Haematology Flashcards
1
Q
What causes increased reticulocytes on an FBC?
A
Bleeding, haemolysis
2
Q
What causes neutrophils to be raised?
A
Acute inflammatory process e.g. infection, acute illness, steroid therapy, myeloid cancer
3
Q
What type of infection raises eosinophils?
A
A parasitic infection
4
Q
What are platelets derived from?
A
Megakaryocytes
5
Q
What leukaemia typically affects children?
A
Acute lymphoblastic leukaemia
6
Q
What investigations would you do for ?leukaemia?
A
FBC, blood film, U&Es, LFTs, clotting and a bone marrow biopsy
7
Q
What are the basics of the treatment of acute leukaemia?
A
Induction - aims to remove 99% of leukaemic cells
Consolidation- lower intensity therapy
Maintenance
CNS prophylaxis - intrathecal
8
Q
What chromosomal abnormality do CML patients tend to have?
A
The philadelphia chromosome
9
Q
How is CML treated?
A
Imatinib
10
Q
What is the most common leukaemia?
A
Chronic lymphocytic leukaemia
11
Q
What makes a Hodgkin’s lymphoma different from non-Hodgkin’s lymphoma?
A
Reed-Steinberg cells
12
Q
What is the common age range for Hodgkin’s lymphoma?
A
20-40
13
Q
What system is used to class Hodgkin’s lymphoma?
A
Ann Arbor system
14
Q
What is multiple myeloma?
A
A malignant proliferation of plasma cells
15
Q
What are the emergencies in haematology?
A
Spinal cord compression SVC obstruction Neutropenic sepsis Tumour lysis syndrome Hypercalcaemia
16
Q
What are the general signs and symptoms of anaemia?
A
Lethargy, SOB, reduced exercise tolerance, headache, pallor of the palmar creases and conjunctiva
Severe: angina, tachycardia
17
Q
What are the different causes of anaemia?
A
Decreased RBC production
Increased RBC destruction
Increased RBC loss
18
Q
What anaemias are microcytic?
A
Iron deficiency
Thalassaemia
Haemaglobinopathies
(Sideroblastic anaemia)
19
Q
What anaemias are normocytic?
A
Acute blood loss Haemolysis Renal failure Chronic disease Cancer
20
Q
What anaemias are macrocytic?
A
B12 deficiency Folate deficiency Alcohol excess Liver/thyroid disease Anti-folate medication (anti-epileptics, trimethoprim and methotrexate)
21
Q
What are the specific signs of iron deficiency anaemia?
A
Kolionychia
Glossitis
Angular stomitis (sores in the corners of the mouth)
22
Q
What investigations would you do for anaemia?
A
FBCs, blood film
Investigate for underlying cause e.g. malignancy
23
Q
What is the management for iron-deficiency anaemia?
A
Treat underlying condition
Iron replacement
Transfuse if very flow
Prescribe laxatives as iron salts constipate patients
24
Q
What types of crises occur is sickle cell anaemia?
A
Haemolytic, occlusive, aplastic and sequestration
25
How do you investigate sickle cell anaemia?
FBC, blood film, Hb electrophoresis, G&S
26
What is the management for sickle cell anaemia?
Supportive e.g. aggressive analgesia, treat underlying cause, folic acid, transfuse with falling Hb
Chronic e.g. hydroxycarbamide
27
What are the causes of B12 deficiency?
Pernicious anaemia, malabsorption (e.g. in Crohn's), dietary
28
What is pernicious anaemia?
An autoimmune condition where antibodies are produced against parietal cells, associated with other autoimmune conditions e.g. Addison's.
Parietal cells produce intrinsic factor which is necessary for B12 absorption.
29
What are the causes of folate deficiency?
Dietary, malabsorption, increased requirements (e.g. pregnancy), folate antagonists
30
Where is folate absorbed?
Duodenum/upper small anaemia
31
What are the congenital causes of haemolytic anaemia?
Hereditary spherocytosis/elliptocytosis, thalassaemia, sickle cell anaemia, G6P deficiency
32
What are the symptoms of haemolytic anaemia?
