haematological diseases Flashcards
what is this abbreviation FBC
full blood count which includes all of the values below
red blood cells, white blood cells, platelets, haematocrit, mean cell volume
what is the abbreviation RBC
red blood cells or RCC red cell count
what is the abbreviation WCC
white cell count
what is the abbreviation PLT
platelets
what is the abbreviation
haematocrit
what is haematocrit
ratio of cells to liquid in the blood
what is the abbreviation MCV
mean cell volume - volume of the average red blood cell
what blood change is in anaemia ?
low haemoglobin
what blood change is in leukopenia
low WCC
what blood change is in thrombocytopenia?
low platelets
what blood change is in pancytopenia?
all cells reduced
one of theses change in the blood is due to what most often?
anaemia
leukopenia
thrombocytopenia
pancytopenia
environment
multiple of these changes in the blood is due to what most often?
anaemia
leukopenia
thrombocytopenia
pancytopenia
bone marrow failure
what blood change is in polycythaemia?
raised haemoglobin (opposite of anaemia)
what blood change is in leukocytosis?
raised WCC (normal if fighting infection)
what blood change is in thrombocythaemia?
raised platelets - can be a reactive change to damage in the body
one of these changes in the blood is what?
polycythaemia
leukocytosis
thrombocythaemia
reactive or pre-neoplastic
multiple of these changes in the blood is likely what?
polycythaemia
leukocytosis
thrombocythaemia
pre-neoplastic, myelodysplasia precancerous
the myeloid stem cell group produces what cells? 5
○ Red cells
○ Platelets
○ Neutrophils
○ Basophils
○ Eosinophils
Monocytes
the lymphoid stem cell group produces what cells? 3
B cells
T cells
NK cells
Blood cancer can derive from either the lymphoid or the myeloid cell line and this determines its ?
behaviour
definition of leukaemia
Neoplastic proliferation of white cells, usually disseminated
definition of lymphoma
○ Neoplastic proliferation of white cells, usually a solid tumour
what is porphyria?
- Abnormality of haem metabolism
- Acute intermittent porphyria’s can be triggered by ? including ?
medicines
LA
clinical effect of porphyria? 3
Photosensitive rash
Neuropsychiatric disturbance in acute attacks
Hypertension and tachycardia
how do u prevent porphyria in pt?
find out trigger
3 causes of anaemia
reduced production
increased losses
increased demand
2 causes of reduced production
Reduction in the number of normal blood cells due to marrow failure
Normal blood cells but reduced haemoglobin
2 causes of normal blood cells but reduced haemoglobin
haematinic deficiency - iron (ferritin), folate, vit B12
Abnormal globin chains that can’t be manufactured into haemoglobin
what are the haematinics? 3
things used to produce red cells
iron - ferritin
folate
Vit b12
Iron absorption
Haem iron absorbed in a ?? in the ? wall
Non-heme iron has to go through a conversion of ? to ? then through a ? in the small intestine wall
Therefore simpler for body to deal with ? iron than ? iron
Once iron in the cell it is stored as ?, which is then passed out into the ? and carried away for ?
heme transporter
small intestine
Fe3+
Fe2+
transporter
heme
non-heme
ferritin
blood
recirculation
name 2 diseases that cause reduced iron absorption
achlorhydria
coeliac disease affecting the small intestine
how does achlorhydria reduce iron absorption?
lack of stomach acid stops iron conversion to haem iron
name 4 diseases that cause iron loss
Gastric erosions and ulcers
Inflammatory bowel disease - Crohns and uclerative colitis
Bowel cancer - colon and rectal
Haemorrhoids
causes for folate deficiency? 2
Lack of intake
Absorption failure - jejunal disease - coeliac disease
folate deficiency can cause what in a foetus?
neural tube defects
Absorption of vit b12
? cell secrete ? which binds to Vitamin B12. absorbed in the ? through a specific ?.
