haem Flashcards
management of post-thrombotic syndrome (swelling, varicose veins, pruritis, heavy calves)
compression stockings
keep leg elevated
the most common inherited clotting bleeding disorder
Von willebrand’s disease
- usually prolonged bleeding after dental extraction
what is the mode of inheritance of von willebrand’s disease
autosomal dominant
there are 3 types of von willebrand disease - what differs between them
Type 1: partial vWF reduction (80%)
Type 2: abnormal vWF
Type 3: complete lack of vWF
what carrier molecule does von willebrand factor help
factor 8
it also promotes platelet adhesion
Prolonged PT
Prolonged APTT
Reduced factor 8
Defective platelet aggregation with ristocetin
Nosebleeds
Heavy periods
What is the diagnosis
von willebrand’s disease
Management of von willebrand’s disease bleeding
Tranexamic acid - for mild bleeding
Desmopressin
Factor 8 concentrate
Cancer patients with VTE treatment and length
6 months of DOAC
Patients taking bone marrow suppression drugs (especially methotrexate) should avoid which eye drops
CHLORAMPHENICOL
can cause aplastic anaemia
In cancer patients who are neutropenic, what type of exam should not be done
PR EXAM
avoid translocation of flora through rectal mucosa
what is neutropenic sepsis defined as
Neut <0.5 * 10^9
And one of:
temp >38 or
septic
What bacteria are the most common cause of neutropenic sepsis
Staphylococcus epidermidis
(Gram +ve coagulase negative)
Management of neutropenic sepsis
Tazocin
If unwell after 48 hours, add meropenem +/- vancomycin
If no response after 4-6 days, investigate for fungal infections e.g. CT-HR
Consider G-CSF
CRAB myeloma
Calcium high
Renal failure
Anaemia
Bony pain and bleeding
in people aged 60 and over with persistent bone pain, particularly back pain, or unexplained fracture, to assess for myeloma what tests should be done
FBC
Calcium
Plasma viscosity
ESR
then if these are positive, do bence-jones protein and protein electrophoresis
+ bone marrow aspiration
+ imaging for bone lesions (MRI whole body)
sickle cell patients need to pneumococcal polysaccharide vaccine how often
every 5 years
long term management of sickle cell anaemia
hydroxyurea - to increase HbF
pneumococcal vaccine every 5 years
tamoxifen therapy effect on the risk of VTE
increases risk of VTE
COCP - which generations have high risk of VTE
3rd gen > 2nd gen
which two hormone cancer medications increase risk of VTE
raloxifene
tamoxifen
DVT investigation: if the scan is negative, but the D-dimer is positive
what should be done
stop anticoagulation
repeat USS scan in 1 week
tamoxifen increases the risk of which cancer
endometrial cancer
which haemophilia accounts for 90% of cases of haemophilia
haemophilia A
what is the mode of inheritance of haemophilia
x-linked recessive
haemophilia A is a deficiency of…
factor 8
haemophilia B (Christmas disease) is a deficiency of..
factor 9
blood tests for haemophilia show what results for:
APTT
Bleeding time
thrombin time
prothrombin time
Prolonged ATT
Normal bleeding time, thrombin time, PT
Which antipsychotic has increased risk of VTE
olanzapine
If investigating a suspected DVT, and either the D-dimer or scan cannot be done within 4 hours, what treatment is given
Start a DOAC
If a 2-level DVT Wells score is ≥ 2 points then what should be done next
ultrasound scan WITHIN 4 hours
If a 2-level DVT Wells score is 1 point or less then what should be done next
D-dimer test
If positive, then do USS within 4 hours
Sickle cell patients with low Hb, low reticulocytes is usually due to what infection
PARVOVIRUS infection
This tends to cause aplastic crisis due to bone marrow suppression
Sickle cell anaemia
Parvovirus infection
Low Hb
Low reticulocytes
What type of crisis is this?
