Brainscape's Knowledge GenomeTM


Top Flashcards (Certified)
All Flashcards

Phase 2 > Haematology > Flashcards

Haematology Flashcards

1
Q

What is lymphoma

A

Malignant proliferation of lymphocytes normally in the lymph nodes but also in the blood, spleen, liver and bone marrow

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

What are the 4 causes of lymphoma

A
  1. Primary immunodeficiency (Ataxia-teangiectasia, Wiscott-Aldrich syndrome)
  2. Secondary immunodeficiency (HIV, Transplant recipients)
  3. Infections (EBV, Helicobacter pylori, Human T-lymphocyte virus
  4. Autoimmune disorders
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

How is lymphoma diagnosed

A

Blood film and bone biopsy

Lymph node biopsy

Immunophenotyping

Cytogenetics (Karyotype, FISH, PCR)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

How do you stage lymphoma

A

Blood tests

CT scan of chest, abdo/pelvis

Bone marrow biopsy

PET scan

Ann Arbor staging system

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

What are the two types of lymphoma

A

Hodgkins

Non-hodgkins lymphoma

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

What are the different types of Non-hodgkins lymphoma

A

Low grade = follicular lymphoma

High grade = Diffuse B cell lymphoma

Severe grade = Burkitt’s Lymphoma

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

What is Hodgkins lymphoma

A

Malignant transformation of normal B/T cells in the lymph nodes

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

Hodgkins lymphoma is most common in which age groups

A

20-29 years

>60 years

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

Hodgkins lymphoma is associated with what virus

A

EBV - impaired immunosurveillnance of infected cells

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

What are the lymphadenopathy symptoms of Hodgkins lymphoma

A

Painless, asymmetric lymph nodes that spread contiguously to adjacent lymph nodes

Cervical, inguinal and axillary lymph nodes

Alcohol makes more painful

Mediastinal lymphadenopathy causes cough, SVC obstruction and bronchial obstruction

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

What are the B cell associated symptoms of Hodgkins lymphoma

A

B cell symptoms
Fever
Night sweats
Wt loss

Other symptoms
Pruritis
Hepato/splenomegaly
Pel Ebstein Fever

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

What are the investigations for someone with suspected Hodgkins lymphoma

A
  1. Bloods (FBC, Film, ESR)
    - lactate dehydrogenase will be elevated
  2. Lymph node biopsy = REED-STEENBERG CELLS

3, Stage using CT/MRI chest, abdomen and pelvis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

Describe the Ann Arbor system of staging lymphoma

A

Stage 1 = Single LN region
stage 2 = >2 nodal areas on same side of the diaphragm
Stage 3 = nodes spread on both sides of diaphragm
Stage 4 = Spread beyond nodes (Liver/bone marrow)

A = No constitutional B cell symptoms except itch

B = With constitutional B cell symptoms

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

What is the management for Hodgkins Lymphoma

A

Stage 1-2a = Short course chemo and radiotherapy

Stage 2a-4b = Cyclic chemo + radiotherapy

Bone marrow transplant for relapse

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

What chemotherapy drugs are used in Hodgkins lymphoma (remember ABVD)

A

Adriamycin
Bleomycin
Vinblastine
Decarbazine

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

What are the complications of Hodgkins lymphoma treatment

A 
Infertility 
Anthracylcines = Cardiomyopathy 
Bleomycin = lung damage 
Vinca Alkaloids= Peripheral neuropathy 
Second cancers 
Psychological issues
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
17
Q

Define non-hodgkins lymphoma

A

Any lymphoma that doesn’t involve reed-steenberg cells

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
18
Q

What age group does non-hodgkins lymphoma normally present in

A

Adults >40 years

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
19
Q

Name a low grade non Hodgkins lymphoma and describe its characteristics

A 
Follicular lymphoma 
 - slow growing 
 - usually advanced at presentation 
- Incurable 
Median survival = 9-11 years
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
20
Q

Name a high grade non Hodgkins lymphoma and describe its characteristics

A

Diffuse large B cell lymphoma

  • Usually nodal presentation
  • 1/3 cases have extra nodal involvement
  • patient often unwell with short history
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
21
Q

Name a severe grade Hodgkins lymphoma

A

Burkitts lymphoma

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
22
Q

What are the lymphadenopathy symptoms of non-hodgkins lymphoma

A

Painless, symmetric lymphadenopathy at multiple sites that spreads discontinuously

