Haem Flashcards
Downs syndrome = which cancer?
ALL
philadelphia chromosome = which cancer?
ALL
CML
smudge cells = which cancer?
CLL
Warm autoimmune haemolytic anaemia = which cancer?
CLL
Auer rods = which cancer?
AML
What are the 3 stages of CML?
- chronic - 5yrs - high white cell count
- accelerated – blasts, thrombocytopenia, anaemia
- blast - pancytopenia
pain in neck when drinking = which cancer?
Hodgkins lymphoma
reed sternberg cells = which cancer?
Hodgkins lymphoma
Owls eye = which cancer?
Hodgkins lymphoma
rouleux formation = which cancer?
myeloma
Lymphoblastic leukaemias come from which cell line?
Lymphocytes (B)
CML and AML come from which cell line?
Myeloid (hence myeloblastic in name)
Chronic leukaemias come from ______ cells whereas acute leukaemias come from _______ cells
chronic - from well defined line
acute - from blasts
Lymphomas are usually from what cell line?
B
3 Causes of microcytic anaemia?
T thallassaemia Anaemia of chronic disease Iron deficiency (most common) Lead Sideroblastic
3 causes of normocytic anaemia?
Anaemia of chronic disease Aplastic Acute bleeding Hypothyroid Haemolytic Sickle cell
Cause of megaloblastic macrocytic anaemia?
Low vit B12
Low folate
3 Causes of normoblastic macrocytic anaemia?
alcohol liver dis hypothyroid azathiopurine reticular cytosis
what is the definition of anaemia?
low haemoglobin in the blood
what is pernicious anaemia?
autoimmune destruction - of parietal cells or intrinsic factor
= cannot absorb B12
2 causes of low folate?
pregnancy
lack of green veg
malabsorption
haemolysis
5 symptoms of anaemia?
tired SOB headache dizzy palpitations pale conjunctival pallor resp rate high tachycardia
5 symptoms / signs specifically of iron deficiency anaemia?
pica hair loss koilonychia angular cheilitis atrophic glossitis brittle hair / nails
investigations for haemolytic anaemia?
haemoglobin, MCV
blood film - spherocytes and polychromasia
haptoglobin (low)
lactate dehydrogenase (high)
blood tests that indicate the amount of iron?
ferritin - low
transferrin saturation - low
total iron binding capacity - high
an example of an oral iron supplement? a problem with this?
ferrous sulphate
slow so not good enough for severe cases
4 presenting features of DVT?
pain unilateral swelling warmth tender discolouration phlegmasia
gold standard investigation for ?DVT?
ultrasound/doppler
first line investigations in DVT?
measure the calf D dimer (sensitive not specific)
management of DVT?
heparin
warfarin
apixaban
recannalisation
what part of haemoglobin is affected in sickle cell?
beta
what part of haemoglobin is affected in sickle cell?
beta
what haemoglobin proteins to adults have?
2 alpha 2 beta
3 main features of sickle cell disease? & the symptoms this causes?
pain
haemolysis = jaundice, gallstones
anaemia = tired, dizzy, palpitations
3 consequences of sequestration crisis in sickle cell?
thrombocytopenia splenomegaly abdo pain hypovolaemic shock fibrosis of spleen infection with encapsulated bacteria
3 complications of sickle cell?
sequestration crisis acute chest sepsis heart failure vaso occlusion of bone (avascular necrosis, dactylitis)
what kind of anaemia in sickle cell?
normocytic
what test for screening newborns for sickle cell?
guthrie
what do you see on the blood film in sickle cell? 3
sickled RBCs
target cells
howell jolly bodies
gold standard for sickle cell?
Hb electrophoresis
drug for sickle cell, how does it work?
hydroxycarbamide, stimulates production of foetal haemoglobin which is unaffected
supportive management for sickle cell?
antibiotics transfusions iron chelation folic acid vaccination bone marrow transplant analgaesia oxygen
5 symptoms / signs of leukaemia?
tiredness failure to thrive anaemia (pallor, dizzy etc) petechiae bleeding lymphadenopathy hepatosplenomegaly infection testicular swelling
gold standard diagnosis for leukaemia?
bone marrow biopsy (trephine or liquid aspiration) shows smudge cells - not specific for CLL
for CLL you would use peripheral blood flow cytometry
risk factors for lymphoma?
immunosupression:
- wiscott aldrich
- drugs eg methotrexate
- HIV
infection eg HIV, H pylori
what are the B symptoms?
fever
night sweat
weight loss
associated with lymphoma
apart from B symptoms 3 other symptoms of lymphoma?
lymphadenopathy (rubbery non tender) fatigue itching SOB infection abdo pain splenomegaly compression syndrome eg optic nerve
gold standard diagnosis for lymphoma?
core needle lymph node biopsy
what staging system is used for lymphoma?
ann arbour
explain the classes of ann arbour staging?
