Haematology Flashcards

1
Q

What is DVT?

A

A blood clot that develops within a deep vein in the body, usually the leg.

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2
Q

What is the differential diagnosis of DVT?

A

Cellulitis

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3
Q

What score determines the possibility of having DVT?

A

Wells score

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4
Q

What affects the Wells score?

A

Affected by active cancer, tenderness along DV system, swollen leg/calf, unilateral pitting oedema and recently bedridden.

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5
Q

What are the symptoms of DVT?

A
  • Nonspecific symptoms
  • Pain and swelling
  • Tenderness
  • Warmth
  • Slight discolouration
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6
Q

What investigations for DVT?

A
  1. D-dimer – look for breakdown of fibrin products, if normal it excludes DVT
  2. Ultrasound compression scan – if you can’t squash the vein it’s a clot.
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7
Q

What is the treatment of DVT?

A
  1. LMWH
  2. Oral Warfarin or DOAC (direct oral anti-coag)
  3. Compression stockings
  4. Treat underlying cause e.g. malignancy
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8
Q

What are 3 risk factors for DVT?

A
  1. Surgery, immobility, leg fracture
  2. OCP,HRT
  3. Long Haul flights
  4. Genetic predisposition
  5. Pregnancy
  6. Age
  7. Obesity
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9
Q

How can you prevent DVT’s?

A
  1. Hydration
  2. Mobilisation
  3. Compression stockings
  4. Low dose LMWH (Low weight molecular heparin
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10
Q

What is the major consequence of a dislodged DVT?

A

Pulmonary embolism

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11
Q

What is the best prophylaxis for DVT?

A

LMWH

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12
Q

Describe arterial thrombosis?

A

Platelet rich - white

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13
Q

Describe venous thrombosis?

A

Fibrin rich - red

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14
Q

What are 3 major consequences of arterial thrombosis?

A
  1. MI
  2. Stroke
  3. Peripheral vasc disease-e.g. gangrene
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15
Q

What is the treatment for arterial thrombosis?

A

Aspirin, LMWH, Thrombolytic therapy.

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16
Q

What is a consequence of venous thrombosis?

A

PE.

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17
Q

How does warfarin work?

A

producing non functional clotting factors 2,7,9,10

Agonist of Vit K.

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18
Q

Why is warfarin difficult to work with?

A

Lots of interactions
Needs constant monitoring
Teratogenic

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19
Q

What is myeloma?

A

Malignancy of plasma cells leading to progressive bone marrow failure, associated with paraprotein, bone disease and renal disease.

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20
Q

What preceding disease is associated with myeloma?

A

monoclonal gammopathy of undetermined significance (MGUS)

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21
Q

What is MGUS?

A

Common disease of paraprotein present in serum but no myeloma, often asymptomatic

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22
Q

What is the treatment for MGUS?

A

Watch and wait.

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23
Q

What are the symptoms of myeloma?

A
  1. Tiredness
  2. Bone/back pain
  3. Infections
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24
Q

What are the signs of Myeloma?