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
23
Q

What are the extra nodal symptoms of non-hodgkins lymphoma

A

Skin
CNS
Oropharynx and GIT
Splenomegaly

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
24
Q

What are the B symptoms of non-hodgkins lymphoma

A

Fever
Night sweats
Wt loss

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
25
What investigations would be conducted in someone with suspected non-hodgkins lymphoma
FBC, U and E and LDH Film - Normal or circulating lymphoma cells Pancytopenias with NO REED STEENBERG CELLS Staging using CT/MRI chest, abdomen and pelvis and Ann Arbor classification
26
Describe the management of high grade non-hodgkins lymphoma
``` R-CHOP regime Rituximab (Monoclonal antibody) Cyclophosphamide Hydro-doxorubicin Vincristine Prednisolone ``` Bone marrow transplant for relapse
27
What does rituximab target
CD20 on B cells
28
What is the management for low grade non-hodgkins lymphoma
``` Watch and wait is asymptomatic if symptomatic = Chemotherapy Radiotherapy Alkylating agents Monoclonal antibodies Bone marrow transplant ```
29
What is T cell engaging therapy
Blinatumomab | - Bispecific antibody that targets CD19 n B cells and CD3 on T cells
30
Describe the pathophysiology of lymphoma associated with EBV
There is impaired immunosurveillance and infected B cells escape regulation and proliferate
31
What is myeloma
Malignant disease of bone marrow plasma cells leading to progressive bone marrow infiltration and failure
32
Describe the epidemeology of myeloma
Peak age = 60-70yrs | Afro-caribbean
33
What is the pathogenesis of myeloma
Clonal proliferation of plasma cells --> Monoclonal Ig production (usually IgG or IgA) Clones produce light chains which are excreted in the kidney (Bence jones protein) Clones produce IL-6 which inhibits osteoblasts and activates osteoclasts
34
What are the symptoms of myeloma (Remember CRAB)
1. Calcium elevated (Increased osteoclast activity leading to hypercalcaemia) 2. Renal impairment (Caused by light chain deposition and increased calcium 3. Anaemia (Decreased bone marrow RBC production leading to normochromic normocytic anaemia) 4. Bone lesions (Increased osteoclast activity leading to lytic lesions = bone pain esp back Recurrent infections due to neutropenia and immunoparesis Hypercalcaemia and bone pain = pepper pot skull Bone marrow infiltration = anaemia, thrombocytopenia and neutropenia Amyloidosis
35
What investigations would be conducted in someone with suspected myeloma
FBC = normocytic normochromic anaemia Film = rouleaux formation, plasma cells and cytopenias Increased ESR, increased calcium Urinalysis and electrophoresis show Bence Jones protein PLAIN X-RAY = LYTIC PUNCHED OUT LESIONS (PEPPER POT SKULL AND OESTEOPOROSIS)
36
What is the management of myeloma
1. Supportive - Bone pain (Analgesics but not NSAIDS - Bisphosphates (Zolendronate to decrease fractures and bone pain) - Transfusions for anaemia - Hydration 3L/day for renal impairment - Broad spectrum antibiotics for infections 2. Chemotherapy (i) Cyclophosphamide, thalidomide, dexamethasone) (ii) Vincristine, adriamycin, dexamethasone)
37
In order to make a diagnosis of myeloma there must be evidence of mono-clonality, what is mono-clonality
Abnormal proliferation of plasma cells leading to immunoglobulin secretion and causing organ dysfunction especially to the kidney
38
What disease often precedes myeloma
Monoclonal gammopathy of undetermined significance (MGUS)
39
What is MGUS
A common disease with paraprotein present in the serum but no myeloma
40
In approx 2/3 people with myeloma, their urine might contain what?
Immunoglobulin light chains with kappa or lamda lineage
41
What would you expect to seen on the blood film of someone with myeloma
Rouleaux formation (Aggregation of RBCs)
42
What is the definition of leukaemia
Malignant proliferation of haemopoietic cells
43
What are the 4 types of leukaemia
1. Acute myeloid leukaemia 2. Acute lymphoblastic leukaemia 3. Chronic myeloid leukaemia 4. Chronic lymphocytic leukaemia
44
What are the risk factors for leukaemia
``` Congenital (Downs syndrome) Environmental - Radiotherapy -Chemotherapy - Benzene ```
45
What investigations would you do in someone with suspected leukaemia
``` Blood film Bone marrow biopsy Lymph node biopsy Immunotyping (Classifying tumour cells by antigen expression profile) Cytogenetics (Karyotyping, FISH, PCR) ```
46
What is the definition of acute lymphoblastic leukaemia
Acute malignant transformation of lymphoid progenitor cells
47
What age group is acute lymphoblastic leukaemia most common in
Most common cancer in children especially 2-5 years
48
