Haematology Flashcards
1
Q
Define anaemia
A
Reduced red cell mass due to reduced haemoglobin concentration or plasma volume
2
Q
Pathophysiology of anaemia
A
Reduced RBC’s -> reduced O2 transport -> tissue hypoxia
3
Q
What 3 compensatory changes happen in anaemia?
A
o Increase tissue perfusion
o Increase O2 transfer to tissues
o Increase red cell production
4
Q
List the 3 main causes of microcytic anaemia
A
o Iron deficiency
o Chronic disease
o Thalassaemia
5
Q
List the 3 main causes of normocytic anaemia
A
o Acute blood loss
o Chronic disease
o Combined haematinic deficiency
6
Q
List 4 causes of macrocytic anaemia
A
o B12/folate deficiency
o Alcohol excess/liver disease
o Hypothyroid
o Haematological – haemolysis, bone marrow failure
or infiltration
7
Q
General symptoms of anaemia
A
Fatigue, faintness, dyspnoea, reduced exercise tolerance, palpitations, headache
8
Q
General signs of anaemia
A
Pale skin, pale mucus membranes, tachycardia
9
Q
List the 3 main causes of iron deficiency anaemia
A
o Blood loss – menorrhagia, GI bleeding
o Poor diet/poverty
o Malabsorption – Coeliac
10
Q
Specific signs of iron deficiency anaemia
A
Koilonychia (spooned nails), atrophic glossitis, angular cheilosis (ulcers at side of mouth)
11
Q
Investigations and diagnosis of iron deficiency anaemia
A
o Blood film – microcytic hypochromic anaemia
o Bloods - low ferritin
o Check Coeliac serology
12
Q
What is the diagnostic result for iron deficiency anaemia?
A
Low ferritin
13
Q
Management of iron deficiency anaemia
A
o Oral iron (ferrous sulphate)
o Improve diet – red meat, spinach
14
Q
What are the common side effects of ferrous sulphate?
A
Nausea, diarrhoea, constipation
15
Q
List the 3 main causes of folate deficiency
A
o Poor diet
o Increased demand – pregnancy
o Malabsorption – Coeliac
16
Q
In which part of the gut is folate absorbed?
A
duodenum/proximal jejunum
17
Q
What investigation confirms a diagnosis of folate deficiency anaemia?
A
Bloods - low folate
18
Q
How is folate deficiency prevented in pregnancy?
A
Folate given during first 12 weeks of pregnancy to prevent spina bifida and anaemia
19
Q
Management of folate deficiency anaemia
A
Oral folic acid for 4 months w/ B12 (unless known to have normal B12 level)
20
Q
Pathophysiology of B12 deficiency anaemia
A
B12 helps synthesize thymidine -> DNA -> slow RBC production in deficiency
21
Q
Pathophysiology of pernicious anaemia
A
lack of intrinsic factor in stomach (autoimmune) -> lack of B12 absorption -> anaemia
22
Q
Causes of B12 deficiency
A
o Dietary - vegan
o Malabsorption - pernicious
o Congenital metabolic disorders
23
Q
Signs and symptoms of B12 deficiency
A
o Lemon tinge to skin – pale and mild jaundice
o Glossitis and angular cheilosis
o Neurological and psychological problems
24
Q
Investigations for diagnosis of B12 deficiency anaemia
A
o Bloods – low B12, high MCV, low haemoglobin
o IF antibodies if pernicious
25
Management of B12 deficiency
Diet, then B12 injections or tablets (hydroxocobalamin)
26
Pathophysiology of thalassaemia
Unbalanced Hb synthesis, under or over production of one globin chain -> unmatched globins precipitate -> damaged RBCs -> haemolysis in marrow
27
Epidemiology of thalassaemia
Common in areas from the Mediterranean to the Far East
28
Causes of thalassaemia
Genetic, inherited
29
Signs and symptoms of thalassaemia
Same as anaemia - fatigue, faintness, syncope ect.
30
Investigations for diagnosis of thalassaemia
o Blood film – microcytic
| o Genetic testing
31
Management of thalassaemia
o Regular, life-long transfusions
o Iron-chelators and ascorbic acid for iron overload
o Stem cell transplant if severe
32
What is the cause of sickle cell anaemia?
Genetic - autosomal recessive deformity of RBC
33
What is the pathophysiology of sickle cell disease?
