Haematology Flashcards
How would you further investigate a microcytic anaemia?
haematinics (iron studies, B12 and folate)
coeliac screen - TTG antibodies
consider endoscopy and colonoscopy if old or RF for bowel CA
what results of blood tests would you expect a patient suffering from DIC to have?
low platelets
low fibrinogen
high PT and APTT
high D-dimer and fibrin degradation products
what are hereditary causes of haemolytic anaemia?
hereditary spherocytosis
hereditary elliptocytosis
G6PD deficiency, pyruvate kinase deficiency
sickle cell disease, thalassaemia
what are acquired causes of a haemolytic anaemia?
autoimmune (SLE)
some drugs
infection
MAHA
What would be the differential for a patient present with backache with hypercalcaemia, low PT and normal ALP ?
multiple myeloma
What features do patients with multiple myeloma have?
CRAB
Calcium is high
Renal impairment
Anaemia
Bone (pain/ache or fracture)
What might patients with polycythaemia present with?
headache
pruritis post hot bath
blurred vision
tinnitus
thombosis
grangrene
choreiform movements
what causes a low reticulocyte count?
parvovirus B19 infection (-> aplastic crisis)
aplastic crisis 2nd to sickle cell
blood transfusion
What is the most common cause of aplastic anaemia?
idiopathic acquired aplastic anaemia
What are some secondary causes of aplastic anaemia?
infection - parvovirus B19
cytotoxic drugs
What do Heinz bodies suggest?
oxidized Hb
GLUCOSE-6-PHOSPHATE DEHYDROGENASE
causes of macrocytosis
B12/folate deficiency
alcoholism
myelodysplastic disorders
hypothyroidism
liver disease
What does a positive Coombs test indicate?
confirms the presence of antibodies to RBC indicating a autoimmune haemolytic anaemia
What do Burr cells indicate?
uraemia
what do target cells indicate?
IDA
What do reed-sternberg cells indicate?
hodgkin’s lymphoma
What is the philadelphia chromosome associated with?
CML
What does a positive Hams test indicate?
paroxysmal nocturnal haemoglobinuria
what is paroxysmal nocturnal haemoglobinuria?
acquire RBC defect making them more susceptible to being destroyed by complement
PC: abdo pain, haemolytic anaemia, dark coloured urine at night or early morning
What are hyperviscosity symptoms?
blurry vision from retinal haemorrhages
headaches
bleeding
severe pruritus post bath
what is the tumour marker for a seminoma?
beta-HCG
What cancer is EBV a RF for?
lymphoma
what is haemophilia?
an inherited deficiency in clotting factors leading to a bleeding disorder
rarely an acquired form may occur whereby an individual makes autoantibodies to clotting factors
What are the main types of haemophilia?
haemophilia A - factor 8 deficiency
haemophilia B - factor 9 deficiency
haemophilia C - factor 11 deficiency
what is the most common type of haemophilia?
haemophilia A
Epidemiology of acute lymphoblastic leukaemia?
most common malignancy in children
peak incidence 2-5 years of age
second peak of incidence in the elderly
what are the stages of CML?
- relatively stable chronic phase (4-6 years)
- accelerated phase (3-9 months)
- acute leukaemia phase characterised by blast transformation
Describe the ann arbor staging system for lymphoma
I = single LN region II = 2+ LN regions on one side of diaphragm III = LN regions on both sides of diaphragm IV = extranodal involvement
A = no B symptoms B = B symptoms S = spleen involvement
Give features of Hodgkin’s lymphoma
15% of all lymphomas
Reed-sternberg cells are diagnostic
EBV infection is a risk factor
bimodal incidence - 20-30s and >50s
M>F
What are some presenting features of Hodgkin’s lymphoma?
enlarged mass in neck/axilla/groin which may spontaneously enlarge or reduce in size
mass painful post alcohol
pruritis, cough, SOB
B symptoms: fever, night sweats, weight loss
what are the B symptoms?
fever, night sweats, weight loss
is extra nodal involvement more common in Hodgkin’s or non-Hodgkin’s?
non hodgkin’s
RF for Non-hodgkin’s lymphoma
radiotherapy, immunosuppresion, chemotherapy, HIV, HBV, HCV, oncogenic viruses (EBV)
Presentation of non-Hodgkin’s lymphoma
painless enlarged mass in neck/axilla/groin
systemic symptoms (FLAWS)
extra-nodal - skin rash, headache, sore throat, abdo pain, testicular swelling
BM failure -> anaemia, infections, bleeding
hypercalcaemia
what is multiple myeloma?
haematological malignancy characterised by proliferation of plasma cells causing increased monoclonal immunoglobulin production (usually IgG or IgA)
what monoclonal immunoglobulin is most commonly implicated in multiple myeloma?