A
  1. Calcium is elevated – increased bone resorption and decreased formation causing raised Ca
  2. Renal failure – light chain deposition
  3. Anaemia – bone marrow infiltrated with plasma cells
  4. Bone lesions
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25
Why is calcium elevated in myeloma?
increased bone resorption and decreased formation causing raised Ca
26
Why is renal failure caused by myeloma?
Light chain deposition
27
Why is anemia caused by myeloma?
Bone marrow infiltrated with plasma cells
28
What investigations would you order in suspected myeloma?
1. Blood film – Rouleaux formation (Aggregations of RBC) 2. Bone marrow aspirate and trephine biopsy - increased plasma cell 3. Electrophoresis – Monoclonal protein band 4. X-ray – bone lesions 5. CT scan 6. MRI scan 7. Chromosomal abnormalities
29
What would you expect to see in a bone marrow biopsy in suspected myeloma?
Increased plasma cell proliferation
30
What would you expect to see using electrophoresis in myeloma?
Monoclonal protein band
31
How would you diagnose myeloma?
Positive investigations Must be evidence of mono-clonality (abnormal proliferation of plasma cell leading to IG secretion causing organ dysfunction In 2/3 of people urine contains ig light chains with kappa or lamda lineage
32
What is the management of symptomatic myeloma?
Chemotherapy, analgesia, bisphosphates Radiotherapy and bone marrow transplant to be done
33
What is lymphoma?
A malignant growth of WBC predominantly in the lymph nodes
34
What organs are often effected in lymphoma?
blood, liver, spleen, bone marrow
35
What are the risk factors for lymphoma?
1. Primary immunodeficiency 2. Secondary immunodeficency e.g. HIV 3. Infection e.g. EBV 4. AI disorders e.g. RA
36
What is the pathophysiology of lymphoma?
Impaired immunosurveillance and infected B cells escape regulation and proliferate
37
What are the symptoms of lymphoma?
1. Enlarged lymph nodes in arms/neck 2. Symptoms of compression syndromes 3. General systemic B symptoms e.g. weight loss, night sweats and malaise 4. Liver and spleen enlargement
38
What are the investigations in lymphoma?
1. Blood film 2. Bone marrow biopsy 3. Lymph node biopsy 4. Immunophenotyping 5. Cytogenetics
39
What are the two subtypes of lymphoma?
1. Hodgkins lymphoma | 2. Non-hodgkins lymphoma
40
Describe Low grade non hodgkins lymphoma
Slow growing, advanced at presentation, often incurable
41
Describe High grade hodgkins lymphoma
Aggressive, nodal presentation, patient unwell, often curable
42
What is the treatment of Low-grade NHL?
If symptomless – do nothing Radiotherapy, combination chemotherapy and mAb may be used if symptomatic
43
What is the treatment for High-grade NHL?
Early – short course chemotherapy and radio therapy Advanced – combination chemotherapy and mAb
44
What are the symptoms of Hodgkins lymphoma?
Painless lymphadenopathy, Presence of B symptoms
45
What must be present for a diagnosis of Hodgkin's lymphoma?
Reed-sternberg cells.
46
What is the staging of Hodgkin's lymphoma
1. Confined to a single lymph node region 2. Involvement of two or more nodal areas on same side of diaphragm 3. Involvement of nodes on both sides of diaphragm 4. Spread beyond the lymph nodes e.g. liver Each stage is either A (absent of B symptoms) or B (presence of B symptoms).
47
What is the treatment of Stage 1-2a Hodgkin's lymphoma?
Short course chemo followed by radiotherapy
48
What is the treatment of Stage 2b-4 Hodgkin's lymphoma?
Combination chemotherapy
49
What are the possible complications of treatments for lymphoma?
``` Secondary malignancies IHD Infertility Nausea Alopecia ```
50
What is leukaemia?
A malignant proliferation of haemopoietic stem cells
51
What are the sub types of leukaemia?
AML – Acute-myeloid leukaemia CML – Chronic-myeloid leukaemia ALL – Acute lymphoblastic leukaemia CLL – Chronic lymphoblastic leukaemia
52
What are the symptoms of Leukaemia?
1. Anaemia 2. Infection 3. Bleeding 4. Hepatomegaly 5. Splenomegaly
53
Why do you get anaemia, bleeding and infection in leukaemia?
Anaemia, bleeding and infection are all due to bone marrow failure
54
Why do you get hepatomegaly and splenomegaly in leukaemia?
Tissue infiltration
55
What investigations would you order for leukaemia?
1. Blood film 2. Bone marrow biopsy 3. Lymph node biopsy 4. Immunophenotyping 5. Cytogenetics
