Haematology Flashcards
(252 cards)
1
Q
What is multiple myeloma?
A
A haematological malignancy characterised by plasma cell proliferation.
2
Q
What are the complications of multiple myeloma?
A
Hypercalcaemia, Renal damage, Anaemia, Thrombocytopenia, Bone lesions, Immune deficiency
(CRABBI)
3
Q
How does multiple myeloma present?
A
Hypercalcaemia: constipation, nausea, confusion.
Renal damage: dehydration, thirst.
Anaemia: fatigue, pallor.
Thrombocytopenia: bleeding, bruising.
Bone lesions: back pain, fragility fractures.
Immune deficiency: freq. infections.
4
Q
What investigations are performed in multiple myeloma?
A
FBC, U&E, blood film, imaging, protein electrophoresis, bone marrow biopsy.
5
Q
What does FBC show in multiple myeloma?
A
Anaemia.
Thrombocytopenia.
6
Q
What do U&Es show in multiple myeloma?
A
Raised urea.
Raised creatinine.
7
Q
What does peripheral blood film show in multiple myeloma?
A
Rouleaux formation.
8
Q
What is a common X-ray finding in patients with multiple myeloma?
A
Rain-drop skull (dark spots on skull due to bone lysis).
9
Q
What does serum/urine protein electrophoresis show in patients with multiple myeloma?
A
Raised concentrations of monoclonal antibody proteins.
10
Q
What does bone marrow biopsy show in patients with multiple myeloma?
A
Raised monoclonal plasma cells (>10%).
11
Q
What is the management of multiple myeloma?
A
Stem cell transplantation and rigorous chemotherapy regimes.
12
Q
What is myelofibrosis?
A
Myeloproliferative disorder thought to be caused by hyperplasia of abnormal megakaryocytes.
13
Q
How does myelofibrosis present?
A
In an elderly person with fatigue, massive hepatosplenomegaly… as well as weight loss and night sweats.
14
Q
What investigations are performed in myelofibrosis?
A
FBC, blood film, bone barrow biopsy.
15
Q
What does FBC show in patients with myelofibrosis?
A
Anaemia, thrombocytopenia, raised WCC.
16
Q
What does blood film show in patients with myelofibrosis?
A
Tear-drop poikilocytes.
17
Q
What does bone marrow aspiration show in patients with myelofibrosis?
A
Bone marrow aspiration may result in a dry tap - no sample is collected because the bone marrow is replaced by collagen.
18
Q
What are thalassaemias?
A
A group of inherited disorders characterised by abnormal haemoglobin production.
19
Q
What is the inheritance pattern of alpha-/beta-thalassaemia?
A
Autosomal recessive.
20
Q
Beta-thalassaemia trait is characterised by what findings on investigation?
A
Hypochromic, microcytic anaemia. As well as raised reticulocytes and raised bilirubin.
21
Q
The clinical severity of alpha-thalassaemia is dependent on what?
A
The number of alpha globulin alleles affected.
22
Q
In alpha-thalassaemia: how does 1 or 2 affected alleles present clinically?
A
Hypochromic, microcytic anaemia but normal haemoglobin. Rarely symptomatic.
23
Q
In alpha-thalassaemia: how does 3 affected alleles present clinically?
A
Hypochromic, microcytic anaemia with splenomegaly.
24
Q
In alpha-thalassaemia: how does 4 affected alleles present clinically?
A
Death in utero (incompatible with life).
25
What is raised HbA2 a sign of?
Beta-thalassaemia.
26
What is factor V Leiden?
The most common cause of inherited thrombophilia.
27
What is antiphospholipid syndrome?
An acquired, autoimmune, hypercoagulable state.
28
How may antiphospholipid syndrome present?
Venous/arterial thrombosis, recurrent foetal loss (in women) and livedo reticularis.
29
What does FBC show in antiphospholipid syndrome?
Thrombocytopenia.
30
What does APTT show in antiphospholipid syndrome?
Prolonged APTT.
31
What prophylaxis is provided for individuals with antiphospholipid syndrome?
Primary: low-dose aspirin.
Secondary: life-long warfarin.