Problem with intrinsic factor secretion, dietary B12 or disease of the terminal ileum then absorption of vitamin B12 orally isn’t possible. Then ? are given.
parietal
intrinsic factor
ileum
transporter
injections
causes of deficiency of vitamin B12? 3
Dietary lack
Lack of intrinsic factor - pernicious anaemia, gastric disease
Disease of terminal ileum - Crohn’s disease
what 2 things can cause abnormal global chains that can’t be manufactured into haemoglobin?
thalassaemia
sickle cell anaemia
clinical affects of thalassaemia? 5
Chronic anaemia
Marrow hyperplasia - bigger to make more haemoglobin
Splenomegaly - spleen recycles RBCs as they are abnormal so have shorter lifespans
Cirrhosis - excess iron
Gall stones - haem reprocessing causes more chemicals to pass the liver
management of thalassaemia 2
Blood transfusion of normal RBCs - issue of iron overload - cirrhosis
Prevent iron overload risk from blood transfusions
what is sickle cells anaemia?
Abnormal globin chains that function normally at standard O2 environments but at low O2 environments change shape which prevents RBC passing through capillaries, which blocks the circulation causing ischaemia to the tissue
The size of the red blood cells (MCV) can show the cause of the anaemia
Raised cell volume indicates what cause?
vit B12 or folic acid deficiency
The size of the red blood cells (MCV) can show the cause of the anaemia
small red blood cells indicated what cause?
iron deficiency or thalassemia
key things to learn with anaemia
anaemia is reduced haemoglobin in the blood from the normal values of the population
haemaglobin issues can be from an inability to make haem or an inability to make the correct globin chains
anaemia is nothing to do with RBC numbers
the size of the red blood cells (MCV) can give a clue into the cause of the anaemia
anaemia diagnosis
haemoglobin levels show what?
the degree of anaemia
anaemia diagnosis
red cells count shows what?
if there is a normal or reduced number of red cells -> cell deficiency or haemoglobin formation deficiency
anaemia diagnosis
haematocrit shows what?
shows if there are fewer cells in the blood -> cell deficiency
anaemia diagnosis
mean cell volume shows what?
different deficiency pictures to work out the deficiency
anaemia with normal red blood cells is caused by what?
bleeding - GI bleeding
anaemia cell terminology - MCV
what does microcytic mean and what does it indicate the cause is?
small RBC
iron deficiency, Thalassemia
anaemia cell terminology - MCV
what does macrocytic mean and what does it indicate the cause is?
large RBC
B12/folate deficiency
anaemia cell terminology - MCV
what does normocytic mean and what does it indicate the cause is?
normal RBC size
bleed, renal, chronic disease
anaemia cell terminology
what does hypo chromic mean?
appear pale under microscope due to less haemoglobin
anaemia cell terminology
what does ansiocytic mean?
very big cells and very small cells
what are reticulocytes?
Almost mature RBCs released early into circulation to replace losses
They still have organelles so are larger and raise the MCV
anaemia signs and symptoms 7
Signs
Pale
Tachycardia
Enlarged liver/spleen
Symptoms
Tired and weak
Dizzy
Shortness of breath
Palpitations due to high heart rate
dental signs of anaemia? 4
pale mucosa
smooth tongue
angular cheilitis
beefy tongue
smooth tongue is a sign of what haemotinic deficiency?
iron deficiency
angular cheilitis is a sign of what haemotinic deficiency?
iron deficiency
beefy tongue is a sign of what haemotinic deficiency?
vit B12 deficiency
what investigations are done for anaemia? 5
full blood count - ferritin, folate, vitamin b12
GI blood loss causing anaemia
- faecal occult blood test
- endoscopy
renal function
bone marrow examination
what is the treatment for anaemia? 4
treat the cause
replace haematinics - iron, folate, vit b12
transfusions for production failure
erythropoietin if production failure due to renal disease (kidneys)
how is the haemotinic iron replaced?
FeSO4 200mg for 3 months
how is the haemotinic vit B12 replaced?
1mg IM vit B12 x 6 then 1mg/2months
how is the haemotinic folate replaced?
5mg folic acid daily
How to differentiate deficiency from bleeding
If bleed RCC ?, MCV is ?, HCT is ?
