activated platelets by fibrin

5) Activated platelets also provide a negatively charged phospholipid surface, which allows coagulation factors to bind and enhance the clotting cascade.

114
Q

What are platelets activated by?

A

Adhesion to collagen via GPIa,
Adhesion to vWF via GPIb and IIb/IIIa

115
Q

What are the platelet surface proteins?

A

ABO
HPA
HLA Class I (not class II)
Glycoproteins e.g. GP1a

116
Q

What are the clinical features of platelet dysfunction?

A

Mucosal bleeding
- Epistaxis, gum bleeding, menorrhagia
Easy bruising
Petechiae, purpura
Traumatic haematomas (inc subdural)

117
Q

What are the causes of low platelet count?

A

Production failure
- congenital
- acquired = drugs, marrow suppression/failure/replacement

Increased removal
- immune
- consumption
- splenomegaly

Artefactual
- EDTA induced clumping

118
Q

What are the causes of impaired platelet function?

A
  1. congenital
    - platelet disorders
    - vWD
  2. Acquired
    - uraemia
    - drugs
119
Q

What are the 4 types of platelet function disorders?

A
  1. Adhesion = Bernard Soulier Syndrome
  2. Aggregation = Glanzmann’s thrombasthenia
  3. Secretion = Hermansky-Pudlak syndrome, Chedial Higashi syndrome (storage pool disorders)
  4. Disorders of the platelet procoagulant surface = Scott syndrome
120
Q

What are the features of Glanzmann’s thrombasthenia?

A
  • platelet aggregation defect
  • autosomal recessive
121
Q

What are the features of Bernard Soulier syndrome?

A
  • Platelet adhesion defect
  • autosomal recessive
  • mild thrombocytopenia and big platelets
122
Q

What is disseminated intravascular coagulation?

A

a serious disorder occurring in response to an illness or disease process which results in dysregulated blood clotting.
- both bleeding and thrombosis simultaneously

123
Q

What is the pathophysiology of DIC?

A
  1. Trigger = sepsis, trauma, pancreatitis, obstetric emergency, malignancy
  2. cytokine release in response to systemic inflammatory response syndrome
  3. systemic activation of clotting cascade
  4. microvascular thrombosis –> organ failure
    AND
    consumption of platelets + clotting factors –> bleeding
124
Q

What is the treatment for lymphomas?

A
  • immunochemotherapy
  • radiotherapy
  • allograft
  • check point inhibitors
  • BITE
  • CAR-T/NK
125
Q

What is the function of TNF-a?

A
  • promotes RBC degradation in macrophages via phagocytosis
  • reduces RBC production in the bone marrow
126
Q

What is the function of IF-y

A

IF-Y increases the expression of a protein channel called divalent metal transporter one on the surface of macrophages.
This channel serves as a pathway for iron to enter the macrophage at increased rates, so less iron is available for the production of haemoglobin.

127
Q

What is the function of IL-10?

A

IL-10 mediates the expression of increased ferritin receptors on the surface of macrophages, which then sequesters even more iron.

128
Q

What is the function of IL-6?

A

IL-6 also works in the liver by increasing production of a molecule called hepcidin, which blocks further uptake of iron from the small intestine.

129
Q

What is the age mnemonic for leukaemia?

A

You can use the mnemonic “ALL CeLL mates have CoMmon AMbitions” to remember the progressive ages of the different leukaemia from 45-75 in steps of 10 years.

  • Under 5 and over 45 –acutelymphoblasticleukaemia (ALL)
  • Over 55 –chroniclymphocyticleukaemia (CeLL mates)
  • Over 65 –chronicmyeloid leukaemia (CoMmon)
  • Over 75 –acutemyeloid leukaemia (AMbitions)
130
Q

What is the difference between lymphomas and leukaemia ?

A

Lymphoma differs from leukaemia in that neoplastic cells predominantly involve the lymph nodes and extranodal sites, unlike leukaemia which predominantly involves the bone marrow and blood.

131
Q

How are myelomas different to leukaemia and lymphomas?

A

Myeloma differs from leukaemias and lymphomas as the malignant cell in myeloma is the plasma cell, rather than the lymphoblast.

132
Q

What is tumour lysis syndrome?

A

A group of metabolic abnormalities that can occur as a complication from the treatment of cancer, where large amounts of tumour cells are killed off from the treatment and releasing their contents into the blood stream

133
Q

How does tumour lysis syndrome affect your body?