32
What is the target INR for an individual with antiphospholipid syndrome after an initial episode of VTE?
2-3.
33
What is the target INR for an individual with antiphospholipid syndrome after recurrent episodes of VTE?
3-4.
34
Neutropenic sepsis is often a complication of what?
Cancer therapy (e.g. chemotherapy).
35
When does neutropenic sepsis typically develop after chemotherapy?
7-14 days post chemotherapy.
36
What are the features of neutropenic sepsis?
Temperature (>38C) or other signs consistent with sepsis.
37
What is the treatment for neutropenic sepsis?
Tazocin (Piperacillin with Tazobactam).
38
What is haemophilia?
A disorder of coagulation that predisposes to bleeding.
39
What is the inheritance pattern of haemophilia?
X-linked recessive.
40
What are the main features of haemophilia?
Haemoarthroses (bleeding into a joint).
Haematomas (bleeding outside of vessels).
Prolonged bleeding.
41
What investigations are performed in the investigation of haemophilia?
Clotting screen.
42
What does clotting screen show in patients with haemophilia?
Prolonged APTT.
Normal PT.
Normal bleeding time.
43
Of haemophilia A and B, which is more common?
Haemophilia A accounts for 90% of cases.
44
Haemophilia A is marked by deficiency of which clotting factor?
Factor VIII.
45
Haemophilia B is marked by deficiency of which clotting factor?
Factor IX.
46
What is Hodgkin's lymphoma?
A malignant proliferation of lymphocytes and their subsequent accumulation in the lymph nodes, blood and organs.
47
What ages does Hodgkin's lymphoma typically affect?
Large peak in 20-30y age group and a smaller peak in older age (>55y).
48
Hodgkin's lymphoma is associated with infection with what virus?
Epstein Barr Virus (EBV).
49
What is the characteristic presentation of Hodgkin's lymphoma?
Painless, rubbery lymphadenopathy (affecting the cervical nodes especially) and B symptoms.
50
What are the B symptoms associated with Hodgkin's and non-Hodgkin's lymphoma?
Systemic symptoms that include fever, night sweats and weight loss (>10% in 6mo).
51
Other than B symptoms... what other systemic symptoms may be associated with HL?
Itch. Alcohol-induced pain in lymph nodes (rare).
52
What is the investigation of choice for diagnosing Hodgkin's lymphoma?
Lymph node biopsy.
53
What does lymph node biopsy show in patients with Hodgkin's lymphoma?
Reed-Sternberg cells (large multinucleate cells with eosinophilic nucleoli).
54
Why is extensive radiotherapy relied on less than previously in the management of Hodgkin's lymphoma?
Risk of secondary malignancy (especially as HL predominantly affects young adults).
55
What is the management of Hodgkin's lymphoma?
Two-eight cycles of ABVD chemotherapy (depends on severity of disease). ABVD = adriamycin, bleomycin, vinblastine, dacarbazine.
56
What is the Ann Arbor staging system?
Staging system for Hodgkin's lymphoma and non-Hodgkin's lymphoma.
57
Interpret the Ann Arbor staging classification (I, II, III and IV).
I - single lymph node region.
II - two or more regions on same side of diaphragm.
III - two or more regions on both sides of diaphragm.
IV - diffuse disease in extra-lymphatic sites.
58
What is non-Hodgkin's lymphoma?
A group of malignant diseases arising from lymphocytes and their precursors. There is a wide spectrum of disease.
59
Give risk factors for non-Hodgkin's lymphoma.
Longevity, prolonged immunosuppression, EBV infection, H. pylori infection, HIV infection.
60
How does non-Hodgkin's lymphoma present?
Superficial, painless lymphadenopathy with B symptoms and hepatosplenomegaly.
61
What is the investigation of choice regarding diagnosis of non-Hodgkin's lymphoma?
Lymph node biopsy.
62
What is follicular lymphoma?
Low-grade B-cell NHL. The most common subtype of indolent NHL.
63
Who does follicular lymphoma typically affect?
Those in older as late-stage disease.
64
What is Burkitt's lymphoma?