Aplastic crisis
4 types of sickle cell crisis
- Thrombotic i.e. vasoocclusive /painful
- Acute chest
- Anaemic
- aplastic
- sequestration - Infection
Thrombotic crisis in sickle cell anaemia (aka painful or vasocclusive crisis) is triggered by what 3 things
- dehydration
- infection
- low O2 e.g. high altitude
Acute chest syndrome is a type of sickle cell crisis. Vasoocclusion happens where?
Within the pulmonary microvasculature
Leads to infarction in lung tissue
Shortness of breath
Chest pain
Pulmonary infiltrates on CXR
Low pO2
What type of sickle cell crisis is this?
Acute chest syndrome
What is the most common cause of death after childhood in sickle cell disease patients
Acute chest syndrome
Increased reticulocyte count
Worsening anaemia
What type of sickle cell crisis is this?
Sequestration crises
Sickling within organs e.g. spleen or lung worsens the anaemia
What is the difference in reticulocytes between sickle cell anaemia - anaemic crises
(a) aplastic
(b) sequestration
(a) aplastic - low reticulocytes (bone marrow suppression)
(b) sequestration - high reticulocytes
Immune thrombocytopenia (ITP) is an immune-mediated reduction in platelet count.
Antibodies are directed against what
Abs against Glycoprotein IIb/IIIa or Ib-V-IX complex
Children with ITP usually have acute thrombocytopenia (low platelets) that follows what
infection or vaccination
(adults tend to have a more chronic condition)
What does FBC show in immune thrombocytopenia (ITP)
Isolated low platelets
First line management of ITP
Oral prednisolone
What is Evan’s syndrome
ITP (low platelets) in association with autoimmune haemolytic anaemia
Management of ITP - three options
- Oral pred - 1st line
- Pooled normal human immunoglobin (IVIg) - acts faster than steroids so if active bleeding or urgent procedure needed
- Splenectomy - less common
3 causes of high APTT
- Heparin therapy
- Haemophilia (A = low factor 8, B low factor 9)
- Antiphospholipid syndrome
Chronic lymphocytic leukaemia (CLL) is caused by monoclonal proliferation of well-differentiated lymphocytes which are usually what cell
B-cells (99%)
What is the most common form of leukaemia in adults
chronic lymphocytic leukaemia (CLL)
FBC shows what in chronic lymphocytic leukaemia (CLL)
Lymphocytosis (high lymphocytes)
Anaemia
Thrombocytopenia
Blood film shows smudge/smear cells - what is likely diagnosis
Chronic lymphocytic leukaemia (CLL)
What is the key investigation for chronic lymphocytic leukaemia - and what does it look for
Immunophenotyping
Uses Abs specific for CD5, CD19, CD20, CD23
Antiphospholipid syndrome - in pregnancy what are the 6 issues that can occur
- recurrent miscarriage
- IUGR
- pre-eclampsia
- placental abruption
- pre-term delivery
- VTE
management of antiphospholipid syndrome in pregnancy
- Low dose aspirin once pregnancy is confirmed
- LMWH once foetal heart seen on USS - discontinued at 34 gestation
When is LMWH started and stopped in pregnancy with antiphospholipid syndrome
Starts: when foetal heart on USS
Stops: 34 weeks gestation
G6PD deficiency is triggered by which types of drugs
Sulph- drugs: e.g. sulphonamides
Sulphasalazine
Sulfonylureas - gliclazide
Ciprofloxacin
Anti-malarials - primaquine
AND BROAD FAVA BEANS!
What is the inheritance of Glucose-6-phosphate dehydrogenase (G6PD) deficiency
X-linked recessive
What is the pathophysiology of G6PD deficiency
Low G6PD
Reduced NADPH production
NADPH helps to convert oxidized glutathine back to reduced form (which would protect RBCs from oxidative damage)
Increases RBC oxidative stress
What type of anaemia occurs with G6PD deficiency
Intravascular haemolysis
Haemolytic anaemia
Neonatal jaundice occurs
What is seen on blood films with G6PD deficiency
Heinz bodies
Bite and blister cells
How do you diagnosis G6PD deficiency
G6PD enzyme assay
Check levels around 3 months after acute episode of haemolysis
What is seen on blood film for:
(a) G6PD deficiency
(b) hereditary spherocytosis
(a) Heinz bodies (bite and blister cells also may be seen)
(b) Spherocytes
Pernicious anaemia have which antibodies
Intrinsic factor antibodies - used for diagnosis
Gastric parietal cell antibodies
What is the mechanism of action in pernicious anaemia of:
(a) Intrinsic factor antibodies
(b) Gastric parietal cell antibodies
(a) Intrinsic factor Abs bind to intrinsic factor blocking the vitamin B12 binding site
(b) Gastric parietal cell Abs reduce IF production and reduce acid production, leads to atrophic gastritis.