Lethargy, SOB, reduced exercise tolerance, headache, pallor of the palmar creases and conjunctiva, jaundice, splenomegaly
33
What is prothrombin time a marker for?
The extrinsic pathway
34
What is the activated Prothrombin time a marker for?
The intrinsic pathway
35
What is the mechanism of action of warfarin?
It is antagonises vitamin K dependant clotting factors e.g. 2, 7, 9, 10
36
How does heparin work?
It inactivates factor Xa
37
What are the causes of DIC?
Sepsis, haematological cancers, eclampsia, anaphylaxis, severe trauma/burns, severe liver disease
38
What is DIC?
A pathological activation of the coagulation cascade. There is formation of microvascular thrombi and multi-organ failure.
39
What causes thrombophilia?
Primary: Factor V Leiden, Protein C deficiency, protein S deficiency, anti-thombin III deficiency
Secondary: Virchow's triad
40
What are the risk factors for DVT?
Increased age, pregnancy, malignancy, trauma, past DVT, smoking, immobility, infection, thrombophilia, HRT
41
What are the symptoms of a DVT?
Pain/ache, unilateral swelling, calf warmth, pitting oedema below the DVT, erythema, Homan's sign (pain on foot dorsiflexion)
42
What is the Well's score?
The diagnostic score for a DVT
43
What investigations would you do for a DVT?
D-dimer, USS doppler, CTPA with a PE
44
What is the management of a DVT?
Anticoagulate with treatment dose dalteparin or LMWH
Warfarin/NOAC
Exclude underlying cause - IVC filters may be used
45
What is the cause of iron deficiency anaemia?
Excessive blood loss e.g. from the GI tract or menorrhagia, dietary inadequacy e.g. in children, failure of iron absorption e.g. coeliac disease or excessive requirements for iron e.g. pregnancy
46
What is the pathophysiology behind iron deficiency anaemia?
Iron is required for haemoglobin synthesis therefore inadequate iron means haemoglobin can't be synthesised properly and the patient becomes anaemic
47
What are the symptoms of iron deficiency anaemia?
Fatigue, shortness of breath on exertion, palpitations, brittle hair, headache, tinnitus, angina (if there's pre-existing coronary heart disease)
48
What are the signs of iron deficiency anaemia?
Spoon shaped nails (koilonychia)
| Angular stomatitis, brittle nails, pallor
49
What tests are used to diagnose iron deficiency anaemia?
FBC - decreased Hb, decreased MCV, decreased MCH (mean corpuscular Hb)
Serum iron (low)
Total iron binding capacity (high)
Serum ferritin (low)
Serum soluble transferrin receptors (high)
OGD in all males and post menopausal females to look for GI causes of bleeding
50
What is the treatment for iron deficiency anaemia?
Find and treat the underlying cause
| Give iron supplements to treat the anaemia and replace stores - oral iron is usually sufficient
51
What type of anaemia is iron deficiency anaemia?
Microcytic
52
What is the aetiology of vitamin B12-deficiency anaemia?
Impaired absorption of vitamin B12 e.g. gastrectomy, ileal resection
Inadequate intake e.g. vegan diet
Drugs e.g. colchicine
53
What is the pathophysiology of pernicious anaemia?
Absorption of vitamin B12 occurs in the terminal ileum and needs intrinsic factor (produced in the stomach). Pernicious anaemia is an autoimmune process causing gastritis which leads to atrophy of all layers of the body and fundus of the stomach leading to a lack of intrinsic factor
54
What is the peak age at diagnosis for pernicious anaemia?
60
55
What are the symptoms of pernicious anaemia?
Fatigue, lethargy, dyspnoea, faintness, palpitations, headache, paraesthesia, numbness, visual disturbances
56
What are the signs of pernicious anaemia?
Pallor, lemon tinge to the skin, glossitis, oral ulceration, angular stomatitis, reflex loss, mild to moderate ataxia
57
What tests are used to diagnose pernicious anaemia?