Autosomal recessive deformity of RBC -> HbS instead of HbA -> HbS polymerises when deoxygenated -> RBCs deform -> sickle cells fragile and haemolyse -> block small blood vessels
34
Which population is sickle cell disease most common in?
African, 1:700 have sickle cell
35
Signs and symptoms of sickle cell disease
o Normal anaemia symptoms
o Vaso-occlusive – hand and feet pain
o Avascular necrosis -> shortened bones
o Recurrent infections (spleen infarction)
o CKD
36
What investigation is used to screen for sickle cell disease in babies?
Heel prick screening, blood film shows sickle cells
37
Management of sickle cell disease
o Hydroxycarbamide if frequent crises -> increases
production of fetal haemoglobin
o Prophylaxis if splenic infarction
o Bone marrow transplant
38
What is intravascular haemolytic anaemia?
Premature breakdown of RBCs in circulation
39
What is extravascular haemolytic anaemia?
Premature breakdown of RBCs in the reticuloendothelial sustem
40
What are the main causes of haemolytic anaemia?
o Acquired – drug-induced, autoimmune, Malaria,
infections
o Hereditary – enzyme defects (G6DP), membrane
defects, haemoglobinopathy (sickle-cell,
thalassaemia)
41
Signs and symptoms of haemolytic anaemia
o Normal anaemia symptoms
o Jaundice, gallstones, leg ulcers, signs of underlying
cause
42
Investigations for suspected haemolytic anaemia
o Blood film
| o Malaria screen
43
Management of haemolytic anaemia
o Folate and iron supplements
o Immunosuppressants if autoimmune
o Splenectomy if hereditary spherocytosis or other
approaches fail
44
Symptoms of DVT
Non-specific, pain, swelling
45
Signs of DVT
Non-specific, tenderness, swelling, warmth, discolouration
46
Risk factors for DVT
o Surgery, immobility
o OC pill, HRT, pregnancy
o Long haul flights/ travel
o Inherited thrombophilia
47
Investigations for suspected DVT
o D-dimer – normal excludes, +ve does not confirm
o Ultrasound compression test proximal veins
o Venogram for calf, recurrence, uncertain
48
Management of DVT
```
o LMW Heparin for min 5 days
o Oral warfarin for 6 months
o Compression stockings, hydration and early
mobilisation
o Treat/seek underlying cause
```
49
What is the target INR range for a patient on warfarin?
2-3
50
Epidemiology of ALL
Most common in age 3-7
51
What cells are affected in ALL?
B and T lymphocytes
52
What is the pathophysiology of ALL?
uncontrolled proliferation of B or T-lymphocytes -> leukaemia cells accumulate -> bone marrow failure and tissue infiltration
53
What are the causes of ALL?
Ionising radiation in pregnancy, Down's syndrome
54
List 3 symptoms of ALL
Anaemia, recurrent infections, bleeding
55
List 3 signs of ALL
Hepatosplenomegaly, CNS involvement (meningism), lymphoadenopathy
56
Investigations for diagnosis of ALL
o Blood film and marrow biopsy – blast cells
o CXR and CT – lymphadenopathy
o Lumbar puncture – check for CNS involvement
o FBC - ↓RBC, ↓platelets, WCC variable
57
Management of ALL
o Support – transfusions/fluids
o Infections – immediate IV antibiotics, prophylaxis
o Chemo – methotrexate
o Bone marrow transplant
58
Epidemiology of CLL
Most common leukaemia, mostly elderly, more common in women
59
Pathophysiology of CLL
Progressive accumulation of malignant functionally incomplete B cells in blood, bone marrow, lymph glands (incl. spleen)
60
What are the causes of CLL?
Genetic mutations, infection, autoimmune, hypogammaglobulinaemia
61
List 4 symptoms of CLL
Anaemic, infection-prone, weight loss, sweats
62
List 2 signs of CLL
Enlarged rubbery nodes, hepatosplenomegaly
63
Investigations for diagnosing CLL
o Blood film – increased lymphocytes, ‘smear cells’
| o Low HB, low neutrophils, low platelets
64
Management of CLL
o Nothing if asymptomatic – monitor
o Chemotherapy
o Monoclonal antibodies (rituximab)
o Bone marrow transplant
65
What cells are affected in CLL?
B lymphocytes
66
What cells are affected in AML?
Myeloblasts
67
Pathophysiology of AML
Neoplastic proliferation of blast cells derived from marrow myeloid elements -> rapidly progresses
68
Epidemiology of AML
Commonest acute leukaemia of adults, incidence increases with age
69
What are the causes of AML?
