Haematology Flashcards
(155 cards)
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
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
3
Q
What complications can myelomas cause?
A
- kidney failure
- destructive bone disease and hypercalcaemia
(stimulate osteoclasts and inhibits osteoblasts - pathological fracture)
CRAB
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
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
6
Q
What are the classifications of myelomas?
A
- Monoclonal gammopathy of undetermined significance = no evidence of impairment
- Smouldering myeloma = more than 10% plasma cells in bone marrow, but no related organ impairment (doesn’t have symptoms)
- Symptomatic myeloma = evidence of related organ/tissue impairment
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)
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
- high dose therapy
- stem cell transplantation
(go into remission but then will relapse)
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
10
Q
What are the initial investigations for patients with myelomas?
A
- FBC
- Serum + urinary electrophoresis
- imaging = whole body MRI
11
Q
What is serum electrophoresis?
A
- helps identify presence of abnormal proteins
by looking at charge of molecules
12
Q
What is the process of measuring the monoclone?
A
- Electrophoresis - If a discrete additional band seen or bands fuzzy or suppressed, proceed to immunofixation
- 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
- Densitometry scan performed on 1. or 2. to quantify the monoclone
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
14
Q
What is the main treatment for myeloma diseases?
A
Bisphosphonates
e.g. clodronate, zoledronic acid or pamidronate
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
16
Q
What are the different types of anti-myeloma chemotherapies?
A
- Monoclonal antibodies – daratumumab (anti CD38)
- Proteasome inhibitors – bortezomib (sc), carfilzomib (iv), ixazomib (po)
- Immunomodulatory drugs – thalidomide and analogues lenalidomide (Revlimid) and pomalidomide
- Corticosteroids – dexamethasone, prednisolone
- Alkylating agents – cyclophosphamide, melphalan
17
Q
What are the 2 types of stem cell transplantation?
A
- allogenic = stem cell donation
- autologous = stem cells from your own bone marrow
18
Q
What is the process of stem cell transplantation?
A
- Stem cell mobilisation
- stem cell collection
- stem cell transplant
- high-dose melphalan (chemo drug)
19
Q
What are the 3 things that can be wrong in RBCs to cause haemoglobinopathies?
A
- membrane
- haemoglobin
- enzymes
20
Q
What is the structure of foetal haemoglobin?
A
2 alpha and 2 gamma chains
21
Q
What is the structure of adult haemoglobin?
A
2 alpha and 2 beta chains
22
Q
What is sickle cell disease?
A
Abnormal Hb
- single point mutation in beta globin gene, HbS
- autosomal recessive
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.
24
Q
What is the epidemiology of sickle cell disease?
A
Black Asian prevalence
- sickle cell allele gives increased protection against malaria
25
What is the effect on HbS carrier?
- healthy
- avoid severe hypoxia (as can then become sickled)
- inheritance risk
26
What are the investigation tests for sickle cell?
- Sickle solubility test (positive result could also be a carrier)
- Hb separation = capillary/ gel electrophoresis, isoelectric focusing
- Antenatal: molecular genetics
27
What are the complications of Sickle cell disease?
- 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
28
What is acute chest syndrome?
lung damage (chest infection, post op, covid etc.) -> hypoxia -> HbS polymerisation -> reduced blood flow -> lung damage worsens
29
How do you prevent sickle cells disease complications?
- supportive = stay warm, vaccinations
- regular blood transfusion
- hydroxycarbamide = increase HbF
- new drugs = crizanlizumab, voxelotor
- donor bone marrow transplant = only curative option
- genetic therapies
30
Does sickle cell disease affect newborns?
No its only when they try to switch from foetal to adult haemoglobin
31
What is thalassaemia?
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
32
What is the treatment and complications for thalassemia?
transfusion only curative option
- can cause iron overload and chelation
33
What is lymphoproliferative disease?
- neoplastic, clonal proliferation of lymphoid cells
(cancer of WBCs)
- typically affects lymph nodes
- can be extranodal (bone marrow/liver/spleen/anywhere)
34
What are the types of lymphomas?
1. Hodgkin lymphoma
2. Non-Hodgkin lymphoma = aggressive or indolent
35
What is the difference between Hodgkin and non-Hodgkin lymphomas?
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
36
What is an indolent lymphoma?
- slow growing and advanced at presentation
- generally incurable
- many different sub-types
37
What cells can cause lymphomas?
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%
38
What is the aetiology/risk factors of indolent lymphomas?
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)
39
What is the B symptom triad for lymphoma diagnosis?
1. Fever
2. Night sweats
3. weight loss
40
What is the clinical presentation for indolent lymphomas?
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
41
What are the investigations for indolent lymphomas?