```
FBC - increased MCV
Blood film - hypersegmented neutrophils
Serum vitamin B12 - low
Plasma total homocysteine - high
Serum folate
```
58
What is the treatment for pernicious anaemia?
Find the underlying cause of low vitamin B12
IM hydroxocobalamin 1mg for 3 times a week for 2 weeks then 1 mg every 3 months
Refer to gastro if the patient has malabsorption or GI symptoms
59
What type of anaemia is pernicious anaemia?
Macrocytic
60
What is the aetiology of folate deficiency?
Dietary deficiency e.g. malabsorption, alcohol excess
Excessive requirements e.g. pregnancy, malignancy, inflammation
Excessive excretion
Drugs e.g. methotrexate
61
What is the pathophysiology of folate deficiency?
Folate is involved in adenosine, guanine and thymidine synthesis so it's required for DNA synthesis. Reduced DNA synthesis results in anaemia
62
What are the symptoms of folate deficiency?
Fatigue, lethargy, dyspnoea, faintness, palpitations, headache, leg cramps
63
What are the signs of folate deficiency?
Pallor, glossitis, angular stomatitis, tachycardia, systolic flow murmur, pale mucus membranes and palmar creases
64
What tests are used to diagnose folate deficiency?
FBC - increased MCV, low Hb
Serum folate - low
RBC folate level - low (more specific than serum folate level)
65
What is the treatment for folate deficiency?
Folic acid 5mg OD
Don't give alone in megaloblastic anaemia of unknown cause as it makes neuropathy of B12 deficiency worse
Treat the underlying cause
66
What type of anaemia does folate deficiency cause?
Macrocytic
67
What causes anaemia of chronic disease?
Chronic infection e.g. TB
Chronic inflammation e.g. Crohn's disease, RA, SLE, malignancy
CKD
68
What is the pathophysiology of anaemia of chronic disease?
Different causes contribute to anaemia in chronic diseases including diversion of iron, reduced erythropoiesis and reduced response to erythropoeitin
69
What are the symptoms of anaemia of chronic disease?
Fatigue, lethargy, dyspnoea, faintness, palpitations, headache, angina, intermittent claudication
70
What are the signs of anaemia of chronic disease?
Pallor, tachycardia, systolic flow murmur, cardiac failure, pale mucus membranes and palmar creases
71
What tests are used to diagnose anaemia of chronic disease?
FBC - low MCV, low Hb
Serum iron - low
Total iron binding capacity - low
Serum ferritin - high (increases due to the inflammatory process)
72
What is the treatment for anaemia of chronic disease?
Treatment and management of the underlying cause
Iron should only be given to patients with established iron deficiency
Consider erythropoiesis-stimulating agents in RA, HF and cancer
73
What type of anaemia is anaemia of chronic disease?
Microcytic
74
What is the aetiology of beta-thalassaemia?
It is an autosomal recessive condition with defects in transcription, RNA splicing & modification and translation leading to unstable and unusable beta-globin chains.
75
What is the pathophysiology of beta-thalassaemia?
There is little or no normal beta-globin and therefore there is excess alpha-globin. Alpha-globin combines with whatever beta, delta or gamma chains are available are there is an increased production of HbA2 and HbF leading to ineffective erythropoiesis and haemolysis.
76
Where is beta-thalassaemia prevalent?
The mediterranean, middle east and Africa
77
What are the symptoms of beta-thalassaemia?
Asymptomatic - minor/trait
Bone abnormalities and recurrent leg ulcers - intermedia
Chronic infections, failure to thrive - major (homozygous)
78
What are the signs of beta-thalassaemia?
Mild/absent anaemia - minor/trait
Moderate anaemia, splenomegaly and gallstones - intermedia
Severe anaemia, skull bossing, thalassaemia facies, hepatosplenomegaly (presents in the first year of life) - major (homozygous)
79
What tests are used to diagnose beta-thalassaemia?
FBC and blood film - hypochromic and microcytic anaemia, irregular and pale RBCS, increased reticulocytes and nucleated RBCs.
Hb electrophoresis - HbA2 >3.5%
80
What is the treatment for beta-thalassaemia?