Long-term complication of chemo, haematological disorders, radiation
70
List 3 symptoms of AML
Anaemia, recurrent infections, bleeding
71
List 3 signs of AML
Lymphadenopathy, hepatosplenomegaly, CNS involvement
72
Investigations for diagnosis of AML
o Blood film – thrombocytopenia, Auer Rods
o Bone marrow biopsy – blast cells
o ↓RBC, ↓platelets, WCC variable
73
Management of AML
o Supportive – transfusions, fluids, prophylaxis
o Human mononuclear leukocytes
o Chemotherapy
o Transplantation
74
What cells are affected in CML?
Basophils, eosinophils and neutrophils
75
Epidemiology of CML
40-60yrs, slight male predominance, rare in childhood
76
What is the main cause of CML?
80% have Philadelphia chromosome
77
What are the symptoms of CML?
Weight loss, tiredness, fever, sweats, gout, bleeding, abdominal discomfort
78
What are the signs of CML?
Hepatosplenomegaly, anaemia, bruising
79
Investigations for diagnosis of CML
o Bloods – high WBC, high urate, low Hb
o Blood film – left shift + basophilia
o Philadelphia Chromosome -> activated tyrosine
kinase
80
Management of CML
o Target molecular therapy – tyrosine kinase inhibitors
– Imatinib (Glivec)
o Chemotherapy if in acute stage
o Stem cell transplant
81
What is the main difference between Hodgkin's and non-Hodgkin's lymphoma?
Reed-Sternberg cells are seen in biopsy of Hodgkin's lymphoma and not in non-Hodgkin's
82
List 3 symptoms of Hodgkin's lymphoma
o Painless lymphoedema
o Sweats
o Weight loss
83
List 3 signs of Hodgkin's lymphoma
o Lymphadenopathy
o Anaemia
o Hepatosplenomegaly
84
Investigations for suspected lymphoma
o Tissue diagnosis – lymph node biopsy
o Bloods – FBC, film, ESR, LFT, LDH
o Imaging – CT/PET, for mets and staging
85
What is the diagnostic feature of Hodgkin's lymphoma?
Reed-Sterberg cells
86
Management of Hodgkins's lymphoma
o ABVD chemo - length determined by stage
o Radiotherapy
o Stem cell transplant
87
Complications of radiotherapy
Increased risk of second malignancies, IHD, hypothyroidism, lung fibrosis
88
Complications of chemotherapy
Myelosuppression, nausea, alopecia, infection
89
Causes of non-Hodgkin's lymphoma
o Immunodeficiency – drugs, HIV
o HTLV-1
o H.pylori
90
Symptoms of non-Hodgkin's lymphoma
o Extranodal disease
o Systemic features – fever, sweats, weight loss
o Infection
o Bleeding
91
Signs of non-Hodgkin's lymphoma
o Anaemia
o Superficial lymphadenopathy
o Bleeding and easy bruising
92
Management of non-Hodgkin's lymphoma
o Low grade – nothing, radiotherapy if localised
o High grade – R-CHOP chemotherapy
o Remission maintained using interferon alfa or
rituximab
93
Definition of myeloma
Malignant proliferation of bone marrow plasma cells, leading to the overproduction of Ig, causing the dysfunction of many organs
94
Which organs are most affected in myeloma?
The kidneys
95
Signs and symptoms of myeloma (CRAB)
• Calcium (hypercalcaemia) - bones, stones, groans,
moans
• Renal impairment -↓urine output, oedema,
dehydration
• Anaemia
• Bone disease - backache and pathological fractures
96
Investigations in suspected myeloma
```
• X-Ray > CT/MRI
• Bone marrow biopsy
• Blood film
• Monoclonal protein band in serum protein
electrophoresis
```
97
How is umsymptomatic myeloma managed?
Watch and wait
98
What is the main treatment for myeloma?
o Chemo/Radiotherapy with autologous stem cell
transplant
o Treat complications
o Supportive treatments
99
List 4 complications of myeloma
o Hypercalcaemia
o Spinal cord compression
o Hyperviscosity
o AKI
100
What supportive treatments are used to manage myeloma?
o Bone Pain: Analgesia and bisphosphonates
o Anaemia: Blood transfusion/Erythropoietin
o Infections: Abx/Regular IV Immunoglobulin infusions
101
Why can myeloma cause hypercalcaemia?
Increased bone resorption and decreased formation -> more calcium in blood
102
Why can myeloma cause anaemia?
