Haematology Flashcards
1
Q
What is vitamin b12 used in ?
A
For red blood cell development and also maintenance of the nervous system.
2
Q
Causes of b12 deficiency
A
- pernicious anaemia = most common cause
- post gastrectomy
- vegan diet or a poor diet
- disorders/surgery of terminal ileum (site of absorption)
- Crohn’s: either diease activity or following ileocaecal resection - metformin (rare)
3
Q
Features of b12 deficiency
A
- macrocytic anaemia
- blood film: hypersegmented neutrophils - sore tongue and mouth
- neurological symptoms
- the dorsal column is usually affected first (joint position, vibration) prior to distal paraesthesia - neuropsychiatric symptoms: e.g. mood disturbances
4
Q
Management of vitamin b12 deficiency
A
- if no neurological involvement = 1 mg of IM hydroxocobalamin 3 times each week for 2 weeks, then once every 3 months
- if a patient is also deficient in folic acid then it is important to treat the B12 deficiency first to avoid precipitating subacute combined degeneration of the cord
5
Q
Causes of macrocytic anaemia
A
- vitamin b12 deficiency
- folate deficiency
- Liver disease/alcoholism
- Hypothyroidism
6
Q
Causes of microcytic anaemia
A
- Iron deficiency
2. Thalassemia
7
Q
Causes of normocytic anaemia
A
- Acute blood loss
2. Chronic renal disease
8
Q
Features of macrocytic anaemia
A
- Loss of appetite/weight
- Brittle nails
- Tachycardia
- Diarrhoea
- Fatigue
- Fake skin (lips and eyelids)
- Dyspnoea
- Poor concentration/confusion
- Memory loss
9
Q
Investigations for macrocytic anaemia
A
FBC – check for enlarged RBC and anaemia (MCV(high))
Haematocrit levels = low
Haemoglobin levels
Blood film to identify megaloblastic anaemia
–> hypersegmented polymorphoneucleated cells
LFT
TFT
10
Q
Features of iron deficiency anaemia
A
- Fatigue
- Shortness of breath on exertion
- Palpitations
- Pallor
- Nail changes: this includes koilonychia (spoon-shaped nails)
- Hair loss
- Atrophic glossitis
- Post-cricoid webs
- Angular stomatitis
11
Q
Investigations for iron deficiency anaemia
A
FBC:
- low Hb
- low MCV
- low ferritin
Blood film:
- microcytic hypochromic RBC
- poikilocytosis
- dimorphic RBCs
Endoscopy:
R/O malignancy
12
Q
Management of iron deficiency
A
- Oral ferrous sulphate (fumarate, gluconate)
- Iron-rich diet: this includes dark-green leafy vegetables, meat, iron-fortified bread
- treat underlying cause
13
Q
What is aplastic anaemia?
A
This type of anaemia occurs when your body fails to produce enough new blood cells
14
Q
Features of aplastic anaemia
A
- normochromic, normocytic anaemia
- leukopenia with
thrombocytopenia - features of acute lymphoblastic or myeloid leukaemia
Symptoms:
- fatigue
- SOB
- tachycardia
- pallor
- headache
- fever
- failure to thrive
- jaundice
- murmurs
15
Q
Investigations for aplastic anaemia
A
Blood:
- pancytopenia
- reticulocyte
Bone marrrow biopsy
- fewer cells than normal
16
Q
Management of aplastic anaemia
A
1st line = immunosuppressants e.g. cyclosporine, corticosteroids
Stem cell transplant
Blood transfusions
17
Q
What is sickle cell anaemia?
A
autosomal recessive condition that results for synthesis of an abnormal haemoglobin chain termed HbS
- more common in afro Caribbean decent
18
Q
When do symptoms develop in sickle cell anaemia?
A
don’t tend to develop until 4-6 months when the abnormal HbSS molecules take over from fetal haemoglobin.
19
Q
Features of sickle cell anaemia
A
- Episodes of pain
- swelling of hands + feet
- frequent infections
- Vision disturbances
- delayed growth/puberty
20
Q
Investigations for sickle cell anaemia
A
Definitive diagnosis = haemoglobin electrophoresis
Blood film: crescent moon
21
Q
Sickle cell crisis management
A
- analgesia e.g. opiates
- rehydrate
- oxygen
- consider antibiotics if evidence of infection
- blood transfusion
- exchange transfusion: e.g. if neurological complications
22
Q
Long-term management of sickle cell
A
- hydroxyurea
- increases the HbF levels and is used in the prophylactic management of sickle cell anaemia to prevent painful episodes - pneumococcal polysaccharide vaccine every 5 years
23
Q
What is G6PD deficiency anaemia?
A
Glucose-6-phosphate dehydrogenase (G6PD) deficiency is the commonest red blood cell enzyme defect.