What are the symptoms of ALL
Bone marrow failure leading to anaemia, thrombocytopenia and leukopenia Infiltration of tissue (Lymphadenopathy, CNS involvement, hepatosplenomegaly, bone pain)
49
What investigations would be conducted in someone with suspected ALL
``` Increased WCC Decreased RBC + Platelets BM aspirate = 20% leukaemia blast cells Cytogenetics = philadelphia chromosome CT and CXR for lymph node involvement ```
50
What is the management of ALL
1. Supportive (Blood products, antifungals, antibiotics) 2. Chemotherapy (Vincristine, dexamethasone and intrathecal methotrexate) 3. Bone marrow transplant best for younger patients
51
Define acute myeloid leukaemia
Acute malignant transformation of myeloid progenitor cells
52
What age range is AML most common in
Mean age 65-70 | but also 10-15% of childhood leukaemia
53
What is the aetiology of AML
Neoplastic proliferate of myeloblasts Preceding haematological disorder or prior chemotherapy Exposure to ionising radiation
54
What are the signs and symptoms of AML
1. Bone marrow failure - anaemia - infection (decreased WCC) - DIC 2. Infiltration - Gum infiltration - Hepatosplenomegaly - Skin involvement - Bone pain - Lymphadenopathy - Orchidomegaly
55
What investigations for AML
Blood film = anaemia and thrombocytopenia but increased WCC Bone marrow biopsy (>20% blast cells) Auer rods are diagnostic of AML over ALL) Lymph node biopsy Immunophenotyping Cytogenetics BM aspirate = leukaemia blast cells
56
What is the management of AML
Supportive care as for ALL Chemotherapy Bone marrow transplant
57
Define chronic myeloid leukaemia
Chronic malignant transformation of myeloid cells (Basophils, eosinophils, neutrophils)
58
What age group is CML most common in
Middle aged 40-60 years
59
What is the cause of chronic myeloid leukaemia
Myeloproliferative disorder with clonal proliferation of myeloid cells
60
What are the symptoms of CML
wt loss, fever, night sweats Massive hepatosplenomegaly Bruising and bleeding
61
What is the key diagnostic feature of CML
Philadelphia chromosome Reciprical translocation t(9,22) leading to formation of BCR-ABL fusion gene leading constitutive tyrosine kinase activity = increase cell growth
62
What investigations would you conduct in someone with suspected CML
Increased WBC Decreased Hb and platelets BM cryogenic analysis is Philadelphia +ve
63
What is the treatment of CML
Imatinib = tyrosine kinase inhibitor Allogenic Stem cell transplant
64
Define chronic lymphocytic lymphoma
Chronic malignant transformation of mature lymphoid cells
65
What is the aetiology of CLL
Clone of mature B cells | Mutation of 11q or 17p
66
What are the features of CLL
Symmetrical painless lymphadenopathy Hepatosplenomegaly Anaemia, infection, bleeding, BM failure Wt loss, fever, night sweats
67
What are the investigations in someone with CLL
Increased WCC Anaemia Immunophenotyping to distinguish it from NHL
68
What is the management of CLL
Supportive chemotherapy (Cyclophosphamide, fludarabine, rituximab) Radiotherapy
69
Define anaemia
Low Hb concentration due to a low red cell mass or increased plasma volume
70
How can you determine if bone marrow production of RBC's is the issue in anaemia
Look at reticulocyte number (Immature RBC's) - If high then removal the issue - If low then production the issue
71
What is the normal haemoglobin concentration for men and women
Male = 133-166g/L Female = 110-147g/L
72
What are the physiological changes seen in anaemia
Reduced HB = reduced O2 transport = hypoxia = compensatory mechanisms such as increased tissue perfusion, increased O2 transfer to tissues and increased RBC production
73
What are the pathological changes seen in anaemia
Heart and liver fat change Ischaemia Skin and Nail atrophy Aggravate angina
74
When might you see microcytic hypo chromic RBCs on blood film
Iron deficiency
75
When might you see polysegmented neutrophils with microcytic anaemia
B12 deficiency
76
What are the non-specific symptoms of anaemia
``` Fatigue Dyspnoea Headaches Faintness Anorexia Palpitations ```
77
What are the signs of anaemia
Conjunctival pallor Hyperdynamic circulation Palpitation Tachycardia
78
What organs are responsible for the removal of RBC's
1. Spleen 2. Liver 3. Bone marrow 4. Blood loss
79
How do we classify the different types of anaemia
By mean corpuscular volume which is the average volume of the RBC's (Their size)
80
What are the normal ranges for MCV for males and females
``` Male = 81.8 - 96.3fl Females = 80.0- 98.1fl ```
81
What are the three different classifications of anaemia
Microcytic Normocytic Macrocytic
82
What are the causes of microcytic anaemia