IgG
accounts for 2/3 of cases
What would the blood results and blood film show for a patient with multiple myeloma?
low Hb
high creatinine
high calcium
high ALP
rouleux formation on blood film
what might you find in urine analysis of a multiple myeloma patient?
serum paraprotein
BENCE JONES PROTEIN
what is myelofibrosis?
obliteration of the bone marrow with fibrosis due to increased fibroblast activity
what conditions are associated with myelofibrosis?
30% of myelofibrosis patients have a Hx of polycythaemia rubra vera or essential thrombocytopenia
What are the symptoms and signs of myelofibrosis?
weight loss, fever, loss of appetite, night sweats, pruritis
splenomegaly, hepatomegaly, symptoms of BM failure
what are the symptoms of polycythaemia?
headache, blurred vision, tinnitus
SOB
pruritis after a hot bath
burning pain in fingers and toes
night sweats
thrombotic events
what are the signs of polycythaemia?
splenomegaly
plethoric complexion [red and ruddy]
scratch marks from ithcing
HTN
what is the inheritance pattern of thalassaemia?
autosomal recessive
what does the impaired Hb synthesis in thalassaemia patients lead to?
- impaired erythropoiesis
- haemolysis
- anaemia
- extramedullary haematopoiesis
how does alpha thalassaemia 3 gene deletion present?
what signs on blood film are there?
severe anaemia, splenomegaly, jaundice and abnormal RBC indices
very early life
HEINZ BODIES
how does beta thalassaemia major present?
3-6 months of age when foetal haemoglobin becomes adult Hb
failure to thrive, recurrent infections, anaemia
what are the signs of major beta thalassaemia?
pallor, malaise, SOB, mild jaundice, frontal bossing, thalassaemia facies, hepato-splenomegaly
what is the pentad of thrombotic thrombocytopenic purpura?
MAHA
thrombocytopenia
renal involvement (impairment)
neurological abnormalities
fever
note majority of patients with TTP don’t have the pentad
what are the causes of TTP?
idiopathic (40%)
autoimmune e.g. SLE
cancer
pregnancy or post-partum (10-25%)
malignancy
drug related (quinine)
bloody diarrhoea prodrome type (progression of HUS from E.coli infection)
what are the symptoms and signs of TTP?
epistaxis, bruising, petechiae, haematuria, menorrhagia
NEURO: confusion, headache, vision changes, aphasia, fits
fever, pallor, myalgia, chest/abdo pain
purpura, jaundice (2nd to haemolysis), severe HTN, splenomegaly
what are the functions of vWF?
- binds to exposed subendothelium
- aids platelet activation and binding to one another
- stabilises factor VIII preventing it’s degradation
what are the type of von willebrands disease
type 1 = most common, autosomal dominant, deficiency in vWF
type 2 = 2nd most common, abnormal vWF, autosomal dominant
type 3 = undetectable vWF levels, autosomal recessive
what clotting factor abnormalities do vWF disease patients have ?
low VIII
vWF abnormality/deficiency
prolonged clotting time (high APTT)
What malignancy are auer rods associated with?
acute myeloid leukaemia
what is aplastic anaemia?
it is a global pancytopenia where cells are normal in morphology but deficient.
BM biopsy shows hypocellular marrow
at least 2 peripheral deficiencies must be detected of either: Hb < 100, platelets < 50 or neutrophil < 1.5 x 10^9
what are some acquired causes of aplastic anaemia?
parvovirus B19
medications such as methotrexate
chemicals such as benzene or DDT
paroxysmal nocturnal haemoglobinuria
what are inherited causes of aplastic anaemia?
fanconi’s anaemia
what is the diagnosis of DIC based on?
presence of > 1 known underlying condition causing DIC + abnormal global coagulation tests
- reduced paltelet count
- increased PT
- increased fibrin-related marker (d-dimer, fibrin degradation production)
- decreased fibrinogen levels
what drugs trigger G6PD deficiency?
sulpha drugs
- sulphonamides - trimethoprim, sulfasalazine
- sulphasalazine
- sulphonylureas - glipizide, gliclazide, glimepiride
who gets irradiated transfusion and who gets CMV -ve