1 - one lymph node or area of nodes
2 - more than one lymph node on same side of diaphragm
3 - in lymph nodes above + below diaphragm
4 - widespread, in organs
what is MGUS?
pre malignant myeloma - 1% progression per year
excess of an antibody, no symptoms
MGUS is also the name of a gene associated with myeloma
what is smouldering myeloma?
pre malignant
plasma cells make up 10% of bone marrow population
what is Waldenstroms macroglobulinaemia?
smoulding myeloma with IgM
what does CRAB stand for?
calcium
renal
anaemia
bone
what is the monoclonal paraprotein?
the antibody that is overproduced in myeloma
what is the benz jones protein and where can you test to find it?
in the urine
the light chai of the monoclonal paraprotein in myeloma
what happens to the bones in myeloma?
lytic and circular lesions
because of osteoclast activity
pepperpot skull
what happens to the viscosity of the blood in myeloma?
viscosity increases because of all the protein in the blood
= vascular eye disease
but there are few platelets (because bone marrow busy making plasma cells) so bleeding, bruising, erythema
what technique do you use to look for the benz jones protein in urine and the monoclonal paraprotein in serum?
electrophoresis
what is the usual treatment for myeloma?
induction therapy (bortezamid, dexamethasone and thalidomide) followed by stem cell transplant
bisphosphonates
thromboembolism prophylaxis
where do malaria parasites go in the body?
mosquito saliva patients bloodstream liver blood cells bitten by mosq -- mosq saliva
3 traits that protect against malaria?
sickle cell
thalassaemia
G6PD deficiency
which species causes the most severe form of malaria?
plasmodium falciparum
presentation of malaria?
cyclical fever (sweats, rigors) and anaemia (jaundice)
hypoglycaemia myalgia vomitting hepatosplenomegaly headache fatigue
3 complications of ‘complicated malaria’?
coagulation = cerebral ischaemia, resp ischaemia, ARDS
vascular permeability = translocation of bacteria from bowel = sepsis
AKI
disseminated intravascular coagulation
tests for malaria?
blood film
- thick - is there malaria?
- thin - what species?
rapid antigen diagnostic test
what type of anaemia in malaria?
normocytic
treatment for complicated malaria?
IV artesunate
quinine dihydrochloride
treatment for mild malaria?
proguanil
atorvaquone
3 causes of secondary polycythaemia?
smoking
hypoxia
altitude
renal or hepatocellular carcinoma that is ectopically producing EPO
what is the most common mutation in polycythaemia? There are 2 more?
JAK 2 - most common (= EPO stimulated proliferation physiologically)
MPL
CALR
what leukaemia are myeloproliferative disorders incl polycythaemia most likely to progress into?
AML
There are 2 phases in polycythaemia vera, what happens in each?
first phase: increase platelets, neutrophils and RBCs
= hyperviscosity of blood = DVT
but also bleeding because even though there are a lot of RBCs they arent properly regulated / functional
spent phase: BM cannot produce cells = low platelets, leucocytes and anaemia, fibrosis of the bone marrow, hepatosplenomegaly in an attempt to compensate
5 presenting features of PCV?
ruddy / plethoric complexion red eyes (conjunctival plethora) palmar erythema HTN hepatosplenomegaly DVT etc symptoms of anaemia eg fatigue, dizzy etc blurred vision sweating/flushing pruritis eythromyalgia
2 ways you can work out if its primary or secondary polycythaemia?