56
What is Acute myeloid leukaemia?
Neoplastic proliferation of blast cells
57
What increases the risk of AML?
Prior chemo, exposure to ionising radiation and preceding haematological disorders
58
What is the treatment for AML?
1. Supportive care 2. Chemotherapy – curative v palliative 3. Bone marrow transplant
59
What is Chronic myeloid leukaemia?
Uncontrolled clonal proliferation of basophils, eosinophils and neutrophils
60
What would you expect to see in the FBC of someone with CML?
High WBC
61
Where is the Philadelphia chromosome present?
In over 80% of people with CML
62
What is the treatment of CML?
Tyranise Kinase inhibitors
63
What is Acute lymphoblastic leukaemia?
Uncontrolled proliferation of immature lymphoblast cells
64
What is the treatment of ALL?
CNS directed therapy and stem cell transplant
65
What is Chronic lymphoblastic leukaemia?
Proliferation of B lymphocytes leads to accumulation of mature B cells that have escaped apoptosis
66
What is the treatment of Chronic lymphoblastic leukaemia?
- Do nothing - Chemotherapy - mAb - Bone marrow transplant
67
What is Anaemia?
Decrease in the amount of Hb in the blood below the reference range.
68
What is the role of Hb in the body?
Carries oxygen to tissues
69
What organ's are responsible for the removal of RBC?
Spleen Liver Bone marrow Blood loss
70
What are the causes of microcytic anaemia?
1. Iron deficiency 2. Anaemia of chronic disease 3. Thalassaemia
71
What are the causes of normocytic anaemia?
1. Acute blood loss 2. Anaemia of chronic disease 3. Combined hematinic deficiency
72
What are the causes of macrocytic anaemia?
1. B12/Folate deficiency 2. Alcohol excess/liver disease 3. Hypothyroid
73
When might a patient present with reduced Hb but increased RCM?
In the 3rd trimester of pregnancy
74
What are the consequences of anaemia?
- Reduced o2 transport | - Tissue hypoxia
75
What are 3 physiological compensatory changes as a result of anaemia?
- Increase tissue perfusion - Increase O2 transfer to tissues - Increase red cell production
76
Where is B12 absorbed at?
The terminal ileum
77
How does pernicious anaemia lead to B12 deficiency?
- Loss of parietal cells --> reduced intrinsic factor production --> B12 Malabsorption
78
What are 5 causes of iron deficiency?
1. Blood loss 2. Poor absorption 3. Decreased intake in diet 4. Hook worm 5. Breast feeding
79
What are 4 symptoms of Iron deficiency?
1. Fatigue 2. Faintness 3. Breathlessness 4. Reduced exercise tolerance
80
What investigations would you carry out in someone with suspected anaemia?
1. Blood tests – FBC + blood film 2. Biopsies 3. Reticulocyte count 4. B12 levels 5. Serum ferritin
81
What is the treatment of anaemia?
- Treat the underlying cause e.g. if iron deficient give ferrous sulphate
82
What is polycythaemia?
Too many RBC - increase in Hb
83
What makes RBC?
Erythropoietin
84
What is EPO stimulated by?
Tissue hypoxia
85
What is the primary cause of polycythaemia?
Polycythaemia rubra vera -- overactive bone marrow
86
What is a secondary cause of polycythaemia?
- Heavy smoking - Lung disease - Cyanotic heart disease - High altitude
87
What is the treatment of polycythaemia?
If primary – treatment aims to maintain a normal blood count and prevent complications e.g. aspirin If secondary – Treat the underlying cause
88
What is neutrophillia?
Too many neutrophils
89
What are the causes of neutrophillia?
1. Infection 2. CML 3. Cancer 4. Inflammation
90
What is lymphocytosis?
Too many lymphocytes
91
What are the causes of lymphocytosis?
1. Viral infection 2. Inflammation 3. Malignancy 4. CLL
92
What is neutropenia?
Not enough neutrophils
93
What are the causes of neutropenia?
1. Marrow failure 2. Marrow infiltration 3. Marrow toxicity
94
What is a major risk of neutropenia?
Infection.
95
Where are platelets produced?
Bone marrow
96
What is platelet regulation controlled by?
Thrombopoietin
97
Where is Thrombopoietin produced?
The Liver
98
What is Thrombocytopenia?
Not enough platelets
99
What is the lifespan of a platelet?
7-10 days
100
What removes platelets?
The Spleen
101
What are the causes of Thrombocytopaenia?
Production failure e.g. marrow suppression or marrow failure Increased removal – splenomegaly, immune response, consumption (DIC)
102
What are the two types of platelet dysfunction?
- Reduced platelet number (thrombocytopenia) | - Reduced platelet function