Blood film findings with pernicious anaemia
Megaloblastic macrocytic anaemia
Hypersegmented polymorphs
NICE specifically state that we should not use QRISK2 for type 1 diabetics. Instead, the four following criteria are used:
Older than 40 years, or
Has had diabetes for >10 years or
Has established nephropathy or
Has other CVD risk factors
n.b. start 20mg atorvastatin for these EVEN if their lipid screen is normal!
Steven Johnson’s syndrome - Nikolsky sign shows …
In erythematous areas, blisters and erosions appear when the skin is rubbed gently
Lymphadenopathy occurs in 75% of Hodgkin’s lymphoma. Where are the glands most commonly?
Neck
i.e. cervical/ supraclavicular
> axillary
inguinal
Painless
Non-tender
Asymmetrical
Hodgkin’s or non-Hodgkin’s lymphoma?
Hodgkin’s lymphoma
Pel-Ebstein fever relates to which condition
Hodgkin’s lymphoma
What blood results are seen in Hodgkin’s lymphoma
Normocytic anaemia
High eosinophils
High LDH
Lymph node biopsy for Hodgkin’s lymphoma shows which type of cells
Reed-Sternberg cells
these are diagnostic
If you are suspicious of leukaemia in ages 0-24 years, e.g. pallor, fatigue, infections, what is an important test to do and timeframe?
FBC within 48 hours
patients with inherited haemolytic anaemias e.g. hereditary spherocytosis and G6PD usually have jaundice alongside what other abdo presentation?
gallstones!
patients with hereditary spherocytosis can be diagnosed without any special tests on the basis of what criteria..
- Family history
- Clinical features
- Lab results e.g. Spherocytes,
High MCHC,
High reticulocytes
to diagnose hereditary spherocytosis when the diagnosis is unsure, what two tests can be done
EMA binding test
cryohaemolysis test
for ATYPICAL presentations of hereditary spherocytosis, what test can be done
electrophoresis analysis of erythrocyte membranes
management of hereditary spherocytosis acute crises
supportive treatment
transfusion if neccessary
management of hereditary spherocytosis acute crises is supportive with transfusion if needed.
what is used for longer term treatment?
folate replacement
splenectomy
which lymphoma has more pain when drinking alcohol
hodgkin’s lymphoma
what are 4 systemic B symptoms of lymphoma
Weight loss
Pruritis
Night sweats
Fever (Pel-ebstein)
After correcting iron deficiency anaemia, iron replacement should be continued for how long, and when should it next be checked?
3 months then stopped
Then monitor FBC every 3 months for one year
What is the profile of the following in iron def anaemia:
Serum iron
Ferritin
TIBC/transferrin
Trans sats
Serum iron - low
Ferritin - low
TIBC/transferrin - high
Trans sats - low
anisopoikilocytosis (red blood cells of different sizes and shapes)
target cells
‘pencil’ poikilocytes
what is the condition
iron deficiency anaemia
Patients with iron def anaemia - it is important to refer for 2ww endoscopy to rule out GI cancer. Who is referred?