``` Iron deficiency Chronic disease (BM/kidney problems) Thalassaemia Sideroblastic anaemia Lead poisoning ```
83
What are the causes of normocytic anaemia
``` Acute blood loss Chronic disease Combined haematinic deficiency (Iron and B12 deficiency) BM failure Renal failure Pregnancy Hypothyroidism ```
84
What are the causes of macrocytic anaemia
``` B12 deficiency Folate deficiency Anti-folate drugs (Methotrexate and Phenytoin) Excess alcohol or liver disease Hypothyroidism Cytotoxics (Hydroxycarbamide) Reticulocytosis ```
85
What is the normal daily intake of iron
15-20mg
86
What are the specific signs of iron deficiency anaemia
Koilonychia (spoon shaped nails) Angular stomatitis (Mouth corners inflamed) Atrophic glossitis Plummer Vinson Brittle Nails and Hair
87
Describe the pathophysiology of iron deficiency anaemia
Lack of iron means no haem production leading to smaller RBCs and microcytic anaemia
88
What are the causes of iron deficiency anaemia
Blood loss - Menorrhagia - Hookworm - GI bleeding Increased demand - pregnancy Decreased intake - diet Malabsorption - Coeliac - Crohns
89
What are the investigations for someone with suspected iron deficiency anaemia
Blood film = Microcytic hypochromic anaemia, low reticulocyte ``` Low ferritin Low Fe Low Hb Low MCV Increased TIBC Ansiocytosis, poikliocytosis, pencil cells Upper and lower GI endoscopy ```
90
How do you manage someone with iron deficiency anaemia
Oral iron = ferrous sulphate
91
What are the side effects of ferrous sulphate
``` Nausea Abdominal discomfort Diarrhoea Constipation Black stools ```
92
What is the pathophysiology of B12 deficiency
Lack of B12/intrinsic factor means B12 is not absorbed in the terminal ileum leading to big fragile RBC due to impairment of DNA synthesis
93
What are the causes of vitamin B12 deficiency
Decreased intake - vegan Decreased intrinsic factor - Pernicious anaemia - Post-gastrectomy Terminal ileum - Crohn's - Illeal resection
94
What are the specific signs of B12 deficiency
``` Glossitis Paraesthesia Optic Atrophy Peripheral neuropathy Symptoms of anaemia Lemon tinge due to pallor and mild jaundice Subacute Cord Degeneration ```
95
What are the investigations for someone with suspected B12 deficiency
``` Decreased WCC Intrinsic factor antibodies Parietal cell antibodies Coeliac Abs Schilling test ```
96
What is the management for someone with B12 deficiency
B12 replacement with hydroxocobalamin
97
What is pernicious anaemia
Autoimmune atrophic gastritis causes by auto antibodies against parietal cells or intrinsic factor
98
What are the causes of folate deficiency
Decreased intake - Poor diet Increased demand - Pregnancy - Malignancy Malabsorption - Coeliac - Crohn's Drugs - EtOH - phenytoin - Methotrexate
99
What are haemoglobinopathies
Disorders of quality = abnormal molecule or variant haemoglobin ie. sickle cell disease Disorders of quantity = reduced production ie. alpha or beta thalasaaemia
100
What is the structures of normal haemoglobin
2 alpha and 2 beta subunits
101
What is the structure of foetal haemoglobin
2 alpha and 2 gamme subunits
102
What is the inheritance pattern in sickle cell anaemia
Autosomal recessive
103
Where is sickle cell disease most prevalent
Africa, caribbean and ME
104
What is the pathogenesis of sickle cell disease
Point mutation in the B-globin gene causing glutamic acid to valine AA change which causes HbA to be changed to HbS
105
What is the HbS globin chain problematic
HbS is insoluble when deoxygenated = sickling Sickle cells have decreased life span = haemolysis Sickle cells get trapped in microvascular = thrombosis
106
When do the clinical features of sickle cell disease begin to show
Manifest from 3-6 months due to decreased foetal Hb
107
What can be a trigger for the manifestation of sickle cell disease
Infection Cold Hypoxia Dehydration
108
What is the presentation of sickle cell anaemia | Remember sickled
``` Splenomegaly Infarction Crises (pulmonary) Kidney disease liver and lung disease Erection Dactylitis ```
109
What is thalassaemia
Point mutations or deletions leading to diminished synthesis of one or more globin chains leading to unmatched haemoglobin molecules which damages the RBC membrane causing their destruction
110
Where is thalassaemia most common
Mediterranean | Far East
111
What is the classification of beta thalassaemia
1. Thalassaemia major - Transfusion dependent 2. Thalassaemia intermedia - Less severe anaemia but can survive without regular blood transfusions 3. Thalassaemia carrier/heterozygote - Asymptomatic
112
What is the clinical presentation of thalassaemia major