Highly aggressive B-cell malignancy with two main variants.
65
What are the two main variants of Burkitt's lymphoma?
Endemic.
| Sporadic.
66
Describe the endemic subtype of Burkitt's lymphoma.
Occurs most commonly in Africa, is strongly associated with infection with EBV and mainly affects children who present with head/neck tumours.
67
Describe the sporadic subtype of Burkitt's lymphoma.
Less associated with EBV and abdominal disease is more common.
68
Under what circumstances does acute haemolytic transfusion reaction occur?
Results from a mismatch of blood group which causes massive intravascular haemolysis.
69
What pathophysiological mechanism causes the development of acute haemolytic transfusion reaction?
Red blood cell destruction by IgM-type antibodies.
70
When does acute haemolytic transfusion reaction begin following transfusion and what are the features?
Features begin minutes after transfusion begins... includes fever, abdominal pain, chest pain, agitation and hypotension.
71
What is the management of acute haemolytic transfusion reaction?
Immediate transfusion termination and generous fluid resuscitation (saline solution).
72
What mechanism causes the development of non-haemolytic febrile transfusion reaction?
Antibodies reacting with white cell fragments in the blood product and cytokines that have leaked from the blood cell during storage.
73
What are the features of non-haemolytic febrile transfusion reaction?
Fever.
| Chills.
74
What is the management of non-haemolytic febrile transfusion reaction?
Slow or stop the transfusion. Give paracetamol.
75
What mechanism causes the development of a minor allergic transfusion reaction?
Foreign plasma proteins seen as allergens.
76
What are the features of a minor allergic transfusion reaction?
Pruritus.
| Urticaria.
77
What mechanism leads to anaphylaxis post transfusion?
Patients with IgA deficiency who have anti-IgA antibodies.
78
What are the features of transfusion-related anaphylaxis?
Hypotension, dyspnoea, wheezing and angioedema.
79
What is the management of transfusion-related anaphylaxis?
Stop the transfusion. Give intramuscular adrenaline followed by antihistamines, bronchodilators and corticosteroids.
80
What is transfusion-related acute lung injury (TRALI)?
TRALI is a rare but potentially fatal complication of blood transfusion characterised by hypoxaemia/acute respiratory distress syndrome within six hours of transfusion.
81
What are the features of transfusion-related acute lung injury?
Hypoxia, pulmonary infiltrates on CXR, fever and hypotension.
82
What is transfusion-associated circulatory overload (TACO)?
TACO is a common reaction due to fluid overload resulting in pulmonary oedema.
83
What are the features of transfusion-associated circulatory overload?
Dyspnoea, raised JVP, fine crackles on chest auscultation, hypertension, afebrile.
84
What is the characteristic finding on lymph node biopsy of Burkitt's lymphoma?
Starry sky appearance.
85
What is polycythaemia vera?
A myeloproliferative disorder caused by clonal proliferation of a marrow stem cell leading to an increase in blood cells (particularly red blood cells).
86
What mutation is present in 95% of patients with polycythaemia vera?
Mutation in JAK2 enzyme.
87
In which age group does polycythaemia vera peak in incidence?
60s
88
What is the definition of polycythaemia?
High concentration of red blood cells in the blood.
89
What are the presenting features of polycythaemia vera?
Pruritus (typically after hot bath), headache, splenomegaly, plethoric appearance and hypertension.
90
Polycythaemia vera carries an increased risk of what?
Thrombotic events such as myocardial infarction and stroke.
91
What investigations are required in the diagnosis of polycythaemia vera?
Full blood count. Positive JAK2 mutation.
92
What does FBC show in patients with polycythaemia vera?
Raised haematocrit, raised neutrophils, raised basophils and (in half of patients) raised platelets.
93
What is the management of polycythaemia vera?
Low dose aspirin (to reduce the risk of thrombotic events). Provide venesection (to maintain a normal range of haemoglobin). Some patients may require chemotherapy - hydroxyurea.
94
What is the inheritance pattern off sickle cell anaemia?
Autosomal recessive.
95
What is sickle cell anaemia?