Blood transfusions - risk of iron overload, give iron chelation (desferroxamine) or ascorbic acid (increases urinary excretion of iron)
Long term folic acid supplements
Promote fitness and a healthy diet
81
What type of anaemia is beta-thalassaemia?
Microcytic anaemia
82
What is the aetiology of sickle cell anaemia?
It is an autosomal recessive genetic disorder in the alpha globin gene
83
What is the pathophysiology involved in sickle cell anaemia?
Substitution of valine for glutamic acid causes a conformational change producing HbS not HbA. Cells become rigid, sickle shaped and dehydrated leading to haemolysis (premature) and obstruction of the microcirculation leading to tissue infarction.
84
What is sickling precipitated by?
Hypoxia, infection, dehydration and cold
85
What are the symptoms of sickle cell anaemia?
Crisis: acute pain in the hands and feet in children, bone pain (ribs, spine and pelvis) in adults
Acute chest syndrome: chest pain, dyspnoea
86
What are the signs of sickle cell anaemia?
Crisis: fever (+pain)
Acute chest syndrome: hypoxia and new CXR changes (consolidation) caused by infection, fat emboli and pulmonary infarction
87
What tests are used to diagnose sickle cell anaemia?
FBC - low Hb, high reticulocytes
Blood film - sickled erythrocytes
Hb electrophoresis - HbS present
88
What is the treatment for sickle cell anaemia?
Prophylactic antibiotics (daily penicillin) due to hyposplenism
Pneumococcal and influenza vaccine
Folic acid
Pain relief for crisis
Hydroxyurea to increase the concentration of HbF
89
What are the complications of sickle cell anaemia?
Stunted growth, infections, leg ulcers, cholelithiasis, cardiac problems, avascular necrosis, spinal cord compression, CKD and pulmonary hypertension
90
What causes haemolytic anaemias?
Genetic - G6PD deficiency, hereditary spherocytosis
| Acquired - immune e.g. blood transfusion reaction or autoimmune e.g. SLE, CLL, DIC
91
What is the pathophysiology involved in haemolytic anaemias?
There may be intravascular haemolysis due to complement fixation or trauma (e.g. heart valve) or it may be extravascular where RBCs are removed from the circulation
92
What is the epidemiology of haemolytic anaemias?
Autoimmune: more common in females
93
What are the symptoms of haemolytic anaemias?
Fatigue, lethargy, dyspnoea, faintness, palpitations, headache, dizziness, intermittent claudication
94
What are the signs of haemolytic anaemias?
Pallor, tachycardia, systolic flow murmur, cardiac failure, pale mucus membranes and palmar creases, jaundice, gallstones, hepatosplenomegaly
95
What tests are used to diagnose haemolytic anaemias?
```
FBC - normal MCV and MCH, normal platelet count
Coombs test
USS of spleen to asses size
CXR
ECG
```
96
What is the treatment for haemolytic anaemias?
Give folic acid as active haemolysis can cause deficiency
Identify and treat the cause of haemolysis
Corticosteroids for autoimmune haemolytic anaemia
Splenectomy for hereditary spherocytosis
97
What type of anaemia is sickle cell anaemia?
Microcytic
98
What type of anaemia is haemolytic anaemia?
Normocytic
99
What are some causes of aplastic anaemia?
Congenital e.g. Fanconi anaemia or Diamond-Blackfan syndrome
| Acquired e.g. EBV, HIV, parvovirus, irradiation, pregnancy, drugs e.g. phenytoin
100
What is the pathophysiology involved in aplastic anaemia?
Pancytopenia with hypocellularity (aplasia) of the bone marrow. The bone marrow is replaced with fat
101
What are the symptoms of aplastic anaemia?
Fatigue, lethargy, dyspnoea, faintness, palpitations, headache, dizziness, skin or mucosal haemorrhages, visual disturbances, petechial rashes
102
What are the signs of aplastic anaemia?
Pallor, bruising, bleeding gums, tachycardia, epistaxis, systolic flow murmur
103
What tests are used to diagnose aplastic anaemia?