Age of presentation at 6-12 months Clinical presentation is severe anaemia symptoms with failure to thrive, jaundice and extra medullary erythropoiesis including frontal bossing, maxillary overgrowth and HSM
113
What is the treatment of thalassaemia major
``` Regular transfusion Iron chelation Endocrine supplementation Bone health Psychological support ```
114
What do you need to monitor in thalassaemia major
``` Ferritin Cardiac and liver MRI Endocrine testing Gonadal function Diabetes screening Growth and puberty Vit D, Calcium and PTH Thyroid Dexa Screening ```
115
What are the complications of blood transfusions for thalassaemia
Iron overload as there is not way to eliminate the excessive iron leading to developing haemosiderosis
116
Describe the inheritance pattern of membranopathies
Autosomal dominant
117
Name 2 common membranopathies
Spherocytosis | Elliptocytosis
118
Describe the physiology of membranopaties
Deficiency of red cell protein (Spectrin) caused by a variety of genetic lesions which causes RBC to become spherical and rigid so gets stuck in the spleen leading to extravascular haemolysis
119
What do membranopathies predispose you to?
Gall stones
120
What is the treatment for membranopathies
Folic acid and splenectomy
121
What is the significance of parvovirus and haemolytic anaemia
Parvovirus is a common infection in children and can lead to a dramatic drop in Hb in patients who already have a reduced cell lifespan
122
What are enzymopathies
Inherited enzyme deficiencies that lead to shortened red cell lifespan from oxidative kinase
123
Name 2 common enzymopathies
G6PD deficiency | Pyruvate kinase deficiency
124
Where is G6PDD most common
Mediterranean and Mid/Far East
125
How do you diagnose G6PD
Screening for NADPH G6PD assay with reticulocytes showing increased G6PD Look on blood fillm for Heinz bodies and bite cells
126
What are the symptoms of G6PD
Mainly asymptomatic but crises are characterised by haemolysis Jaundice Anaemia
127
What things might precipitate G6PD
``` Broad beans Infection Drugs - Primaquine Sulphonamides Nitrofurantoin Quinolones Dapsone ```
128
Define polycythaemia
Too many RBCs
129
What are the two types of polycythaemia
Primary = polycythaemia rubra vera Secondary = Smoking, Lung/heart disease, excess EPO, altitude
130
Describe the pathophysiology of primary polycythaemia
Bone marrow overactivity leading to increased RBC, WBC and Plt caused by JACK2 mutation
131
What are the signs of polycythaemia
Hyperviscosity = headaches, dizziness, visual disturbances, thrombosis Pruritis after hot bath (Aquagenic pruritus) Erythromelagia Signs - red face and hepatosplenomegaly
132
What investigations for polycythaemia
FBC - High RBC, WCC and Plt BM is hyper cellular and erythroid JAK2 is 99% +ve
133
What is the management of polycythaemia
Venesection in young patients or hydroxycarbamide (BM suppression in older patients) Aspirin
134
What is the complication of polycythaemia
Can progress to acute myeloid leukaemia
135
Where are platelets produced and what are they produced from
In the bone marrow from megakaryocyte fragments
136
Which hormone regulates platelet production
Thrombopoietin
137
What is the lifespan of platelets
7-10 days
138
What is the normal platelet count
150-400x10^9/L
139
How are platelets removed from the circulation
By the spleen
140
What surface proteins are found on a platelet
ABO HPA HLA class I Glycoproteins
141
What causes a platelet to become activated
Adhesion to collagen via GPIa Adhesions to VWF by GPIb and IIb/IIIa
142
What does platelet activation lead to
Release of alpha granules containing PDGF, Fibrinogen, VWF and PF4 Release of dense granules containing nucleotides (ADP), Ca2+ and serotonin Membrane phospholipids acitivate clotting factors II, V and X Change in shape to spiculated shape
143
Describe the process of platelet activation in primary haemostasis
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.
144
What tests can you carry out to determine platelet function
FBC for number Blood film for appearance Platelet function analysis for function FLow cytometry for surface proteins
145
What problems can cause bleeding
1. Injury/trauma 2. Vascular disorders (Abnormality of collagen = Ehlers Danlos Syndrome) 3. Low platelets (Thrombocytopenia) 4. Abnormal platelet function 5. Defective coagulation (Haemophilia)
146
What clinical features would you seen in someone with platelet dysfunction
``` Mucosal bleeding - Epistaxis - Gum bleeding - Menorrhagia Easy bruising Petechiae Purpura Traumatic haemorrhage (Inc subdural) ```
147
What are the causes of low platelets
1. Production failure - Congenital (Thrombocytopenia) - Acquired (Drugs, BM suppression, Marrow failure, BM replacement) 2. Increased removal (immune, consumption (Bleeding), Splenomegaly) 3. Artefactual (EDTA induced clumping)