Synthesis of an abnormal haemoglobin chain (HbS) which causes a rigid sickle-like shape of red blood cell.s
96
Which screening test includes screening for sickle cell anaemia?
The newborn heel-prick test at five days old.
97
When does a child begin to show signs/symptoms of sickle cell anaemia?
Five-to-six months old.
98
Why do signs/symptoms of sickle cell anaemia develop around six months old?
Foetal haemoglobin is protective in the first few months of life until levels begin to reduce.
99
Describe the pathophysiology of sickle cell disease.
Sickle cells cause increase blood viscosity, reducing blood flow through the small vessels and leading to tissue infarction.
Sickle cells are prematurely destroyed resulting in haemolytic anaemia.
100
What investigation is used for the definitive diagnosis of sickle cell anaemia?
Haemoglobin electrophoresis.
101
Maintenance therapy for sickle cell anaemia includes what?
Vaccinations, folic acid and hydroxyurea/hydroxycarbamide.
102
What advice should be given to the patient and family of sickle cell patients to avoid sickle cell crises?
Avoid hypoxia (air travel), the cold and dehydration.
103
Sequestration crisis (sickle cell crises) is associated with what findings on investigation?
Worsening anaemia.
| Raised reticulocyte count.
104
Acute chest syndrome (sickle cell crises) is associated with what features?
Dyspnoea, chest pain, pulmonary infiltrates and low partial pressure of oxygen.
105
Aplastic crisis (sickle cell crisis) is associated with what findings on investigation?
Sudden fall in haemoglobin and reduced reticulocyte count.
106
What is the most common inherited bleeding disorder?
von Willebrand's disease.
107
What is the most common inherited clotting disorder?
Factor V Leiden.
108
What is the inheritance pattern of von Willebrand's disease?
Autosomal dominant.
109
What is the pathophysiology behind von Willebrand disease?
Deficiency in von Willebrand factor, a large glycoprotein which promotes platelet adhesion to damaged endothelium and a carrier molecule for factor VIII.
110
What are the features of von Willebrand disease?
Epistaxis.
| Menorrhagia.
111
What investigations are performed in suspected von Willebrand disease and what are the results?
```
Bleeding time (prolonged).
APTT (prolonged).
```
112
How is von Willebrand disease managed?
Give tranexamic acid for mild bleeding. Can give desmopressin.
113
By what mechanism is desmopressin useful in the treatment of von Willebrand disease?
Raises levels of vWF by inducing release of vWF from Weibel-Palade bodies in endothelial cells.
114
What is idiopathic thrombocytopenic purpura (ITP)?
An isolated low platelet count with normal bone marrow in the absence of other causes of low platelets.
115
Idiopathic thrombocytopenic purpura typically follows what?
A viral illness.
116
How does idiopathic thrombocytopenic purpura present?
Characteristic red or purple bruise-like rash and an increased tendency to bleed.
117
In patients with idiopathic thrombocytopenic purpura what are the results of a full blood count?
Low platelets.
118
What is the prognosis of idiopathic thrombocytopenic purpura?
Typically self-limiting course between one and two weeks.
119
How should significant bleeding in patients with idiopathic thrombocytopenic purpura be treated?
Oral prednisolone.
120
What is a deep vein thrombosis (DVT)?
The formation of a thrombus in a deep vein, usually in the legs, which partially or completely obstructs blood flow.
121
What is the most serious complication of DVT?
Pulmonary embolism.
122
What scoring system can be used to assess an individual's risk of DVT?
Well's score.
123
What presenting features are included in Well's score for the assessment of DVT?
Localised tenderness along distribution of the venous system.
Entire leg swollen.
Calf swelling ≥ 3 cm larger than the asymptomatic side.
Pitting oedema confined to the asymptomatic leg.
Collateral superficial veins.
124
Other than presenting features, what factors are included in Well's score for the assessment of DVT?
Active cancer.
Paralysis, paresis or recent immobilisation of leg.
Recently bedridden for ≥ 3 days or major surgery within the prev. 12 weeks.
Previous DVT.
125
How is Well's score interpreted?
Score < 2 = DVT unlikely.
| Score ≥ 2 = DVT likely and requires further investigation.