Deficiency RCC is ?, MCV is ? or ?, HCT is ?
low
normal
low
low
micro
macro-cytic
normal
normal haemoglobin range in male and female
male 130-180, female 115-165 g/litre
normal RCC range in male and female
male 4.5-6.5, female 3.8-5.8 x 1012/litre
normal WCC range
3.6-11 x 109/litre
normal MCV range
80-100 fl
normal haematocrit range
male 0.4-0.54, female 0.37-0.47 L/L
normal PLT range
140-400 x 109/L
what are the ferritin levels in iron deficiency anaemia?
<30 micrograms/litre
folate/vitamin B12 anaemia results in micro or macro cytic anaemia?
macrocytic anaemia -> large RBCs
common causes of blood loss that leads to anaemia? 5
Ulcers
Polyps
Cancers in large intestine - colon, rectal
Kidney/bladder tumours
Menstrual bleeding
name 2 types of anti thrombotic medication
Injectable anticoagulants
oral anticoagulants
are injectable anticoagulants a dental issue?
no
are oral anticoagulants a dental issue?
yes
oral anticoagulants interfere with what?
the clotting cascade
what are the two types of oral anticoagulation?
coumarin and non-coumarin
is warfarin a coumarin or non-coumarin?
coumarin
mechanism of action for warfarin
vitamin K antagonist - slow onset, initially hypercoagulable
warfarin
treat normally if INR below ?, delay treatment if above ?
INR measured within ?, preferably ?, prior to treatment
4
4
72hrs
24hrs
drugs to avoid prescribing if pt taking warfarin 3
Aspirin, NSAIDS
Azole antifungal drugs
name a novel oral anticoagulant
apixaban
NOAC apixaban mechanism of action
Factor Xa inhibitor
for the NOAC apixaban what should u do for low risk procedures? 3
don’t interrupt medication
treat early in morning
treat in stages
for the NOAC apixaban what should u do for higher risk procedures? 2
miss/delay morning dose, restart after treatment
do NOACs require monitoring?
no
how often should a pt take apixaban?
twice daily
drugs to avoid prescribing for NOACs 3
NSAIDs
Macrolide antibiotics -> erythromycin and clarithromycin
Carbamazepine
mechanism of action for anti platelet medication
○ Interfere with platelet number or function
name 2 antiplatelet medications
aspirin
clopidogrel
should u interrupt anti platelet medications for treatment
no but treat in stages
If two antiplatelet drugs are taken without aspirin then speak to doctor to stop one ? days prior to surgery
7
drugs to avoid prescribing if pt on antiplatelet medication 6
NSAIDs
Erythromycin
Fluconazole
Clarithromycin
Carbamazepine
Omeprazole
indications for anticoagulation requirement
Atrial fibrillation
Deep venous thrombosis
Heart valve disease
Mechanical heart valves
Thrombophilia
inherited bleeding disorder is an acquired defect that affects what?
the coagulation of the blood
what three things can inherited bleeding disorders affect?
coagulation cascade
platelets
a combined deficiency
how can bleeding disorders affect the coagulation cascade
reduction in one or more of the coagulation factors or control proteins
what is haemophilia?
too much clot formed
does haemophilia affect males or females?
males as on X chromosome
what are the two types of haemophilia?
haemophilia A and B
haemophilia A is caused by a deficiency in what?
Factor VIII deficiency
for bleeding disorders what is deemed moderate to severe?
<0.09 iu/ml of the factor
for bleeding disorders what is deemed mild?
0.1-0.05 iu/ml of the factor
how is severe haemophilia A managed?
recombinant factor VIII
how is mild haemophilia A managed?
Drug DDAVP
Oral tranexamic acid
how does the drug DDAVP help mild haemophilia A?
desmopressin temporarily releases factor VIII bound to endothelial cells to boost levels and clotting ability
how does oral tranexamic acid help haemophilia A?
prevent fibrinolysis - clot breakdown
what is another name for haemophilia B?
christmas disease
what is haemophilia B caused by a deficiency in?
Factor IX deficiency
how is severe/moderate haemophilia B managed?
recombinant factor IX
how is mild/carriers haemophilia B managed?
recombinant factor IX
why are the drugs DDAVP and tranexamic acid not used to treat haemophilia B?