148
What are the causes of impaired platelet function
1. Congenital (Platelet storage disorders = - Glanzmann (GpIIb/IIIa deficiency) - Bernard Soulier (GPIb deficiency) - Von Willebrand Disease (Lack of VWF which binds platelets to damaged endothelium) 2. Acquired - uraemia - Drugs (Aspirin, clopidogrel, NSAIDs)
149
Glanzmann diseases is a deficiency in what
GPIIb/IIIa fibrinogen receptor
150
Bernard soupier disease is a deficiency in what
Reduction in GP1b/ VW receptor
151
What are the two main causes of thrombocytopenia
1. Decreased platelet production | 2. Increased platelet destruction
152
What are the causes of decreased platelet production
1. Congenital thrombocytopenia 2. Infiltration of BM - Leukaemia - Metastatic malignancy - Lymphoma - Myeloma 3. Reduced platelet production by BM - Low B12 - Reduced TPO - Medication (Methotrexate, chemo - Toxins - Infection (HIV, TB) 4. Dysfunctional production of platelets in BM - Myelodysplasia
153
What are the causes of increased platelet destruction
1. Autoimmune thrombocytopenia 2. Hypersplenism 3. Drug related immune destruction (Heparin) 4. Consumption of platelets - DIC - Thrombotic thrombocytopenc purport - Haemolytic uraemia syndrome - Haemolysis - Major haemorrhage
154
How does clopidogrel affect platelet functions
Binds P2Y12 inhibitor
155
How does aspirin affect platelet function
Irreversible inhibitor of COX1
156
How does Tirofiban affect platelet function
Inhibits GP IIb/IIIa
157
What is immune thrombocytopenia
IgG antibodies form to platelet and megakaryocyte surface glycoproteins
158
What is the treatment for immune thrombocytopenia
Immunosuppressants Treat underlying cause Tranexamic acid (inhibits fibrin breakdown)
159
Describe the pathophysiology of disseminated intravascular coagulation
Cytokine release in response to systemic inflammatory response syndrome leads to systemic activation of the clotting cascade which... 1. Leads to microvascular thrombosis and organ failure 2. Consumption of platelets and clotting factors leading to bleeding
160
What is the treatment for DIC
Treat underlying cause Supportive provision of - platelets - FFP
161
What is thrombotic thrombocytopenia purpura
Spontaneous platelet aggregation in microvasculature
162
What is the aetiology of thrombocytopenia purpura
Reduction in protease enzyme ADAMTS13 which leads to failure to break down high molecular weight VWF multimers leading to massive blood clots capable of causing infarcts
163
1. What is the characteristic genetic abnormality in chronic myeloid leukaemia? (i) ARTA Gene (ii) Philadelphia chromosome (iii) AML/ETO gene (iv) cMYC oncogene
Philadelphia chromosome
164
2. What class of drug best describes rituximab? (i) Cytotoxic chemotherapy (ii) Disease modifying therapy (iii) Monoclonal antibody (iv) Antibiotic
Monoclonal Antibody
165
3. Which age group is characteristically affected by Hodgkins lymphoma? (i) Children (ii) Teenagers and young adults (iii) Middle aged (40-60 years) (iv) Older age (>60 years)
Teenagers and young adults | Older age >60 years
166
4. How is myeloma bone disease usually assessed? (i) Plain X-ray (ii) Clinical assessment (iii) Isotope bone scan (iv) PET scan
Plain X-ray
167
5. What is the correct mechanism of action for anti-emtic drug ondanseteron (i) Peripheral D2 antagonist (ii) Central D2 antagonist (iii) Anti-cholinergic (iv) 5-HT3 antagonist
5-HT3 antagonist
168
6. What is the commonest cause of microcytic anaemia? (i) B12 deficiency (ii) Iron deficiency (iii) Haematologic malignancy (iv) Hereditary spherocytosis
Iron deficiency
169
7. In sickle cell anaemia what would you expect to see the reticulocyte count? (i) Absent (ii) Low (iii) Normal (iv) Raised
Raised
170
8. Bacterial infections usually causes? (i) Low lymphocytes (ii) Low neutrophils (iii) High lymphocytes (iv) High neutrophils
High neutrophils
171
9. Which best outlines the approach to the management of a patient with suspected febrile neutropenia (i) Encourage fluids and paracetamol (ii) Perform cultures and wait for results before antibiotics (iii) Perform cultures and start oral antibiotics (iv) Perform culrures and start broad spectrum IV antibiotics - Use tanzosin and gentamicin
(iv) Perform culrures and start broad spectrum IV antibiotics - Use tanzosin and gentamicin
172
10. How does aspirin exert is antiplatelet effect? (i) ADP receptor antagonist (ii) Inhibition of cyclooxygenase (iii) Inhibition of glycoprotein IIb-IIIa (iv) Inhibition of PAR4 receptor
Inhibition of cycle-oxygenase
173
What is polycythaemia
Too many red blood cells
174
What are the primary causes of polycythaemia