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
42
What is the treatment for indolent lymphomas?
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
43
What are myelodysplastic syndromes?
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
44
What is acute myeloid leukaemia?
heterogenous clonal malignancy characterised by:
- immature myeloid cell proliferation
AND
- bone marrow failure (abnormal cells crowd out normal ones)
45
What are the laboratory features of MDS?
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)
46
What are auer rods?
crystallised aggregates of the myeloperoxidase enzyme
(seen in AML blood film)
47
What is the symptoms for low RBC count?
- fatigue
- SOB
- Lightheadeness
48
What are the symptoms for low WBC count?
- increased risk of frequent and/or severe infections
49
What are the symptoms of low platelet count?
Bleeding/bruising
50
What are the laboratory features of AML?
1. WBC count can be low, normal or high
2. RBCs and platelet are usually low
51
What are some key indications a patient needs to be referred to a haematologist?
- there are blasts on peripheral blood
- deterioration in FBC parameters is very rapid
52
What investigations are done for MDS and AML diagnosis?
1. blood tests = full blood count + blood film
2. bone marrow aspirate and trephine biopsy
3. morphology (appearance of cells on slides)
53
What other conditions can present with dysplasia?
B12/ folate or mixed haematinic deficiency
Infection (e.g. retroviral disease, herpesvirus)
Medications
Autoimmune
Liver disease (e.g. cirrhosis)
54
What hormone regulates RBC production?
Erythropoietin
55
What is polycythaemia vera?
a myeloproliferative disorder characterised by neoplasia of mature myeloid cells, in particular those involved in the red cell lineage, within the bone marrow.
56
What are the 3 requirements for a diagnosis of polycythemia vera?
1. Hb >16.5
2. Bone marrow tai-lineage proliferation with pleomorphic mature megakaryocytes
3. Presence of JAK2 mutation
57
What is the treatment for polycythaemia?
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
58
What investigations should you do for someone with a high platelet count?
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
59
What is thrombocytosis?
Excess production of platelets
60
What would you see on the blood count in a splenectomy?
High WBC
High platelet
High Jolly bodies (RBC with nucleus as no spleen to remove deranged RBCs)
61
What would you see on the blood count in anaemia?
Pencil cells
small red blood cells
62
What is JAK2 positive essential thrombocythaemia?
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
63
What is the treatment for thrombocythemia?
- aspirin
- hydroxycarbamide
(aim to keep platelet count between 150-400)
- treat cardiovascular risk factors = smoking, obesity, diabetes, hypertension, high cholesterol
64
What are the 3 main symptoms of an enlarged spleen?
1. fever
2. night sweats
3. weight loss
65
What are the causes of an enlarged spleen?
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
What is shown on a leucoerythroblastic blood film?
Nucleated RBCs
(proliferation of immature RBCs, erythroblasts)
erythroblast --> reticulocyte --> erythrocyte
67
What are tear drop poikilocytes and what do they signify?
Oval, teardrop shaped RBCs
Myelofibrosis
68
What is myelofibrosis?
Scar tissue builds up inside the bone marrow and RBCs are not made properly
69
What is the treatment of myelofibrosis?
- started on Ruxolitinib (JAK2 inhibitor)
- erythropoietin injections
- analgesia for splenic discomfort
- blood transfusion support if required
70
What causes infectious mononucleosis?
aka Glandular fever
Epstein-Barr virus (EBV)
71
What is chronic myeloid leukaemia?
involves the proliferation of partially mature myeloid cells, in particular granulocytes, within the bone marrow and blood.
72
What is the treatment for CML?
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
What are the main causes of high white blood cell count?
(in order of MC)
1. Infection
2. medication
3. tissue ischemia
4. paraneoplastic
5. haematological
74
What is the lifecycle of a RBC?
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
What are the 2 main causes of reduced RBC count?
Decreased production:
- iron deficiency
- folate deficiency
- B12 deficiency
- bone marrow failure
Increased Loss:
- BLEEDING
- Haemolysis
76
What are normal Hb levels?
Female normal = 110-147g/l
Male normal = 131-166g/l
77
What is anaemia?
Haemoglobin below lower limit of normal
78
What are the 3 types of MCV?
MCV : mean cell volume (cell size)
Macrocytic = cells larger than normal
Microcytic = cell smaller than normal
Normocytic = normal size
79
What conditions have microcytic anaemia?
TAILS
- Thalassaemias
(hypochromic, pale cells, less Hb)
- Anemia of chronic disease
- Iron deficiency
- Lead poisoning
- Sideroblastic anaemia
80
What anaemias have normocytic RBCs?
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
Which anaemias have macrocytic RBCs?