126
What is the next step in management if Well's score indicates DVT is unlikely?
Perform D-dimer within four hours.
127
What is the next step in management if Well's score indicates DVT is unlikely and subsequent D-dimer is negative?
DVT is unlikely and an alternative diagnosis should be considered.
128
What is the next step in management if Well's score indicates DVT is unlikely and subsequent D-dimer is positive?
Offer a proximal leg vein ultrasound within four hours.
129
What is the next step in management if Well's score indicates DVT is likely?
Proximal leg vein ultrasound within four hours.
130
What is the next step in management of DVT if proximal leg vein ultrasound is positive?
Diagnosis confirmed - commence treatment.
131
What is the treatment for DVT?
Anticoagulant therapy (rivaroxaban or apixaban).
132
What is the treatment for DVT if DOACs are contraindicated?
Low molecular weight heparin.
133
How should suspected DVT be managed if proximal leg vein ultrasound cannot be obtained within the required four hours?
Interim therapeutic anticoagulation should be given.
134
The length of anticoagulant therapy following DVT depends on what?
Whether the DVT was provoked or unprovoked.
135
How long should anticoagulant therapy last following an unprovoked DVT?
Six months.
136
How long should anticoagulant therapy last following a provoked DVT?
Three months.
137
What disease(s) should be considered in patients following unprovoked DVT?
Undiagnosed active cancer.
| Thrombophilia (antiphospholipid syndrome or a hereditary cause).
138
What is the most common cause of anaemia worldwide?
Iron deficiency anaemia.
139
Why does iron deficiency cause anaemia?
Iron is required to produce haemoglobin in red blood cells.
140
Give four causes of iron deficiency anaemia.
```
Excessive bleeding (menorrhagia, gastrointestinal bleeding).
Inadequate dietary intake (vegans, vegetarians).
Poor intestinal absorption (coeliac disease).
Increased iron requirements (children, pregnant women).
```
141
What does a blood film show in patients with iron deficiency anaemia?
Hypochromic, microcytic anaemia.
142
What do investigations of ferritin, TIBC and transferrin levels show in iron deficiency anaemia?
Low serum ferritin.
Raised TIBC.
Raised transferrin.
143
How does iron deficiency anaemia present?
Fatigue, shortness of breath on exertion, palpitations, pallor, koilonychia, hair loss, atrophic glossitis.
144
What is the management of iron deficiency anaemia?
Give oral ferrous sulphate.
145
How long should oral ferrous sulphate be given for the treatment of iron deficiency anaemia?
At least three months after the iron deficiency has been corrected.
146
What are side effects of oral ferrous sulphate?
Nausea, abdominal pain, constipation and diarrhoea.
147
What is vitamin B12 used for in the body?
Red blood cell development and myelination of neurones.
148
Where is vitamin B12 absorbed?
The terminal ileum.
149
Vitamin B12 is absorbed in the terminal ileum after binding to which protein?
Intrinsic factor.
150
Intrinsic factor is secreted by which cells?
Parietal cells.
151
Vitamin B12 is found in what foods?
Animal-sourced foods such as meat, fish, milk and eggs.
152
What are causes of vitamin B12 deficiency anaemia?
Pernicious anaemia, post-gastrectomy, vegan or poor diet, disorders of the terminal ileum
153
What does a blood film show in patients with vitamin B12 deficiency anaemia?
Macrocytic anaemia.
154
What does a blood film show in patients with vitamin B12 deficiency anaemia?
Macrocytic, megaloblastic anaemia.
155
How is vitamin B12 deficiency anaemia treated?
1mg intramuscular hydroxocobalamin three times per week for two weeks... then once every three months for life.
156
What is the most common cause of vitamin B12 deficiency anaemia?
Pernicious anaemia.
157
What is pernicious anaemia?
An autoimmune condition that attacks parietal cells.
158
What test may be used in the investigation of pernicious anaemia?
Anti-intrinsic factor antibodies (highly specific but 50% sensitive).
159
What test may be used in the investigation of pernicious anaemia?
Anti-intrinsic factor antibodies (highly specific but 50% sensitive).