Polycythaemia rubra vera - over-reactive bone marrow
175
What are the reactive/secondary causes of polycythaemia
``` Smoking Lung disease Cyanotic heart disease Altitude EPO and androgen excess ```
176
What is polycythaemia rubra vera
Myeloproliferative disorder resulting in bone marrow overactivity resulting in predominantly increased WBC's and platelets
177
What mutation causes polycythaemia rubra vera in 95% of cases
Mutation in JAK2
178
What is the clinical presentation of polycythaemia rubra vera
``` Plethoric appearance Thrombosis Itching Splenomegaly Abnormal FBC ```
179
What is the treatment for polycythaemia rubra vera
Aspirin Venesection Bone marrow suppressive drugs (Hydroxycarbamide) If secondary then treat the underlying cause
180
Define neutrophilia
Too many neutrophils
181
What are the primary causes of neutrophilia
Chronic myeloid leukaemia
182
What are the reactive causes of neutrophilia
Bacterial Infection Malignancy Inflammation
183
What is lymphocytosis
Too many white blood cells mainly lymphocytes
184
What are the primary causes of lymphocytosis
Chronic lymphoid leukaemia
185
What are the reactive causes of lymphocytosis
Viral Infection Inflammation Malignancy
186
Define thrombocytopenia
Not enough platelets
187
Define thrombocytosis
Too many platelets
188
What are the primary causes of thrombocytosis
Essential thrombocythaemia
189
What are the reactive causes of thromocytosis
Infection Inflammation Malignancy
190
Define Neutropaenia
not enough neutrophils
191
Define normal neutrophil levels
1.7-6.5
192
Define mild neutropaenia
1-1.7
193
Define moderate neutropaeia
0.5-1.0
194
Define a severe neutropenia
<0.5
195
What are the causes of neutropenia
1. Underproduction - marrow failure - marrow infiltration - Marrow toxicity ie drugs 2. Increased removal - Autoimmune - Felty's syndrome - cyclical
196
What hormone is responsible for regulating RBC production?
Erythropoietin.
197
What stimulates EPO?
Tissue hypoxia.
198
Give 2 causes of thrombocytopenia.
1. Production failure e.g. marrow suppression, marrow failure. 2. Increased removal e.g. immune response (ITP), consumption (DIC), splenomegaly.
199
Where are platelets produced?
In the bone marrow. They are fragments of megakaryocytes.
200
What is the definition of febrile neutropenia.
Temperature >38°C in a patient with neutrophil count <1x10^9/L.
201
Give 4 risk factors for febrile neutropenia.
1. If the patient had chemotherapy <6 weeks ago. 2. Any patient who has had a stem cell transplant <1 year ago. 3. Any haematological condition causing neutropenia. 4. Bone marrow infiltration.
202
What is the presentation of febrile neutropenia?
1. Pyrexia, 38°C. 2. Generally unwell. 3. Confusion. 4. Hypotensive. 5. Tachycardic.
203
Describe the management of febrile neutropenia.
1. Thorough history and examination. 2. Bloods. 3. Antibiotics within 1 hour!
204
Give a risk factor for spinal cord compression.
Any malignancy that can cause compression e.g. bone metastasis.
205
Describe the presentation of spinal cord compression.
1. Back pain. 2. Weakness in legs. 3. Inability to control bladder. 4. Spastic paresis. 5. Sensory level.
206
Describe the management of spinal cord compression.
1. Bed rest. 2. High dose steroids. 3. Analgesia. 4. Urgent MRI of the whole spine.
207
What is tumour lysis syndrome?
Break down of malignant cells -> content release -> metabolic disturbances; can cause hyperuricaemia, hyperkalaemia, hypocalcaemia.
208
Give 3 risk factors for tumour lysis syndrome.
1. High tumour burden. 2. Pre-existing renal failure. 3. Increasing age.
209
Describe the treatment of tumour lysis syndrome.
1. Aggressive hydration. 2. Monitor electrolytes. 3. Drugs to reduce uric acid production e.g. allopurinol.
210
What is hyperviscosity syndrome?
Increase in blood viscosity usually due to high levels of immunoglobulins.
211
Give 2 consequences of hyperviscosity syndrome
1. Vascular stasis. | 2. Hypoperfusion.
212
Describe the presentation of hyperviscosity syndrome.
1. Mucosal bleeding. 2. Visual change. 3. Neurological disturbances. 4. Breathlessness. 5. Fatigue.
213
What investigations might you do in someone who you suspect has hyperviscosity syndrome?
1. FBC and blood film; look for rouleaux formation. 2. U&E. 3. Immunoglobulins.
214
What is the treatment for hyperviscosity syndrome?
1. Keep hydrated! 2. Avoid blood transfusion. 3. Treat the underlying cause.
215
What does rituximab target?
Targets CD20 on the surface of B.
216
What is the characteristic genetic abnormality in chronic myeloid leukaemia?
t(9; 22) - philadelphia chromosome.
217
How is myeloma bone disease usually assessed?
X-ray.
218