Post-menopausal women with Hb <10
Men Hb <11
Age over 60 with IDA
Management of iron def anaemia
Oral ferrous sulfate
Take for full 3 months even if it has been corrected
Then recheck - if normal then stop iron
Monitor FBC every 3 months for one year thereafter
Acute myeloid leukaemia FBC profile
Low Hb - anaemia
Low platelets
Low neutrophils
(blood fim - bilobed large mononuclear cells)
Poor prognostic features of acute myeloid leukaemia
> 60 years
> 20% blasts after first course of chemo
Genetics: Chr 5 or Chr 7 deletions
Acute promyelocytic leukaemia M3 is associated with which Chr translocation
t(15;17)
PML and RAR-alpha genes is seen with which cancer
Acute promyelocytic leukaemia M3 (type of AML)
Auer roads (seen with myeloperoxidase stain) is associated with which cancer
Acute promyelocytic leukaemia M3 (Type of AML)
t(15;17) is seen with what condition
Acute promyelocytic leukaemia M3 (Type of AML)
There are 8 types of acute myeloid leukaemia - what classification is it
French-American-British (FAB)
M0 - M7
vitamin B12 is absorbed after binding to …
intrinsic factor
secreted from parietal cells in the stomach
and is absorbed in terminal ileum
what is the most common cause of vit B12 def
pernicious anaemia
vit B12 deficiency can cause neurological symptoms - what is usually affected first
dorsal column
i.e. joint position, vibration
prior to distal paraesthesia
management of vitamin B12 deficiency if no neurological involvement
1mg IM hydroxocobalamin
3 times each week for 2 weeks
Then once every 3 months
Drug induced pancytopenia from bone marrow suppression can occur from some medications e.g. carbimazole, sulphonylureas, trimethoprim, chloramphenicol, carbamazepam etc
What symptoms may the patient develop
Sore throat
Nose bleeds/menorrhagia
Pancytopenia
Primary causes of polycythaemia (high Hb)
polycythaemia rubra vera
Secondary causes ofpolycythaemia (high Hb)
COPD
High altitude
Obstructive sleep apnoea
Excess EPO: haemangioma, hypernephroma, hepatoma, fibroids
To work out if it is TRUE polycythaemia versus relative (i.e. dehydration), what studies are used
Total red cell mass studies
In true polycythaemia, RBC mass increases:
Males >35
Women >32
polycythaemia rubra vera is primary polycythaemia. what gene has the mutation
JAK2 gene mutation
How to distinguish between primary and secondary true polycythaemia causes
Primary polycythaemia - reduced EPO
Secondary polycythaemia - increased EPO
What is the most common cause of thrombophilia (clotting) disorder
Factor V Leiden
i.e. activated protein C resistance
Factor V Leiden is a disorder that leads to what clotting issue
Activates protein C resistance
5 inherited clotting disorders
- Factor V Leiden
- Prothrombin gene mutation
- Antithrombin 3 deficiency
- Protein C deficiency
- Protein S deficiency
Acquired clotting disorder
Antiphospholipid syndrome
which antibiotic leads to jaundice and haemolysis in G6PD
CIPROFLOXACIN
3 key changes in NICE 2020 VTE guidelines
- Use of DOACs as first line
- DOACs should be used for VTE in active cancer patients
- Routine cancer screening is NOT recommended after VTE diagnosis
Patient with VTE with renal impairment that is severe e.g. eGFR <15, what should be given to treat it?
LMWH
Unfractionated heparin
Or LMWH with VKA
Unprovoked VTE treatment duration
6 months
Provoked VTE treatment duration
3 months
hereditary spherocytosis inheritance mode
autosomal dominant
what antiepileptic drug interacts with DOACs
carbamazepine
In myeloma, what is seen on peripheral blood film?
Rouleaux formation
Protein electrophoresis shows raised concentrations of what in myeloma
Monoclonal IgA/IgG proteins
X-rays: ‘rain-drop skull’
what is this seen in?
Myeloma
X-ray - pepperpot skull
what is this seen in?
Primary hyperparathyroidism
What 8 symptoms in a person aged from 0-24 years prompt an urgent FBC within 48 hours to look for leukaemia?
- Pallor
- Persistent fatigue
- Unexplained fever
- Persistent infections
- Lymphadenopathy
- Bone pain
- Unexplained bruising
- Unexplained bleeding
What does FBC show in IDA
Hypochromic microcytic anaemia