A
  • hyperuricemia (high uric acid = kidney dysfunction)
  • hyperphosphatemia = affect kidney function
  • hyperkalaemia = heart attack, nausea, vomiting, diarrhoea
  • uremia = kidney stops filtering toxins from your blood (bone muscle, heart and blood vessel issues)
  • hypocalcemia = changes to heart rhythm, muscle cramps, confusion, numbness, cardiac arrest
134
Q

What is APTT?

A

Activated partial thromboplastin time test
- measure of the functionality of the INTRINSIC and common pathways of the coagulation cascade
(want it to be shorter)

135
Q

What is the difference between thrombocytopenia, thrombophilia, thrombocytosis and thrombocythemia?

A

Thrombocytopenia = low platelet count
Thrombophilia = blood has increases tendency to clot
Thrombocytosis = too many platelets
Thrombocythemia = too many platelets

136
Q

How does hydroxycarbamide work and what conditions is it used for?

A

Chemotherapy drug = These drugs stop cells making and repairing DNA.

It is a treatment for:

chronic myeloid leukaemia (CML)
polycythaemia vera (PCV)
essential thrombocythemia (ET)
acute myeloid leukaemia (AML)
cervical cancer (cancer of the neck of the womb)

137
Q

What cells appear as smudge cells on a blood film?

A

B- cell lymphocytes
- fragile and break apart, leaving cell remnants without a clear membrane

138
Q

What antigen do mature B-cell lymphocytes express?

A

CD20 antigen
- helps indicate chronic lymphocytic leukaemia

139
Q

What are the main genes that cause inherited thrombophilia?

A
  • Factor V leiden (MC)
  • Prothrombin gene
  • antithrombin III deficiency
  • Protein S deficiency
140
Q

What blood condition can COPD be responsible for causing?

A

Polycythaemia
(high Hb due to chronic hypoxia)

141
Q

Which condition causes enlarged lymph nodes to become more painful when drinking alcohol?

A

Hodgkin’s lymphoma

142
Q

In which condition do schistocytes appear on the blood film?

A

Disseminated Intravascular coagulopathy

(excess fibrin strands (increased fibrinolysis) cause mechanical damage to the RBCs causing them to form schistocytes)

143
Q

What are Heinz bodies?

A
  • derivations of Hb
  • phagocytosed and have a ‘bite’ taken out of them
144
Q

What are the key presentations of uncomplicated malaria?

A

Warm and cold/ fever
Recent travel

145
Q

In which condition is a Rouleaux formation seen?

A

Multiple Myeloma

146
Q

Which leukaemia is most likely in children?

A

ALL

147
Q

Explain the pathophysiology of thrombotic thrombocytopenic purpura.

A
  1. deficiency in ADAMTS13 enzyme
  2. causes vWF to form thromboses
  3. to compensate, the body will break down these clots to prevent ischaemic damage to organs
  4. haemolytic anaemia = causes raised bilirubin levels
148
Q

What is the pathophysiology of Immune thrombocytopenic purpura?

A
  1. it affects platelet aggregation
  2. antibodies attach the GpIIB/IIIa receptors preventing platelet aggregation
149
Q

How does hypothyroidism cause anemia?

A
  • slows down the bone marrow
150
Q

Why does the sclera go yellow first in jaundice?

A

Contains elastin that has a high affinity for bilirubin

151
Q

Difference between thrombocytosis and thrombophilia?

A

Thrombocytosis = too many platelets
Thrombophilia = increased tendency to produce clots

152
Q

Difference between leukaemia, lymphoma and myeloma?

A

Leukaemia = start in bone
Lymphoma = start in lymph
Myeloma = start with plasma cells

153
Q

What causes a raised WCC?

A

Reactive = infection, inflammation, steroids
Persistent = chronic infection, leukaemia

154
Q

What is the difference between extravascular and intravascular haemolysis?

A

Intravascular hemolysis
- occurs when erythrocytes are destroyed in the blood vessel itself
e.g. G6Dp deficiency

extravascular hemolysis
- occurs in the hepatic and splenic macrophages within the reticuloendothelial system.
e.g. Sickle cell anaemia

155
Q

What are the different treatments for Hodgkin’s and Non-Hodgkin’s lymphoma?

A

Hodgkin’s
ABVD = Adriamycin, Bleomycin, Vincistine, Dacarbazine

Non-Hodgkin’s
R-CHOP = Rituximab (mAB), Cyclophosphamide, Hydroxydaunorubicin, Vincistine (Oncovin), Prednisolone

Emily Phase 2a (15 decks)

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