What is the affect of sickle cell anaemia on reticulocyte count?
Reticulocyte count is raised.
219
Why is reticulocyte count raised in sickle cell anaemia?
Sickle cell disease is haemolytic, there is increased degradation of RBC's. Production therefore increases in order to keep up with degradation and so reticulocyte count is raised.
220
What clotting factors depend on vitamin K?
2, 7, 9 and 10.
221
Haemophilia A is due to deficiency of what clotting factor?
Factor 8 deficiency
222
Haemophilia B is due to deficiency of what clotting factor?
Factor 9 deficiency.
223
What kind of anaemia could methotrexate cause?
Macrocytic due to folate deficiency.
224
Give 4 causes of folate deficiency.
1. Dietary. 2. Malabsorption. 3. Increased requirement e.g. in pregnancy. 4. Folate antagonists e.g. methotrexate.
225
Give 3 signs of haemolytic anaemia.
1. Pallor. 2. Jaundice. 3. Splenomegaly.
226
Give 3 signs of haemolytic anaemia.
1. Pallor. 2. Jaundice. 3. Splenomegaly.
227
Give 4 causes of haemolytic anaemia.
1. GP6D deficiency. 2. Sickle cell anaemia. 3. Spherocytosis/elliptocytosis (membranopathies). 4. Autoimmune haemolytic anaemia.
228
Give 3 things that can cause coagulation disorders.
1. Vitamin K deficiency. 2. Liver disease. 3. Congenital e.g. haemophilia.
229
How does warfarin work?
It antagonises vitamin K and so you get a reduction in clotting factors 2, 7, 9 and 10.
230
How does heparin work?
It activates antithrombin which then inhibits thrombin and factor Xa.
231
What is disseminated intravascular coagulation (DIC)?
Pathological activation of the coagulation cascade -> fibrin in vessel walls. There is platelet (thrombocytopenia) and coagulation factor consumption.
232
Give 3 causes of disseminated intravascular coagulation (DIC).
1. Sepsis. 2. Major trauma. 3. Malignancy.
233
What is the affect on TT, PTT and APTT in someone with disseminated intravascular coagulation (DIC)?
All increased
234
What is the affect on fibrinogen in someone with disseminated intravascular coagulation (DIC)?
Decreased
235
Give 5 risk factors for DVT.
1. Increasing age. 2. Obesity. 3. Pregnancy. 4. OCP (hyper-coagulability). 5. Major surgery. 6. Immobility. 7. Past DVT.
236
Give 3 symptoms of DVT.
Unilateral warm, tender, painful, swollen leg.
237
What forms the differential diagnosis for a DVT?
Cellulitis.
238
What investigations might you do in someone to see if they have a DVT?
1. D-dimer in those patients with a low clinical probability. 2. US compression.
239
What is the name of the score used to determine someones probability of having a DVT?
The Wells score.
240
The Wells score determines someones clinical probability of having a DVT. Give 3 factors the score takes into account.
1. Active cancer. 2. Recently bedridden or major surgery. 3. Tenderness along deep venous system. 4. Swollen leg/calf. 5. Unilateral pitting oedema.
241
Describe the management for a DVT.
Aim of management is to prevent a PE! | - Anticoagulants e.g. warfarin/heparin.
242
Explain why philadelphia chromosome causes CML.
Philadelphia chromosome leads to a fusion gene that has tyrosine kinase activity and enhanced phosphorylating activity -> altered cell growth.
243
Where would you normally take a bone marrow biopsy from?
Posterior iliac crest.
244
What is the most important medical treatment for DVT prophylaxis?
LMWH.
245
What is the affect of iron deficiency anaemia on iron binding capacity?
Iron binding capacity will be raised.
246
Why might measuring serum ferritin be inaccurate for looking at iron levels?
Ferritin is an acute phase protein and so its concentration will increase in response to inflammation.
247
Describe the treatment for iron deficiency anaemia.
Ferrous sulphate tablets.
248
What is aplastic anaemia?
When bone marrow stem cells are damaged -> pancytopenia.
249
Why does sickle cell anaemia not present until after 6 months of age?
HbF is not affected by sickle cell anaemia as it is made up of 2 alpha and 2 gamma chains.
250
What drug can be used to prevent painful crises in people with sickle cell anaemia?
Hydroxycarbamide.
251
Give 3 clinical features of a patient with a membranopathy.
1. Jaundice. 2. Anaemia. 3. Splenomegaly.
252
Why does a deficiency in glucose-6-phosphate dehydrogenase lead to shortened red cell lifespan?
G6PD protects cells against oxidative damage.
253
What 3 blood test values would be increased in someone with polycythaemia?
1. Hb. 2. RCC. 3. PCV.
254
Give 3 symptoms of polycythaemia.
1. Itching. 2. Headache. 3. Dizziness. 4. Visual disturbance.

Phase 2 (16 decks)

Brainscape helps you reach your goals faster, through stronger study habits.
© 2025 Bold Learning Solutions. Terms and Conditions