FBC - normal MCV, pancytopenia, reticulocyte count low or absent, blood film, bone marrow biopsy (hypocellular)
104
What is the treatment of aplastic anaemia?
Withdrawal of offending agent and supportive care
Blood and platelet transfusions
Prophylactic antibiotics or prompt treatment of infections
If <40 then a bone marrow transplant is curative
If >40 then immunosuppression with anti-thymocyte globulin and ciclosporin
105
What is the aetiology of polycythaemia?
Primary - polycythaemia vera, mutations in Epo receptor
| Secondary - high altitude, lung disease, dehydration, burns, RCC, Wilms' tumour
106
What is the pathophysiology of polycythaemia?
There is an increase in Hb, PCV and red cell count. It can be divided into absolute erythrocytosis (true increase in red cell volume) or relative erythrocytosis (red cell volume is normal but plasma volume is low)
107
What are the symptoms of polycythaemia?
Polycythaemia vera - tiredness, depression, vertigo, tinnitus and visual disturbances, itching, hyperviscosity symptoms e.g. headache
108
What are the signs of polycythaemia?
Thrombosis, splenomegaly, HTN, stroke/CVA, MI, PE, DVT
109
What tests are used to diagnose polycythaemia?
FBC - primary: high Hb, PCV, WCC and platelets; secondary: increased RBCs
Bone marrow aspirate - tends to be hypercellular
110
What is the treatment of polycythaemia?
Aspirin, venesection, bone marrow suppression with hydroxycarbamide
111
What is the aetiology of myeloma?
Monoclonal gammopathy of undetermined significant (MGUS) increases the risk of developing myeloma, obesity is also a risk
112
What is the pathophysiology of myeloma?
Clonal expansion of abnormal, proliferating plasma cells producing monoclonal paraprotein, malignant cells accumulate in the bone marrow leading to bone marrow failure.
113
Who does myeloma affect predominantly?
Elderly males
114
What are the symptoms of myeloma?
Bone pain, particularly backache, lethargy, anorexia, bleeding and/or bruising, recurrent bacterial infections, symptoms of hypercalcaemia e.g. thirst
115
What are the signs of myeloma?
Hypercalcaemia, renal injury, anaemia, bone lesions, UMN muscle weakness, sensory level sensation loss, urinary incontinence - spinal cord compression
116
What tests are used to diagnose myeloma?
FBC - low Hb, neutrophils and platelets
U&Es - high creatinine
Serum calcium - high
Urine electrophoresis - Bence Jones protein
Skeletal survey - pepper pot skull
Bone marrow aspirate - increased BM plasma cells
117
What is the treatment for myeloma?
Anaemia - give transfusions and Epo
Infection prophylaxis - pneumococcal/influenza
Bone pain - radiotherapy and systemic chemotherapy or high dose dexamethasone, bisphosphonates
Kidney - dialysis
Stem cell transplant
Combination chemotherapy - steroid & melphalan & thalidomide
118
What are some risks for developing Hodgkin's lymphoma?
EBV, having had mononucleosis, HIV, immunosuppression, smoking
119
What is the pathophysiology of Hodgkin's lymphoma?
It is a malignant tumour of the lymphatic system characterised by Reed-Steinberg cells (multinucleated giant cells)
120
What is the epidemiology of Hodgkin's lymphoma?
Young patients: males > females
121
What are the symptoms of Hodgkin's lymphoma?
Enlarged but otherwise asymptomatic lymph node, typically in the lower neck/supraclavicular region
Night sweats, unexplained fever, weight loss, cough, dyspnoea
122
What are the signs of Hodgkin's lymphoma?
Lymphadenopathy, hepatomegaly, splenomegaly, SVC syndrome
123
What tests are used to diagnose Hodgkin's lymphoma?
FBC, U&E, ESR, LFTs, HIV (used to exclude DDx)
Lymph node biopsy - excisional is better than fine needle/core needle
CT scans for staging
CXR
Bone marrow aspiration
Ann Arbor Staging
124
What is the treatment for Hodgkin's lymphoma?
ABVD combination chemotherapy
Involved field radiotherapy
BEACOPP
125
What are some problems associated with chemotherapy?