No other alternatives as not bound to endothelial cells and tranexamic acid not enough
management of severe haemophilia (<0.9iu/ml) in dental setting
Dental unit attached to haemophilia centre
Non risk treatments in primary care e.g. pros
Observe pt overnight after extractions and surgery
management of mild/carriers haemophilia (0.1-0.5iu/ml)
Shared between public dental service and GDP
Extractions and surgery done in haemophilia unit and observed for 2-3hrs
how should a pt with severe/moderate haemophilia be managed after extraction/surgery?
Observe pt overnight after extractions and surgery
how should a pt with mild/carrier haemophilia be managed after extraction/surgery?
Extractions and surgery done in haemophilia unit and observed for 2-3hrs
what should u avoid with pt with haemophilia?
LA blocks and lingual infiltration
what are coagulation factor inhibitors
antibodies that form in response to recombinant factor VIII and IX.
why is it important to give recombinant factor VIII and IX as infrequently as possible
each time they are given an antibody response occurs producing coagulation factor inhibitors. the next dose will need to be much greater to overcome these
what is thrombophilia?
too much clot formed
Clot formation ability greater than clot breakdown leading to excessive stable clot formed inappropriately
what can thrombophilia lead to?
pulmonary embolism
name the two ways in which people can get thrombophilia?
inherited hyper coagulation
acquired hyper coagulation
what can cause inherited hyper coagulation (thrombophilia)?
Protein C and S deficiency, factor V Leiden, antithrombin III deficiency
what can cause aqcuired hyper coagulation?
Oral contraceptives, surgery, trauma, cancer, pregnancy, immobilisation, antiphospholipid syndrome (lupus anticoagulants)
what is thrombocytopenia?
Reduced platelet numbers
what is thrombocythaemia?
Increased platelet numbers but platelets are defective
people with thrombocythaemia are given what to prevent clot formation?
aspirin
describe what a qualitative bleeding disorder that affects platelets
Normal platelet number but abnormal function
dental care for pt with platelet disorders
No additional precautions if platelet count is greater than ? and no functional issues
Special care is required for:
Extractions, minor oral surgery, periodontal surgery, biopsies
100x10*9 per litre
what is the most common bleeding disorder that is a combined deficiency?
von willebrand’s disease
what is von willebrands disease?
deificiency in factor VIII and reduced platelet aggregation
Defective von Willebrand’s factor on platelets interacts badly with factor VIII so poor clot activation by platelets
what is the management for severe/moderate von willebrand’s disease (<0.09iu/ml)
DDAVP - release factor VIII bound to endothelial cells temporarily
what is the management of von willebrand’s disease for mild/carriers? (0.1-0.5iu/ml)
Tranexamic acid - inhibit fibrinolysis
red blood cells have antigens on their surface. these form 2 types of systems for classifying blood. what are they?
ABO system -> pt can be A,B O or AB
d system (rhesus) -> + or -
name 2 indications for blood transfusion
blood loss
production failure
why should blood transfusions be avoided if possible? 2
due to risk of transfusion reactions and transmission of infection
what antibodies do people with group A blood have?
anti-B antibodies in their plasma
what antibodies do people with group B blood have?
anti-A antibodies in their plasma
what antibodies do people with group AB blood have?
no antibodies
what antibodies do people with group O blood have
anti-A and anti-B antibodies in their plasma
what antigens do each group have on the surface of their red blood cells?
A
B
AB
O
A=A
B=B
AB= A and B
0= none
name 3 possible complications of blood transfusions
Red blood cell lysis - fever, jaundice, death
Fluid overload - heart failure
Transmission of infection
define lymphoma
is clonal proliferation of lymphocytes arising in a lymph node or associated tissue
how is leukaemia different from lymphoma?
leukaemia is a disseminated tumour of white cells which is within circulation
lymphoma is clonal proliferation of lymphocyte arising in a lymph node or associated tissue
symptoms of lymphoma
Swelling/lump in the face or neck
Fever
Excessive sweating at night
Weight loss
Loss of appetite
Breathlessness
Weakness
what are the 4 stages of lymphoma?