- more common = Mediterranean and Africa
- inherited in an X-linked recessive fashion.
- Many drugs can precipitate a crisis as well as infections and broad (fava) beans
24
Q
Features of G6PD deficiency anaemia
A
- neonatal jaundice
- intravascular haemolysis
- gallstones are common
- splenomegaly may be present
- Heinz bodies on blood films.
- Bite and blister cells may also be seen
25
Investigations for G6PD deficiency anaemia
Diagnosis made using G6PD enzyme assay
26
Drugs causing G6PD deficiency anaemia
1. anti-malarials: primaquine
2. ciprofloxacin
3. sulph- group drugs: sulphonamides, sulphasalazine, sulfonylureas
27
Management of G6PD deficiency anaemia
Treat cause of symptoms
if anaemia has progressed to haemolytic anaemia = blood transfusion
28
Define haemolytic anaemia
a disorder in which red blood cells are destroyed faster than they can be made.
29
Causes of haemolytic anaemia
autoantibodies, medications, and underlying malignancy
hereditiary
30
Features of haemolytic anaemia
```
Pallor
jaundice
Fatigue
SOB
dizziness
splenomegaly
```
31
Investigations for haemolytic anaemia
Bloods:
- low hb
- high MCHC
- increase reticulocyte
Film:
- RBC fragment
- schistocytes
- spherocytes
- reticulocyte
- nucleated RBC
Coombs test= positive
- immune mediated
32
Management of haemolytic anaemia
1. Supportive care:
- Folic acid
- transfusion of packed RBCs
2. Coombs positive:
- remove insult
- corticosteroids
- splenectomy
https://bestpractice.bmj.com/topics/en-gb/98/management-approach
33
What is idiopathic thrombocytopenic purpura (ITP)?
Immune-mediated reduction in the platelet count.
34
Symptoms for ITP
1. petichae, purpura
2. bleeding (e.g. epistaxis)
3. catastrophic bleeding (e.g. intracranial)
35
Blood findings for ITP
Low platelet
36
Management for ITP
1st line = oral prednisolone
2. pooled normal human immunoglobulin (IVIG)
- it raises the platelet count quicker than steroids
- used if active bleeding or an urgent invasive procedure is required
3. splenectomy is less common
37
Pathogenesis of Thrombotic thrombocytopenic purpura
1. abnormally large and sticky multimers of von Willebrand's factor cause platelets to clump within vessels
2. in TTP there is a deficiency of ADAMTS13 (a metalloprotease enzyme) which breakdowns ('cleaves') large multimers of von Willebrand's factor
(symptoms similar to ITP)
38
What is Von Willebrand's disease?
Most common inherited bleeding disorder
| - VWF responsible for platelets sticking together
39
Features of Von Willebrand's disease
Large bruises or bruising easily
Frequent nose bleeds
Bleeding gums
Longer lasting bleeding from cuts
Heavy periods or heavy bleeding after labour
Long lasting tooth bleeds
40
Investigation for on Willebrand's disease
1. prolonged bleeding time
2. APTT may be prolonged
3. factor VIII levels may be moderately reduced
4. defective platelet aggregation with ristocetin
41
Management for Von Willebrand's disease
1. tranexamic acid for mild bleeding
2. desmopressin (DDAVP): raises levels of vWF by inducing release of vWF from Weibel-Palade bodies in endothelial cells
3. factor VIII concentrate
4. Avoid: NSAIDs, Aspirin\|
42
Define Haemophilia
Haemophilia is an X-linked recessive disorder of coagulation.
Haemophilia A = deficiency of factor VIII
Haemophilia B (Christmas disease) = deficiency of factor IX
43
Features of haemophilia
1. haemoarthroses
2. haematomas
3. prolonged bleeding after surgery or trauma
44
Bloods test for haemophilia
1. prolonged APTT
2. bleeding time, thrombin time, prothrombin time normal
3. Bloods : low Hb + haematocrit
45
Management of haemophilia
Prevention:
- Prophylactic infusions of factor VIII given
- Some patients may need this life long
- Antibodies to factor VIII can develop over time making treatment difficult
Avoid NSAIDs
Avoid IM injections
Avoid spots with high risk of trauma
Avoid manual labour
46
What is factor XI disorder?
Haemophilia C
47
Define thrombocytopenia
Deficiency of platelets in the blood
48
Causes of thrombocytopenia
```
Severe:
ITP
DIC
TTP
Haematological malignancy
```
```
Moderate:
heparin induced thrombocytopenia (HIT)
drug-induced (e.g. quinine, diuretics, sulphonamides, aspirin, thiazides)
alcohol
liver disease
viral infection (EBV, HIV, hepatitis)
pregnancy
SLE/antiphospholipid syndrome
vitamin B12 deficiency
```
49
Diagnosis of thrombocytopenia
- History
- Exaination
- FBC
- Peripheral smear
Review differential with above
Then bone marrow biopsy
50
What is thalassaemia?