Infertility, lung damage, peripheral neuropathy, cardiomyopathy
126
What are some risk factors associated with Non-Hodgkin's lymphoma?
Chromosomal translocations, EBV, HTLV-1, Hepatitis C, environmental factors e.g. pesticides, autoimmune disorders
127
What is the pathophysiology of Non-Hodgkin's lymphoma?
A heterogenous group of lymphoproliferative malignancies with different patterns of behaviour and responses to treatment, can be low or high grade.
80% are of B cell origin
128
What is the epidemiology of Non-Hodgkin's lymphoma?
Adults >50
129
What are the symptoms of Non-Hodgkin's lymphoma?
Painless, peripheral lymphadenopathy, night sweats, fever, weight loss, testicular mass, bleeding, recurrent infections
130
What are the signs of Non-Hodgkin's lymphoma?
Pancytopenia, splenomegaly, hepatomegaly, skin lesions, SVC syndrome and an abdominal mass
131
What tests are used to diagnose Non-Hodgkin's lymphoma?
```
FBC - anaemia, thrombocytopenia, neutropenia
U&Es
Serum calcium - high
LFTs
Serology
CXR
CT head to pelvis
Lymph node biopsy
```
132
What is the treatment of Non-Hodgkin's lymphoma?
Depends on the type of lymphoma
Gastric lymphoma - eradicate H. pylori
Aggressive e.g. B cell lymphoma - rituximab and combination chemotherapy
133
What are the risk factors for DVT and VTE?
Previous VTE, family history of VTE, having a cancer, age >60, immobilisation, smoking, BMI >30, being male, pregnancy, combined OCP and HRT
134
What is the pathophysiology involved in DVT and VTE?
A thrombus occurs when Virchow's triad is met - hypercoagulability, stasis and endothelial injury
135
What are the symptoms of DVT/VTE?
Limb pain and tenderness, skin discolouration (erythema, purple or cyanosed), unilateral swelling of the calf/thigh
136
What are the signs of a DVT/VTE?
Pitting oedema, distension of the superficial veins, increase in skin temperature, palpable cord
137
What tests are used to diagnose DVT/VTE?
FBC, U&E, CRP (to rule out cellulitis), D-dimer
| Imaging: USS of the leg with a colour doppler, CT or MRI venography
138
What is the treatment for DVT/VTE?
Low molecular weight heparin or fondaparinux, novel oral anticoagulants e.g. rivaroxaban for 3 months (at least)
IVC filter
Catheter-directed thrombolytic therapy
139
What is the aetiology of AML?
Most cases arise without apparent cause but some conditions predispose to it e.g. aplastic anaemia, myelofibrosis, polycythaemia rubra vera
140
What is the pathophysiology of AML?
There is clonal expansion of myeloblasts in the bone marrow, blood and other tissues; failure of apoptosis leads to accumulation in various organs.
141
What is the epidemiology of AML?
It is the most common leukaemia in adults
142
What are the symptoms of AML?
Fatigue (over weeks/months), dizziness, dyspnoea on exertion, bleeding, recurrent infections, bone pain
143
What are the signs of AML?
Pallor, gum hypertrophy and bleeding, fever, splenomegaly, hepatomegaly, signs of infection e.g. fever, petechiae
144
What tests are used to diagnose AML?
FBC - anaemia, thrombocytopenia, increased WCC, decreased neutrophils
Clotting screen - DIC is common
LFTs and ECG - do before chemotherapy
Bone marrow aspiration - >20% blast cells, immunophenotyping
Blood film - Auer rods
145
What is the treatment for AML?
Supportive treatment for infections etc
If <60: intense combination chemotherapy, consolidation and transplant
If >60: palliative chemotherapy
146
What is the aetiology of ALL?
Genetic factors e.g. trisomy 21
Environmental factors e.g. high doses of radiation, infection - insulation from common infection in early life leads to abnormal immune responses later in life which increases the risk of ALL
147
What is the pathophysiology of ALL?