Megaloblastic:
- folate deficiency
- B12 deficiency
Non-megaloblastic:
- alcohol
- reticulocytosis
- liver disease
- drugs
- hypothyroidism
82
What conditions other than anaemia can cause macrocytosis?
- reticulocytosis
-raised immunoglobins
- hypothyroidism
- alcohol
83
What is the Coombes test?
Shows if antibodies are stuck to RBCs to see if someone has an autoimmune disease destroying the RBCs
84
Where is iron absorbed?
Duodenum and upper jejunum
85
How does pregnancy affect iron deficiencies?
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
What is ferritin? And what can ferritin levels tell you?
Helps to store iron in cells
- tells you how much iron is the body and if you have iron deficiency
87
What does an iron study tell you?
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
What results would you get from an iron study in iron deficiency?
Low transferrin saturation
High total iron binding capacity (has higher capacity to bound as less iron is bound to it)
89
How much folate do you need a day? and where is it absorbed?
0.1-0.2 mg (can't be stored)
absorbed in proximal jejunum
90
What are the causes of folate deficiency?
- poor nutrition (found in green veg, nuts)
- malabsorption = coeliac, crohns, pregnancy, haemolysis
91
What is folate needed for?
needed for DNA replication
92
Why should B12 levels be checked when treating folate deficiency?
if give more folate causes bone marrow to make more RBCs causing B12 to become even lower
- so treat B12 deficiency first
93
What is B12 required for?
- DNA synthesis
- fatty acid synthesis
94
Where is B12 absorbed?
terminal ileum - must bind to intrinsic factor produced by gastric parietal cells
95
What are the causes of B12 deficiency?
- 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
How do you test for B12 deficiency?
- intrinsic factor antibodies
97
How do you treat B12 deficiency?
- intramuscular replacement = initially frequent, then maintenance
98
What are the 2 types of hameolysis?
Compensated haemolysis = increased destruction matched by increased synthesis
Decompensated haemolysis = rate of destruction exceeds rate of synthesis, causing anemia
99
What is haemolysis?
Reduction in red cell lifespan due to increased red blood cells destruction
100
What are the different bleeding disorders?
haemophilia
Von Willebrands disease
Idiopathic thrombocytopaenic purpura
Functional platelet disorders
Disseminated intravascular coagulation
101
What is haemophilia A?
X-linked recessive disorder causing mutation that leads to increased bleeding and bruising
- deficiency in clotting factor VIII
102
How is haemophilia categorised?
Severe = <1% frequent spontaneous bleeds, joint deformity
Moderate = 1-5% occasional spontaneous bleeds, post traumatic bleeding
Mild = 5-20% post traumatic bleeding
103
What is Von Willebrand's disease?
- autosomal bleeding disorder causing mucocutaneous bleeding
104
Where is von Willebrand factor synthesised and stored?
Synthesised in endothelial cells and megakaryocytes
Stored in Weibel-Palade bodies and alpha granules in platelets
105
What is the role of von Willebrand factor?
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
What are the types of von Willebrand's disease?
Type 1 = MC, partial quantitative deficiency (AD), variable phenotype
Type 2 = qualitative deficiency
Type 3 = rare, Complete quantitative deficiency (AR), concordant extreme reductions
107
What are the features of Type 2A vWD?
vWF can attach to collagen and factor VIII, but unable to bind platelets
108
What are the features of Type 2B vWD?
vWF binds to platelets in the bloodstream, without injury. These platelets are cleared by the liver and spleen causing thrombocytopenia
109
What are the features of Type 2N vWD?
vWF binds to collagen and platelets, but not to factor VIII
110
When are the steps in a FVIII binding assay test?
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
What are the results of a FVIII binding assay test?
Normal binding – normal
Reduced binding – heterozygous for 2N
Absent binding – homozygous for 2N
112
What are the features of Type 2M vWD?
vWF can attach to collagen and factor VIII, but unable to bind platelets
113
Explain the role of platelets in endothelial damage?
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
What are platelets activated by?
Adhesion to collagen via GPIa,
Adhesion to vWF via GPIb and IIb/IIIa
115
What are the platelet surface proteins?
ABO
HPA
HLA Class I (not class II)
Glycoproteins e.g. GP1a
116
What are the clinical features of platelet dysfunction?
Mucosal bleeding
- Epistaxis, gum bleeding, menorrhagia
Easy bruising
Petechiae, purpura
Traumatic haematomas (inc subdural)
117
What are the causes of low platelet count?
Production failure
- congenital
- acquired = drugs, marrow suppression/failure/replacement
Increased removal
- immune
- consumption
- splenomegaly
Artefactual
- EDTA induced clumping
118
What are the causes of impaired platelet function?