Inherited blood disorder that causes your body to have less haemoglobin than normal
51
Alpha-thalassaemia vs Beta-thalassaemia
Alpha-thalassaemia: deficiency of alpha chains in haemoglobin
Beta-thalassaemia: Absence of beta globulin chains
52
Features of Beta-thalassaemia major
1. presents in the first year of life with failure to thrive and hepatosplenomegaly
2. microcytic (blood film) anaemia
3. HbA2 & HbF raised
4. HbA absent
53
Management of Beta-thalassaemia major
repeated transfusion
- this leads to iron overload → organ failure
- iron chelation therapy is therefore important (e.g. desferrioxamine)
54
Define acute leukaemia
Uncontrolled proliferation of partially developed white blood cells which build up in the blood
55
Types of acute leukaemia
Acute myloid leukaemia (AML) – affects myeloid cells which fight bacterial infection, defending the body against parasites
Acute lymphoblasitc leukaemia (ALL) – affects lymphocytes which fight viral infections
- children
56
Features of acute lymphoblastic leukaemia (ALL)
1. anaemia: lethargy and pallor
2. neutropaenia: frequent or severe infections
3. thrombocytopenia: easy bruising, petechiae
4. other:
bone pain (secondary to bone marrow infiltration)
splenomegaly
hepatomegaly
fever is present in up to 50% of new cases (representing infection or constitutional symptom)
testicular swelling
57
Investigations for acute lymphoblastic leukaemia (ALL)
Bloods:
- low RBC
- normal WCC
Bone marrow biopsy
58
Treatment for acute lymphoblastic leukaemia (ALL)
Chemotherapy
Radiotherapy
Stem cell transplant
?immunotherapy
59
Define chronic lymphocytic leukaemia (CLL)
Slower growth of white blood cells than acute leukaemia – progresses slowly over many years
60
Features of chronic lymphocytic leukaemia (CLL)
Does not cause any symptoms early on and may only be picked up on a routine blood test
Symptoms can include:
- recurring infections
- anaemia
- bleeding and bruising easily
- high temprature
- nigh sweats
- swollen lymph nodes
- unintentional weight loss
61
Diagnosis of chronic lymphocytic leukaemia (CLL)
Blood test:
- large lymphocyte
- lymphocyte count > 5.0 for > 3 months
Bone marrow biopsy
62
Management of chronic lymphocytic leukaemia (CLL)
1. Wait + watch
| 2. Immunochemotherapy
63
Indications for treatment in chronic lymphocytic leukaemia (CLL)
1. Autoimmune haemolytic anaemia (AIHA) or Immune Thrombocytopenic Purpura (ITP) not responsive to steroid treatment.
2. B Symptoms
3. Symptomatic, splenomegaly or massive lymph nodes (>10cm)
4. Rising ALC ( Absolute Lymphocyte Count) with an increase of more than 50% over a 2-month period or a lymphocyte doubling time (LDT) <6 months (for ALC >30,000)
64
Features of AML
1. anaemia: pallor, lethargy, weakness
2. neutropenia: whilst white cell counts may be very high, functioning neutrophil levels may be low leading to frequent infections etc
3. thrombocytopenia: bleeding
4. splenomegaly
5. bone pain
65
Bloods film for AML
Auer rods (seen with myeloperoxidase stain)
66
Blood findings of AML
DIC
| Thrombocytopenia
67
Investigations for AML
1. Bloods test:
- low RBS
- Normal WCC
- Platelets
2. Bone marrow biopsy
3. Cytogenetic investigations
68
Treatment for AML
1. Chemotherapy
2. Stem cell transplant
3. Regular blood transfusions
69
Define CML
CML is a type of cancer that affects the white blood cells and tends to progress slowly over many years.
70
Features of CML
60-70 years:
1. Anaemia: lethargy
2. weight loss and sweating are common
3. splenomegaly may be marked → abdo discomfort
4. an increase in granulocytes at different stages of maturation +/- thrombocytosis
5. decreased leukocyte alkaline phosphatase
6. may undergo blast transformation (AML in 80%, ALL in 20%)
71
Investigation for CML
1. Blood tests – FBC, CRP/ESR, LTFs, U&Es --> changes in the number and pattern of white blood cells. This suggests the diagnosis of CML.
2. A bone marrow biopsy
72
Management of CML
1. 1ts line = imatinib
2. hydroxyurea
3. interferon-alpha
4. allogenic bone marrow transplant
73
What is lymphoma?
cancer that starts in the lymph glands or other organs of the lymphatic system.