There is clonal expansion of lymphoblasts, the majority of cases are B cell origin but can also arise from T cell precursors
148
What is the epidemiology of ALL?
It is the most common cancer in children
149
What are the symptoms of ALL?
Fatigue, dizziness, palpitations, bone and joint pain, dyspnoea, recurrent and severe infections (oral, throat and skin), headaches
150
What are the signs of ALL?
Pallor, tachycardia and systolic flow murmur, hepatomegaly, splenomegaly, fever, petechiae, lymphadenopathy, testicular enlargement, gum hypertrophy
151
What tests are used to diagnose ALL?
FBC - anaemia, thrombocytopenia, neutropenia, increased WCC
Blood film - increase in blasts
Bone marrow aspirate - lymphoblasts >20%
152
What is the treatment of ALL?
Chemotherapy: induction then intense consolidation then maintenance
CNS therapy as patients can have meningeal leukaemia
Stem cell transplant
Allopurinol - prevents tumour lysis syndrome
153
What factors affect the prognosis of ALL?
Age of the patient and whether or not they have the Philadelphia chromosome t(9;22)
154
What is the aetiology of CML?
Unknown - there are no known hereditary, familial, geographic, ethnic or economic associations.
155
What is the pathophysiology of CML?
It is characterised by the Philadelphia chromosome which produces a fusion protein - BCR-ABL which becomes a constitutively active tyrosine kinase. This increases cell division and affects basophils, neutrophils and eosinophils
156
What is the epidemiology of CML?
It only affects adults (40-60)
157
What are the symptoms of CML?
Fatigue, night sweats, weight loss, abdominal fullness/distension, left upper quadrant pain
158
What are the signs of CML?
Splenomegaly, hepatomegaly, enlarged lymph nodes, anaemia, easy bruising, fever, gout
159
What tests are used to diagnose CML?
FBC - leukocytosis, anaemia
U&Es, LFTs, blood film
Bone marrow aspirate - percentage of blasts and basophils
Cytogenetics - Philadelphia chromosome
160
What is the treatment for CML?
Tyrosine kinase inhibitors e.g. imatinib, nilotinib, dasotinib
Stem cell transplant
161
What are some risk factors for CLL?
Trisomies and pneumonia
162
What is the pathophysiology of CLL?
There is malignant monoclonal expansion of B lymphocytes with accumulation of abnormal lymphocytes in the blood, bone marrow, spleen, lymph nodes and liver
163
What is the mean age at diagnosis of CLL?
72
164
What are the symptoms of CLL?
Susceptibility to infection, symmetrically enlarged lymph nodes, abdominal discomfort, bleeding, petechiae, fatigue
165
What are the signs of CLL?
Local or generalised lymphadenopathy, splenomegaly, hepatomegaly, petechiae, pallor, skin infiltration, tonsillar enlargement
166
What tests are used to diagnose CLL?
FBC - lymphocytosis and anaemia
Blood film - smudge cells
Bone marrow aspirate - lymphocytes
Lymph node biopsy
167
What is the treatment for CLL?
Supportive treatment e.g. transfusions, Epo, infection prophylaxis
Watch and wait
Chemotherapy - only for patients with symptomatic disease
Purine analogues
Rituximab (anti-CD20 mAb)
Allogenic stem cell transplant
168
What is the aetiology of immune thrombocytopenic purpura?
Primary - after a viral infection
| Secondary - associated with malignancies e.g. CLL and infections e.g. HIV, hep C
169
What is the pathophysiology of immune thrombocytopenic purpura?
There is immune destruction of platelets. IgG antibodies form to the platelet/megakaryocyte surface, they're removed by the reticuloendothelial system
170
What are the symptoms of immune thrombocytopenic purpura?
Can be asymptomatic to life threatening haemorrhage; petechiae, bruising, nose bleeds
171
What is the epidemiology of immune thrombocytopenic purpura?
Mean age of onset is 5.7 years
172
What are the signs of immune thrombocytopenic purpura?
Bleeding - GI bleeding and haematuria
173
What tests are used to diagnose immune thrombocytopenic purpura?
```
Tends to be a diagnosis of exclusion.