1. congenital
- platelet disorders
- vWD
2. Acquired
- uraemia
- drugs
119
What are the 4 types of platelet function disorders?
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
What are the features of Glanzmann's thrombasthenia?
- platelet aggregation defect
- autosomal recessive
121
What are the features of Bernard Soulier syndrome?
- Platelet adhesion defect
- autosomal recessive
- mild thrombocytopenia and big platelets
122
What is disseminated intravascular coagulation?
a serious disorder occurring in response to an illness or disease process which results in dysregulated blood clotting.
- both bleeding and thrombosis simultaneously
123
What is the pathophysiology of DIC?
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
What is the treatment for lymphomas?
- immunochemotherapy
- radiotherapy
- allograft
- check point inhibitors
- BITE
- CAR-T/NK
125
What is the function of TNF-a?
- promotes RBC degradation in macrophages via phagocytosis
- reduces RBC production in the bone marrow
126
What is the function of IF-y
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
What is the function of IL-10?
IL-10 mediates the expression of increased ferritin receptors on the surface of macrophages, which then sequesters even more iron.
128
What is the function of IL-6?
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
What is the age mnemonic for leukaemia?
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 – acute lymphoblastic leukaemia (ALL)
- Over 55 – chronic lymphocytic leukaemia (CeLL mates)
- Over 65 – chronic myeloid leukaemia (CoMmon)
- Over 75 – acute myeloid leukaemia (AMbitions)
130
What is the difference between lymphomas and leukaemia ?
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
How are myelomas different to leukaemia and lymphomas?
Myeloma differs from leukaemias and lymphomas as the malignant cell in myeloma is the plasma cell, rather than the lymphoblast.
132
What is tumour lysis syndrome?
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
How does tumour lysis syndrome affect your body?
- 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
What is APTT?
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
What is the difference between thrombocytopenia, thrombophilia, thrombocytosis and thrombocythemia?
Thrombocytopenia = low platelet count
Thrombophilia = blood has increases tendency to clot
Thrombocytosis = too many platelets
Thrombocythemia = too many platelets
136
How does hydroxycarbamide work and what conditions is it used for?
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
What cells appear as smudge cells on a blood film?
B- cell lymphocytes
- fragile and break apart, leaving cell remnants without a clear membrane
138
What antigen do mature B-cell lymphocytes express?
CD20 antigen
- helps indicate chronic lymphocytic leukaemia
139
What are the main genes that cause inherited thrombophilia?
- Factor V leiden (MC)
- Prothrombin gene
- antithrombin III deficiency
- Protein S deficiency
140
What blood condition can COPD be responsible for causing?
Polycythaemia
(high Hb due to chronic hypoxia)
141
Which condition causes enlarged lymph nodes to become more painful when drinking alcohol?
Hodgkin's lymphoma
142
In which condition do schistocytes appear on the blood film?
Disseminated Intravascular coagulopathy
(excess fibrin strands (increased fibrinolysis) cause mechanical damage to the RBCs causing them to form schistocytes)
143
What are Heinz bodies?
- derivations of Hb
- phagocytosed and have a 'bite' taken out of them
144
What are the key presentations of uncomplicated malaria?
Warm and cold/ fever
Recent travel
145
In which condition is a Rouleaux formation seen?
Multiple Myeloma
146
Which leukaemia is most likely in children?
ALL
147
Explain the pathophysiology of thrombotic thrombocytopenic purpura.
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
What is the pathophysiology of Immune thrombocytopenic purpura?
1. it affects platelet aggregation
2. antibodies attach the GpIIB/IIIa receptors preventing platelet aggregation
149
How does hypothyroidism cause anemia?
- slows down the bone marrow
150
Why does the sclera go yellow first in jaundice?
Contains elastin that has a high affinity for bilirubin
151
Difference between thrombocytosis and thrombophilia?
Thrombocytosis = too many platelets
Thrombophilia = increased tendency to produce clots
152
Difference between leukaemia, lymphoma and myeloma?
Leukaemia = start in bone
Lymphoma = start in lymph
Myeloma = start with plasma cells
153
What causes a raised WCC?
Reactive = infection, inflammation, steroids
Persistent = chronic infection, leukaemia
154
What is the difference between extravascular and intravascular haemolysis?
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
What are the different treatments for Hodgkin's and Non-Hodgkin's lymphoma?
Hodgkin's
ABVD = Adriamycin, Bleomycin, Vincistine, Dacarbazine
Non-Hodgkin's
R-CHOP = Rituximab (mAB), Cyclophosphamide, Hydroxydaunorubicin, Vincistine (Oncovin), Prednisolone