74
Types of lymphoma
1. Hodgkin's lymphoma = is a malignant proliferation of lymphocytes , presence of the Reed-Sternberg cell
2. Non-Hodgkin's lymphoma (every other type of lymphoma that is not Hodgkin's lymphoma)
- Affects B or T- cells
- Further classified as high or low grade
75
Features of Hodgkin's lymphoma
1. lymphadenopathy - painless, non-tender, asymmetrical
2. systemic : weight loss, pruritus, night sweats, fever (Pel-Ebstein)
3. alcohol pain in HL
4. normocytic anaemia, eosinophilia
5. LDH raised
76
Investigation for Hodgkin's lymphoma
1. Biopsy of swollen lymph nodes
- reed-sternberg cells
2. CT, MRI, PET for staging
77
Treatment for Hodgkin's lymphoma
Chemotherapy
Radiotherpay
Stemcell transplant
78
Features of Non- Hodgkin's lymphoma
1. Painless lymphadenopathy (non-tender, rubbery, asymmetrical)
2. Constitutional/B symptoms (fever, weight loss, night sweats, lethargy)
3. Extranodal Disease - gastric (dyspepsia, dysphagia, weight loss, abdominal pain), bone marrow (pancytopenia, bone pain), lungs, skin, central nervous system (nerve palsies)
79
Investigations for Non- Hodgkin's lymphoma
Excisional node biopsy = diagnostic investigation
CT chest, abdomen and pelvis (to assess staging)
80
Management of Non- Hodgkin's lymphoma
- watchful waiting
- chemotherapy
- radiotherapy
Abx prophylaxis for neutropenia
81
Define polycythaemia vera
- myeloproliferative disorder caused by clonal proliferation of a marrow stem cell leading to an increase in red cell volume
- accompanied by overproduction of neutrophils and platelets.
82
Features of polycythaemia vera
- hyperviscosity
- pruritus, typically after a hot bath
- splenomegaly
- haemorrhage (secondary to abnormal platelet function)
- plethoric appearance
- HTN
- low ESR
83
Investigation for polycythaemia vera
1. Bloods:
- raised haematocrit
- raised neutrophils
- raised basophils
- raised platelets
2. JAK2 mutation
3. serum ferritin
4. renal and liver function tests
84
Other investigations for polycythaemia vera
- red cell mass
- arterial oxygen saturation
- abdominal USS
- serum erythropoietin level
- bone marrow aspirate and trephine
- cytogenetic analysis
- erythroid burst-forming unit (BFU-E) culture
85
Management of polycythaemia vera
1. aspirin
- reduces the risk of thrombotic events
2. venesection
1st line to keep the haemoglobin in the normal range
3. chemotherapy
- hydroxyurea
- phosphorus-32 therapy
86
Define Multiple myeloma
Haematological malignancy characterised by plasma cell proliferation.
It arises due to genetic mutations which occur as B-lymphocytes differentiate into mature plasma cells.
87
Features of Multiple myeloma
CRABBI:
1. Calcium
- Hypercalcaemia occurs as a result of increased osteoclast activity within the bones
- This leads to constipation, nausea, anorexia and confusion
2. Renal impairement
- Monoclonal production of immunoglobulins results in light chain deposition within the renal tubules
- This causes renal damage which presents as dehydration and increasing thirst
3. Anaemia
- Bone marrow crowding suppresses erythropoiesis leading to anaemia
- This causes fatigue and pallor
4. Bleeding
- bone marrow crowding also results in thrombocytopenia
- which puts patients at increased risk of bleeding and bruising
5. Bones
- Bone marrow infiltration by plasma cells and cytokine-mediated osteoclast overactivity creates lytic bone lesions
- This may present as pain (especially in the back) and increases the risk of fragility fractures
5. Infection
- a reduction in the production of normal immunoglobulins results in increased susceptibility to infection
88
Investigations for Multiple myeloma
1. Bone marrow aspirate and trephine biopsy
2. Bloods:
- thrombocytopenia
- raised urea & creatinine
- Film: rouleaux formation
3. Serum or urine protein electrophoresis:
- raised concentrations of monoclonal IgA/IgG proteins in serum.
- In the urine = Bence Jones proteins
4. Whole-body MRI = look for bone lesions
89
Management of Multiple myeloma
1. Chemotherapy
2. Immunotherapy (Daratumumab –CD38 antibodies)
3. Bone protectors (Zoledronic Acid)
4. Radiotherapy (plasmacytoma, large bony lesions)
90
Define leucopenia
decrease in the number of white blood cells, which puts a person at risk for infection
91
Blood findings for leucopenia
FBC= lowered WCC
92
Management of leucopenia
1. Antibiotics/antifungals if the cause is infection related
2. Stop any treatment inducing the leucopenia
3. Growth factors and diet changes