160
What is folate (vitamin B9) used for in the body?
The synthesis of DNA and RNA.
161
Folate is found in what foods?
Brocolli, brussel sprouts and leafy green vegetables.
162
What does a blood film show in patients with folate deficiency anaemia?
Macrocytic, megaloblastic anaemia.
163
Give four causes of folate deficiency.
Phenytoin use (treating tonic-clonic seizures).
Methotrexate use.
Pregnancy.
Alcohol excess.
164
In patients with vit B12 deficiency and folate deficiency, which condition should be treated first?
Vitamin B12 deficiency.
165
Why should vit B12 deficiency be treated before folate deficiency?
To prevent subacute combined degeneration of the spinal cord.
166
There is an increased risk of which cancer in patients with pernicious anaemia?
Gastric cancer.
167
Give three mechanisms by which chronic disease causes anaemia.
Reduced iron release from bone marrow.
Inadequate secretion of EPO for erythropoiesis.
Reduced red cell survival.
168
What do investigations of iron, TIBC and ferritin levels show in anaemia of chronic disease?
Low iron, low TIBC and raised ferritin.
169
On investigation, what is the differentiating factor between iron deficiency anaemia and anaemia of chronic disease?
Ferritin is raised in anaemia of chronic disease.
170
Why is ferritin raised in anaemia of chronic disease?
Ferritin is an acute phase reactant and is raised in states of chronic inflammation.
171
What is leukaemia?
A group of blood cancers that usually begin in the bone marrow.
172
What are the four main types of leukaemia?
Acute lymphoblastic leukaemia.
Acute myeloid leukaemia.
Chronic lymphocytic leukaemia.
Chronic myeloid leukaemia.
173
How does leukaemia generally present?
Fatigue, bruising, fever and infection with a feeling of general unwellness.
174
What is acute lymphoblastic leukaemia?
A malignancy of lymphoid cells, affecting B- or T-lymphocyte cell lineages, arresting maturation and promoting uncontrolled proliferation of immature blast cells, with marrow failure and tissue infiltration.
175
Is acute lymphoblastic leukaemia more common in children or adults?
ALL is the most common cancer in childhood and is rare in adults.
176
Give two associations that may predispose to acute lymphoblastic leukaemia.
Down's syndrome.
| Foetal exposure to ionising radiation during pregnancy.
177
In acute lymphoblastic leukaemia: presentation develops secondary to what two processes?
Bone marrow failure and tissue infiltration.
178
In acute lymphoblastic leukaemia: what presentation occurs secondary to bone marrow failure?
Fatigue (anaemia), fever (infection), bleeding and bruising (thrombocytopenia).
179
In acute lymphoblastic leukaemia: what presentation occurs secondary to tissue infiltration?
Hepatosplenomegaly, lymphadenopathy, orchidomegaly and CNS involvement (cranial nerve palsies, meningism).
180
In acute lymphoblast leukaemia: what are six common infections?
Bacterial septicaemia, zoster, cytomegalovirus, measles, candidiasis, Pneumocystitis pneumonia.
181
In acute lymphoblastic leukaemia: what investigations are performed?
FBC, blood film and bone marrow biopsy, imaging (CXR, CT scan).
182
In acute lymphoblastic leukaemia: how would you provide supportive care?
Blood / platelet transfusions.
Intravenous fluids.
Allopurinol (to prevent tumour lysis syndrome),.
183
In acute lymphoblastic leukaemia: what is the mainstay of treatment?
Chemotherapy.
184
What is acute myeloid leukaemia?
A neoplastic proliferation of blast cells derived from marrow myeloid elements.
185
What is the prognosis of acute myeloid leukaemia?
AML progresses rapidly and without treatment typically causes death within two months of onset.
186
What is the most common acute leukaemia of adults?
Acute myeloid leukaemia.
187
In acute myeloid leukaemia: presentation develops secondary to what two processes?
Bone marrow failure.
| Tissue infiltration.
188
In acute myeloid leukaemia: what presentation occurs secondary to bone marrow failure?
Fatigue (anaemia), fever (infection), bleeding and bruising (thrombocytopenia).