- Single lymph node region or site
- Two or more sites on one side
- Both sides of the diaphragm
- Diffuse involvement
name 2 types of lymphoma?
Hodgkin’s lymphoma
Non-Hodgkin lymphoma
clinical presentation of hodgkin’s lymphoma
Painless lymphadenopathy
Fever, night sweats, weight loss, itching, infection
cause of non-hodgkin lymphoma 3
infection
autoimmune disease
immunosuppression
clinical presentation of non-hodgkin lymphoma
Extra-nodal disease - oropharyngeal involvement
Symptoms of marrow failure
which has a poorer prognosis, Hodgkin or non-hodgkin lymphoma?
non-hodgkin lymphoma
what is a malignant myeloma?
- Malignant proliferation of plasma cells
name 3 features of multiple myeloma
Monoclonal paraprotein in the blood/urine
Lytic bone lesions - fracture and pain
Excess plasma cells in marrow - marrow failure
what is the treatment of multiple myeloma? 2
Biologic monoclonal antibodies
Bone turnover suppression medicines e.g. bisphosphonate
name the 4 phases of treatment for haematological malignancy
induction
remission
maintenance and consolidation
relapse
what happens in the induction phase of treatment for haematological malignancy
high dose chemotherapy to remove disease and return to normal state
what happens in the remission phase of treatment for haematological malignancy?
pt has normal bone marrow and no evidence of disease
what happens in the maintenance and consolidation phase of treatment for haematological malignancy?
pt requires low level treatment to maintain at a normal state
what happens in the relapse phase of treatment for haematological malignancy?
disease comes back again and the process of induction and remission will need to be repeated
chemotherapy targets what kind of cells?
cells with a high turnover rate
remember
Chemotherapy targets cells with a high turnover rate
Radiotherapy - cytotoxic effect of ionising radiation to target tissues
Monoclonal antibodies have specific cancer antigens
Haemopoietic stem cell transplant
Allogenic transplant is from who?
a donor
Haemopoietic stem cell transplant
Autologous transplant is from who?
the pt
Haemopoietic stem cell transplant
why is it usually stem cells from the blood rather than bone marrow?
don’t need to remove all bone marrow
Haemopoietic stem cell transplant is a high risk procedure.
what are the risks? 4
Infection
graft vs host disease
graft failure
total marrow failure
leukaemia is what cella lineage?
either myeloid or lymphoid
The earlier in the cell line it turns neoplastic the potentially more ? the malignancy
aggressive
Haematological malignancy
Usually starts with a ?? which switches off a ??? or switches on an ?. This results in ?? and cancer when there is ??, loss of ? and loss of normal ?/?
DNA mutation
tumour supressor gene
oncogene
clonal proliferation
uncontrolled proliferation
apoptosis
functions
products
name the types of leukaemia? 5
acute or chronic
Lymphocytic, lymphoblastic or myeloid describe the point in the cell lines or cell type at fault
what is leukaemia?
describes a group of cancers of the bone marrow which prevent normal manufacture of the blood
what does leukaemia result in?
anaemia
infection - neutropenia
bleeding - thrombocytopenia
what is acute lymphoblastic anaemia?
- Large numbers of immature lymphocytes
acute lymphoblastic anaemia typically occurs in what type of people?
children
clinical presentation of lymphoblastic anaemia 5
Lymphadenopathy, tissue infiltration, fever, sweats, malaise
acute myeloid leukaemia typically occurs in what type of ppl?
elderly
clinical presentation of acute myeloid leukaemia
Lymphadenopathy, tissue infiltration, fever, sweats, malaise
Chronic lymphocytic leukaemia
is ? cell based?
B
what is the clinical presentation of chronic lymphocytic leukaemia?
Mostly asymptomatic and discovered on routine blood tests
chronic lymphocytic leukaemia has fast or slow progression?
slow and may not require treatment
what is chronic myeloid leukaemia?
Increase in neutrophils and their precursor
most pt with chronic myeloid leukaemia have what?
Philadelphia chromosome
clinical presentation of chronic myeloid leukaemia 6
Fatigue, weight loss, sweating
Anaemia, bleeding, splenomegaly