103
What are the causes of Decreased platelet numbers?
1. Congenital causes e.g. malfunctioning megakaryocytes 2. Infiltration of bone marrow e.g. leukaemia 3. Alcohol 4. Infection e.g. HIV/TB
104
What are the causes of increased platelet destruction?
1. Autoimmune e.g. ITP 2. Hypersplenism 3. Drug related e.g. heparin induced 4. DIC and TTP --> increased consumption
105
What are the causes of reduced platelet function?
1. Congenital abnormality 2. Medication e.g. aspirin 3. VWF disease 4. Uraemia
106
What are the symptoms of platelet dysfunction?
1. Mucosal bleeding 2. Easy bruising 3. Petechiae/purpura – blood spots
107
What are the causes of bleeding?
1. Trauma 2. Platelet deficiency e.g. thrombocytopenia 3. Platelet dysfunction e.g. aspirin induced 4. Vascular disorders
108
What is febrile neutropaenia?
Temperature over 38 degrees with low neutrophils
109
What are the risk factors of febrile neutropaenia?
1. Patient had chemo less than 6 weeks ago 2. Patient who had a stem cell transplant in last year 3. Any haematological condition causing neutropenia 4. Bone marrow infiltration
110
What is the presentation of Febrile neutropaenia?
1. Pyrexia 38 degrees 2. Generally unwell 3. Confusion 4. Hypotensive 5. Tachycardia
111
What is the management of Febrile neutropaenia?
1. Thorough history and examination 2. Bloods 3. Antibiotics within 1 hour
112
What is thalassemia?
Haemoglobin disorder of quantity --> reduced synthesis of one or more globin chains which leads to a reduction in Hb --> anaemia.
113
What is Beta thalassemia?
Very few beta chains, alpha chains in excess
114
What is the classification of B Thalassemia?
1. Thalassemia major – relies on regular transfusions 2. Thalassemia intermedia 3. Thalassemia carrier/heterozygote – often asymptomatic
115
When do people with Thalassemia major present?
Very young - severely anaemic and failure to thrive.
116
Why must Thalassemia major be monitored constantly?
To prevent iron overload after blood transfusions
117
What blood tests would you see in someone with thalassemia?
- Raised reticulocyte count | - Microcytic anaemia
118
What is Sickle cell disease?
A haemoglobin disorder of quality. HbS polymerises  sickle shaped RBC
119
What is the genetic inheritance of sickle cell disease like?
Autosomal recessive and homozygous.
120
What is sickling induced by?
Trauma, cold, stress and exercise
121
What are the chance of offspring having SSA if both parents are carriers?
25%
122
What are the chance of offspring carrying SSA if both parents are carriers.
50%
123
What is an advantage of sickle cell anaemia?
Protection against falciparum malaria
124
How long does Sickle cells last for?
5-10 days
125
How long does a normal red blood cell last for?
120 days
126
Why is Sickle cell anaemia not present until after 6 months of age?
Due to the fact HbF is not affected by sickle cell disease as its made out of 2 A and 2 G chains
127
What are the acute complications of sickle cell disease?
1. Very painful crisis 2. Stroke in children 3. Cognitive impairment 4. Infections
128
What are the chronic complications of sickle cell disease?
1. Renal impairment 2. Pulmonary hypertension 3. Joint damage
129
What is the treatment of Sickle cell disease?
- Transfusion - Hydroxycarbamide – prevents crisis - Stem cell transplant
130
What are the investigations necessary in sickle cell anaemia?
FBC/Blood film Raised reticulocyte count due to increased RBC degradation
131
Why is the parovirus significant?
Common infection in children Leads to decreased RBC production Cause’s a drop in Hb in patients who have an already reduced RBC lifespan
132
What is a membranopathy?
Deficiency of red cell membrane proteins caused by genetic lesions
133
What are two examples of membranopathies?
1. Spherocytosis | 2. Elliptocytosis
134
Are membranopathies autosomal dominant or recessive?
Dominant
135
What are enzymopathies?
Enzyme deficiencies leading to shortened RBC lifespan
136
What is an example of an enzymopathy?
G6PD Deficency
137
What are the signs of G6PD deficiency?
1. Haemolysis 2. Jaundice 3. Anaemia
138
Name 3 categories of red cell disorders
Haemoglobinopathies Membranopathies Enzymopathies
139
What is normal adult haemoglobin made of?
2 alpha and 2 beta chains
140
What is foetal haemoglobin made of?
2 alpha and 2 gamma chains.
141
What is Haemoglobin S?