Bone marrow infiltration
103
Why might myeloma cause renal failure?
Light chain deposition
104
What is malaria?
Infection by plasmodium transmitted by mosquito (protozoa)
105
Pathology of malaria cycle
1. Bite by infected mosquito
2. Sporozoites in saliva travel to liver to mature (in
hepatocytes)
3. Rupture to release merozoites into the blood
4. Invade RBC and undergo asexual reproduction to
create sporozoites a mosquito can pick up (cycle)
106
List 5 symptoms of malaria
FEVER, chills, cough, myalgia, abdominal discomfort
107
List 3 signs of malaria
Anaemia, thrombocytopenia, hepatosplenomegaly
108
How long is the incubation period for malaria?
A few weeks to 3 months
109
Investigations for suspected malaria
* Urine dip – blood and leukocytes
* Bloods - LFTS, AKI, anaemia
* Thick and thin blood smears
110
Management of malaria
* Fluids and road spectrum antibiotics
* Chloroquine to treat acute infection
* Primiquine to kill hypnozoites
111
What medication is used to treat complicated malaria?
IV artesunate
112
When is an exchange transfusion needed to treat malaria?
When the disease burden is huge
113
What is a complication of malaria?
Multiple organ and failure -> death
114
What causes polycynthaemia vera?
Genetic mutation in JAK2 gene -> increased proliferation of RBC in bone marrow
115
What is polycynthaemia?
An abnormally increased concentration of haemoglobin in the blood, either through reduction of plasma volume or increase in RBC
116
Symptoms of polycynthaemia
• Hyperviscosity – headaches, dizziness, tinnitus,
visual disturbances
• Itching after a hot bath, burning sensation in fingers
and toes
117
Signs of polycynthaemia
Facial plethora, splenomegaly
118
Investigations for suspected polycynthaemia
* Genetic testing for JAK2 gene
* Bone marrow biopsy
* FBC – high haematocrit, haemoglobin and platelets
* Serum EPO – low
119
Management of polycynthaemia
* Venesection in younger low risk
* Hydroxycarbamide in higher risk
* Aspirin
* Treat cause if not genetic
120
What are the most common anticoagulation medications?
Warfarin and NOACs
121
What is warfarin?
Vitamin K antagonist
122
What is the main benefit of using a NOACs instead of warfarin?
Review is needed much less regularly
123
What are the 2 main causes of over-anticoagulation?
o Poor patient compliance
| o New/interacting drugs
124
Symptoms of over-anticoagulation
Bleeding, bruising, melena (black stool), epistaxis (nose bleeds), hematemesis (vomiting blood), haemoptysis (coughing up blood)
125
Investigations for suspected over-anticoagulation
```
o APTT (activated partial thromboplastin time) –
intrinsic pathway
o PTT (prothrombin time) – extrinsic pathway
```
126
What is disseminated intravascular crisis (DIC)?
Generation of fibrin within blood vessels and consumption of platelets/coagulation factors -> secondary activation of fibrinolysis
127
List 4 causes of DIC
Malignancy, septicaemia, trauma, infections
128
Management of DIC
Treat cause, transfusion, activated protein C
129
What is thrombocytopenia?
Deficiency of platelets in the blood
130
List 3 general causes of thrombocytopenia
o Reduced platelet production in bone marrow
o Excessive peripheral destruction of platelets
o Splenomegaly
131
What is immune thrombocytopenia?
Autoimmune destruction of platelets
132
Signs and symptoms of thrombocytopenia
Easy bruising, purpura, epistaxis/menorrhagia
133
Investigations for suspected thrombocytopenia
o Bloods – low platelets
| o May have detection of platelet autoantibodies
134
Management of immune thrombocytopenia
o Splenectomy
o IV IG
o Corticosteroids i.e. prednisolone
o Anti D
135
What is thrombotic thrombocytopenia?
Extensive microvascular clots form in small vessels -> low platelet count and organ damage
136
Management of thrombotic thrombocytopenia
Plasma exchange and immunosuppression
137
What are the 2 types of haemophilia?
A (VIII) and B (IX)
138
What causes haemophilia?
Inherited, X-linked recessive -> more common in males
139
Symptoms of haemophilia
Easy bruising, bleeding
140
Investigations for suspected haemophilia
Normal PTT but prolonged APTT
| Genetic testing
141
What cells are typically seen on a blood film from a patient with thalassaemia?
Target cells