189
Acute promyelocytic leukaemia (a subtype of AML) can result in what complication?
Disseminated intravascular coagulation.
190
In acute myeloid leukaemia: what presentation occurs secondary to tissue infiltration?
Hepato-/splenomegaly, gum hypertrophy, skin involvement (purple deposits).
191
In acute myeloid leukaemia: what investigations are performed?
Blood testing (FBC), bone marrow biopsy.
192
In acute lymphoblastic leukaemia: what is the finding on blood film?
Characteristic blast cells.
193
In acute myeloid leukaemia: what is the finding on bone marrow biopsy?
Myeloblasts present in > 20% of bone marrow.
| Auer rods on biopsy.
194
What is the normal myeloblast count in healthy bone marrow?
< 5%.
195
How is acute lymphoblastic leukaemia differentiated from acute myeloid leukaemia on bone marrow biopsy?
The presence of Auer rods on biopsy of AML.
196
In acute myeloid leukaemia: how would you provide supportive care?
Blood/platelet transfusions.
Intravenous fluids.
Allopurinol (to prevent tumour lysis syndrome).
197
In acute myeloid leukaemia: what is the mainstay of treatment?
Intensive chemotherapy.
198
What is a treatment option for individuals with acute myeloid leukaemia if chemotherapy fails or they have relapses?
Bone marrow transplant.
199
What is a complication of bone marrow transplant for patients with acute myeloid leukaemia?
Graft-versus-host disease (GvHD).
200
What is a graft-versus-host disease (following bone marrow transplant)?
New marrow attacks the patient's body. The donor's immune system white blood cells reject the recipient.
201
How is graft-versus-host disease managed following bone marrow transplant?
Ciclosporin and methotrexate can be given to reduce the effect.
202
What is chronic lymphocytic leukaemia?
CLL is characterised by a progressive accumulation of a malignant clone of functionally competent B-cells which effectively crowd out healthy blood cells.
203
What is the most common leukaemia in those aged > 60 years?
Chronic lymphocytic leukaemia.
204
Does chronic lymphocytic leukaemia develop slowly or quickly?
CLL develops slowly over several years.
205
How does chronic lymphocytic leukaemia present in the early stages of disease?
CLL is typically asymptomatic in the early stages of the disease and may be an incidental finding on routine blood testing.
206
How does chronic lymphocytic leukaemia present in later stages of the disease?
Later presentation may include non-painful lymph node swelling, hepatosplenomegaly, fatigue, fever, night sweats and/or weight loss.
207
In chronic lymphocytic leukaemia: what is the finding on FBC?
Raised lymphocytes.
208
In chronic lymphocytic leukaemia: what is the finding on blood film?
Smudge cells.
209
How is chronic lymphocytic leukaemia managed if the patient is asymptomatic?
Watch and wait, monitoring FBC every three months.
210
In chronic lymphocytic leukaemia: what is the first-line therapy for symptomatic individuals?
Fludarabine.
Rituximab.
Cyclophosphamide.
211
What is the prognosis for individuals with chronic lymphocytic leukaemia?
1/3 never progress.
1/3 progress slowly.
1/3 progress actively.
212
Give two complications of chronic lymphocytic leukaemia.
Warm autoimmune haemolytic anaemia.
| Richter's transformation.
213
Describe the pathophysiology of warm autoimmune haemolytic anaemia (complication of CLL)?
IgG antibodies attach to red blood cells, which are transformed into spherocytes.
214
What test is positive in patients with warm autoimmune haemolytic anaemia?
Coombs' test.
215
Describe the pathophysiology of Richter's transformation (complication of CLL)?
Leukaemia cells enter the lymph node and change into a high-grade, fast-growing non-Hodgkin's lymphoma.
216
What is chronic myeloid leukaemia?
CML is characterised by an uncontrolled clonal proliferation of myeloid cells.
217
In what age group does chronic myeloid leukaemia most commonly occur?
40-60 years.
218
Chronic myeloid leukaemia is strongly associated with what genetic abnormality?
Philadelphia chromosome - present in over 80% of individuals with CML.