Variant of Hb arising from point mutation in b globin gene Mutation causes single AA change from valine to glutamine.
142
What is a risk factor for spinal cord compression?
Any malignancy that can cause compression e.g. bone metastasis
143
What is the presentation of spinal cord compression?
1. Back pain 2. Weakness in legs 3. Inability to control bladder 4. Spastic paresis 5. Sensory level
144
Describe the management of spinal cord compression
1. Bed rest 2. High dose steroids 3. Analgesia 4. Urgent MRI of the whole spine
145
What is tumour lysis syndrome?
Break down of malignant cells --> Content release --> metabolic disturbances which can cause Hyperuricaemia Hyperkalaemia Hypocalcaemia
146
What are the risk factors for tumour lysis syndrome?
1. High tumour burden 2. Pre-existing renal failure 3. Increasing age.
147
What is the treatment for Tumour lysis syndrome?
1. Agressive hydration 2. Monitor electrolytes 3. Drugs to reduce uric acid production e.g. allopurinol
148
What is hyperviscosity syndrome?
Increase in blood viscosity usually due to high levels of Ig
149
Consequences of hyperviscosity syndrome
Vascular stasis | Hypoperfusion
150
What is the presentation of hyperviscosity syndrome?
``` Mucosal bleeding Visual change Neurological distrubances Breathlessness Fatigue ```
151
What investigations would you do in someone who has hyperviscosity syndrome?
1. FBC and blood film --> look for rouleaux formation 2. U and E 3. Immunoglobulins
152
What is the treatment of hyperviscosity syndrome?
Keep hydrated Avoid blood transfusion Treat the underlying cause
153
What does rituximab target?
Targets CD20 on the surface of B2.
154
How is myeloma bone disease usually asssessed?
X-ray
155
Haemophilia A is due to a deficiency of what clotting factor?
Factor 8 deficiency
156
Haemophilia B is due to a deficiency of what clotting factor?
Factor 9 deficiency
157
What anaemia could methotrexate cause?
Macrocytic due to folate deficiency
158
Causes of folate deficiency
1. Dietary 2. Malabsorption 3. Increased Requirement e.g. in pregnancy 4. Folate antagonists e.g. methotrexate.
159
Give 3 signs of haemolytic anaemia
1. Pallor 2. Jaundice 3. Splenomegaly
160
Give 4 causes of Haemolytic anaemia
1. GP6D deficiency 2. Sickle cell aenmia 3. Sphereocytosis/eliptocytosis 4. AI haemolytic anaemia.
161
What 3 disorders can cause coagulation disorders?
1. Vit K deficiency 2. Liver disease 3. Congenital e.g. Haemophilia.
162
How does Heparin work?
Activates Antithrombin which inhibits thrombin and factor Xa
163
What is disseminated intravascular coagulation?
Pathological activation of coagulation cascade --> Fibrin in vessel walls . There is platelet and coagulation factor consumption
164
Give 3 causes of disseminated intravascular coagulation (DIC)
Sepsis Major Trauma Malignancy
165
What is the affect on TT, PTT and APTT in someone with DIC.
All increased
166
What is the affect in fibrinogen in someone with DIC
Decreased
167
What is the affect of iron deficiency anaemia on iron binding capacity?
Iron binding capacity will be raised
168
Why might measuring serum ferritin be inaccurate for looking at iron levels?
Ferritin is an acute phase protein so its concentration will increase in response to inflammation.
169
What is aplastic anaemia?
When bone marrow stem cells are damaged --> Pancytopenia.
170
How can multiple myeloma cause AKI?
1. Deposition of Lighht chain 2. Hypercalcaemia Hyperuricaemia
171
Some patients with myeloma can present with blurred vision, gangrene and bleeding. What is the pathology behind this?
Paraproteins form aggregates in the blood which increase visoosity.
172
Why are patients with myeloma be susceptible to recurrent infections.
Reduction in polyclonal Ig levels.
173
What anaemia is seen in patients with multiple myeloma?
Normochromic normocytic.
174
What chemotherapy agent is used in patients with myeloma?
VAD or CTD.
175
What combination therapy is used in patients with non HL?
RCHOP
176
Give 3 environmental causes of leukaemia
Radiation exposure Benzene compounds Drugs
177
Give 3 clinical features of membranopathy
1. Jaundice Anaemia Splenomegaly
178
Why does a deficiency in Glucose-6-phosphate dehydrogenase cause shortened red cells life span
G6PD protects against oxidative damage
179
What 3 blood test values are increased in polycythaemia?
Hb RCC PCV