FBC - decreased platelets
Blood film
Bone marrow aspirate
Investigations for a cause if it's secondary e.g. serology for HIV and Hep C
```
174
What is the treatment for immune thrombocytopenic purpura?
Treat the cause of ITP
| Prednisolone, IV IG, IV anti-D IG (in rhesus +ve children)
175
What are some risk factors for DIC?
Sepsis, trauma, surgery, burns, promyelocytic leukaemia, incompatible blood transfusion, dissecting aortic aneurysm
176
What is the pathophysiology of DIC?
There is increased/exposed tissue factor on the endothelium which leads to activation of the clotting cascade. All the clotting factors are used up and so there is bleeding too.
177
What are the symptoms of DIC?
Spontaneous bleeding at venepuncture sites, bleeding, confusion or disorientation, petechiae
178
What are the signs of DIC?
Bleeding from at least 3 unrelated sites, fever, signs of haemorrhage, signs of ARDS, signs of thrombosis, localised infarction or gangrene
179
What tests are used to diagnose DIC?
D-dimer
Prothrombin time - increased
Activated partial thromboplastin time - increased
FBC - decreased platelets and fibrinogen
180
What is the treatment for DIC?
ABCDE
Treat the cause e.g. sepsis
Supportive treatment e.g. platelets, FFP, cryoprecipitate
Heparin where thrombosis predominates
181
What is a complication of DIC?
Organ failure due to infarction
182
What are the risk factors for heparin-induced thrombocytopenia?
Prolonged use of heparin for post-operative thromboprophylaxis
Heparin flushes for maintaining line patency
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What is the pathophysiology of heparin-induced thrombocytopenia?
IgG antibodies form against the platelet-heparin complex forming an IgG/platelet/heparin complex leading to platelet activation and thrombosis
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What are the symptoms of heparin-induced thrombocytopenia?
Symptoms of arterial thrombosis e.g. CVA, MI, acute leg ischaemia
Symptoms of venous thrombosis e.g. DVT, PE
Symptoms of a systemic reaction
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What are the signs of heparin-induced thrombocytopenia?
Enlargement or extension of a previously diagnosed blood clot, HTN, dyspnoea, tachycardia, skin necrosis
186
What tests are used to diagnose heparin-induced thrombocytopenia?
FBC - sharp decrease in platelets 5-10 days after starting heparin
ELISA for anti-heparin antibodies
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What is the treatment for heparin-induced thrombocytopenia?
Immediately stop the heparin, give alternative anticoagulation, never expose the patient to heparin again
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What is the cause of malaria?
It is caused by infection by species of the genus Plasmodium e.g. Plasmodium falciparum, Plasmodium vivax, Plasmodium ovale and Plasmodium malariae
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What is the pathophysiology of malaria?
Sporozites in mosquitos saliva infect the liver where they mature (or lie dormant), they then rupture and release merozites. The merozites then invade the red blood cells and undergo asexual reproduction
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What are the risk factors for developing malaria?
Lack of access to effective treatment e.g. the poor, young children and infants, pregnant women, the elderly and non-immune people
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Where is malaria common?
The tropics and subtropics
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What are the symptoms of malaria?
Fever (often recurring), chills, rigors, headache, cough, myalgia, GI upset
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What are the signs of malaria?
Fever, splenomegaly, hepatomegaly, jaundice, abdominal tenderness, bleeding, dyspnoea, fits, hypovolaemia, AKI, nephrotic syndrome
194
What tests are used to diagnose malaria?
Thick and thin blood smears with Giemsa stain; PCR
FBC - thrombocytopenia, anaemia
LFTs - abnormal
U&Es - hyponatraemia, increased creatinine
Low blood glucose in severe disease
Ill patients: ABGs, CXR, LP, blood cultures, MC&S of the urine and stool, clotting screen
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What is the treatment of malaria?
Non-falciparum: chloroquine or quinine
| Falciparum: Quinine sulfate and doxycycline
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What are some complications of malaria?
Cerebral malaria, ARDS, acidosis, DIC, anaemia, splenic rupture, renal impairment