EPO: low in primary, high in secondary
(secondary polycythaemia = high EPO so high RBCs etc)
ABG: high pCO2 could explain the need for secondary polycythemia
On a blood film in PCV what would you see?
tear drop cells
poikylocytosis
blasts
management of PCV?
venesection
aspirin
stem cell transplant
chemo
what are the intrinsic and extrinsic clotting pathways?
intrinsic = activated by platelets close to the damage extrinsic = activated by exposed tissue factor
how is von Willebrand disease inherited & what does it cause?
dominant
deficiency of vWF = abnormal bleeding
what is ITP?
immune system produces antibodies against platelets
= destruction of platelets, rubbish clotting, purpura, bruising, bleeding
treat with prednisolone, retuximab
what is TTP?
mutation in or autoimmunity to the protein that activates vWF
multiple clots in small vessels = angiopathy and uses up the platelets
Heinz bodies / bite cells indicate?
G6PD deficiency
Investigations for haemophilia?
coagulation factor assay
genetic testing
PT time normal (as is extrinsic and intrinsic pathways)
activated partial prothrombin time is prolonged (as is only the intrinsic and common pathway. Haemophilia affects intrinsic pathway only)
what is haemophilia A and B?
A = low factor 8 B = low factor 9
Which leukaemia is most likely to affect young children?
ALL
How long do you need to be on anticoagulants for after DVT?
3 months if provoked
6 months if unprovoked
low haemoglobin but high reticulocyte is ..
sickle cell
hereditary spherocytosis
low Hb because of increased haemolysis
high reticulocytes bc body attempting to replace damaged
what is thalassaemia?
a defect in alpha or beta globin – abnormally functioning Hb – microcytic anaemia and haemolysis
presentation of thalassaemia?
prominent forehead/cheek bones due to bone marrow expansion fractures splenomegaly jaundice symptoms of anaemia failure to thrive gallstones
what is hereditary spherocytosis?
jaundice, anaemia and splenomegaly RBCs are more permeable to sodium aplastic crisis with parvovirus autosomal dominant inheritance north europe management = splenectomy
what condition causes jaundice, splenomegaly and anaemia especially after antimalarials/antibiotics, broad beans or infections?
G6Pd deficiency
are lymphoma myeloproliferative disorders?
no, myeloproliferative disorders affect the myeloid line not the lymphoid line (so some leukaemia are myeloproliferative)
what bleeding disorder causes raised bilirubin?
TTP
bilirubin from the body breaking down all the clots that TTP goes about making
3 things that myeloma is characterised by?
Malignant proliferation of plasma cells
Lytic deposits in bones
Presence of abnormal globulin (usually IgG or A)
Which cancer has gum hypertrophy?
AML
Treatment for Hodgkin lymphoma?
ABVD (Adriamycin, bleomycin, vinblastine, dacarbazine)
Treatment for non Hodgkin lymphoma?
R-CHOP rituximab cyclophosphamide prednisolone vincristine
Treatment for ALL and AML?
Chemo Radio Stem cell transplant allopurinol - for all blood/platelet tranfusion
Treatment for CML and CLL?
CML - imatinab (philadelphia)
CLL - chemo and radio, nothing is curative
presentation of TTP?
neuro - eg headache confusion dysarthria fever microangiopathic haemolytic anaemia thrombocytopenia - bleeding bruising renal failure
Tests and results for TTP?
Blood smear shows fragmented erythrocytes, ie schistocytes
high LDH.
Indirect bilirubin level
reticulocyte count is elevated
Urinalysis shows proteinuria and microscopic haematuria
treatment for TTP?
plasma exchange
IV prednisolone
IV rituximab
presentation of DIC?
asymptomatic bleeding bruising petechiae SOB haemoptysis
investigations and results for DIC?
Low platelets
Prolonged APTT, prothrombin and bleeding time
Fibrin degradation products raised (eg. D-dimer)
Blood film; Schistocytes due to microangiopathic haemolytic anaemia
3 presentations of ‘bleeding disorders’
gum bleeding menorrhagia epistaxis petechiae bruising
which species of protozoa cause relapses of malaria?
p ovale
p vivax
What chromosomal translocation is there in myeloma?
11;14
Which leukaemia can undergo a Richter’s transformation to malignancy?
CLL
3 symptoms of G6PD deficiency?
fatigue
palpitations
SOB
pallor
4 things in the wells score?
heart rate over 100
haemoptysis
malignancy
signs of DVT
explain the pathophysiology of DIC?
consumptive coagulopathy
tissue damage = release of tissue factor = widespread clot
tissue plasminogen activator = fibrinolysis
thrombosis and then fibrinolysis of those clots = bleeding
sepsis 6?
give fluuid give broad spec abx give oxygen take blood culture lactate dehydrogenase urine output