219
How does chronic myeloid leukaemia present?
Presentation of CML includes weight loss, tiredness, fever and sweats. There may be features of gout, bleeding and abdominal discomfort.
220
In chronic myeloid leukaemia: why may patients experience gout?
Increased cell turnover leads to excess purines... which are broken down to uric acid which accumulates and causes gout.
221
In chronic myeloid leukaemia: what is found on FBC?
Raised WCC (raised neutrophils, monocytes, basophils, eosinophils).
222
In chronic myeloid leukaemia: what is found on blood film?
All stages of granulocyte maturation.
223
In chronic myeloid leukaemia: what is the management?
Intravenous fluids and allopurinol.
Imatinib.
Chemotherapy (in patients with lymphoblastic transformation).
224
What is disseminated intravascular coagulation?
DIC is an inappropriate activation of the clotting cascade that results in an increased risk of bleeding.
225
Describe the pathophysiology of disseminated intravascular coagulation?
Release of procoagulants into the circulation causes widespread activation of coagulation, consuming clotting factors and platelets.
226
How does disseminated intravascular coagulation present?
Presentation may vary from no bleeding to severe epistaxis and gingival bleeds. Other features include bruising, petechiae, haematuria, fever, confusion and coma.
227
In disseminated intravascular coagulation: what does a clotting screen show?
Reduced platelets.
Prolonged prothrombin time (PT).
Prolonged activated partial thromboplastin time (aPTT).
Fibrinogen reduced.
228
In disseminated intravascular coagulation: what does a D-dimer show?
Markedly raised.
229
In disseminated intravascular coagulation: what does a blood film show?
Schistocytes (broken RBCs).
230
What is the presentation of autoimmune haemolytic anaemia?
Fatigue and shortness of breath.
231
What tests are performed in autoimmune haemolytic anaemia (AIHA)?
Haemoglobin, lactate dehydrogenase, bilirubin and haptoglobin.
232
What are the results of haemoglobin level in AIHA?
Anaemia.
233
What are the results of bilirubin and haptoglobin level in AIHA?
Raised bilirubin and low haptoglobin.
234
What is G6PD deficiency?
Inborn error of metabolism that predisposes to red blood cell breakdown (haemolytic anaemia).
235
What is the inheritance pattern of G6PD deficiency?
X-linked recessive.
236
What is the presentation of G6PD deficiency?
Jaundice, pallor, dark urine, shortness of breath and fatigue.
237
G6PD deficiency presentation typically follows which triggers?
Infection, medication (ciprofloxacin) and stress.
238
What investigations are performed in an individual suspected of having G6PD deficiency?
Full blood count and blood film.
239
What does FBC reveal in an individual with G6PD deficiency?
Anaemia.
240
What does blood film reveal in an individual with G6PD deficiency?
Heinz bodies and bite cells.
241
In G6PD deficiency: how are bite cells formed?
Macrophage removal of Heinz body.
242
How is G6PD deficiency managed?
Avoid triggers. Treat infection // stop offending medications.
243
What is the most common hereditary haemolytic anaemia in people of Northern European descent?
Hereditary spherocytosis.
244
What is the inheritance pattern of hereditary spherocytosis?
Autosomal dominant.
245
What is the presentation of hereditary spherocytosis?
Failure to thrive (children), jaundice, gallstones, splenomegaly.
246
What investigations are performed in an individual suspected of hereditary spherocytosis?
Reticulocyte count, blood film.
247
What does reticulocyte count reveal in an individual with hereditary spherocytosis?
Raised reticulocytes.
248
What does blood film reveal in an individual with hereditary spherocytosis?
Spherocytes on blood film.
249
Post-thrombotic syndrome is a potential complication following what?
DVT.
250
Describe the pathophysiology of post-thrombotic syndrome.
Venous outflow obstruction and venous insufficiency result in chronic venous hypertension.
251
What are the features of post-thrombotic syndrome?
Heavy, painful calves, pruritus, swelling, varicose veins and venous ulceration.
252
What is the management of post-thrombotic syndrome?
Graduated compression stockings and leg elevation.
