Haematology Flashcards
(84 cards)
What is myelodysplasia and what is the risk?
Ineffective blood cell production due to bone marrow failure, from fibrosis or lymphocytic infiltration of the marrow.
30% transform to become Acute Myeloid Leukaemia
Elderly lady at her GP is found to have a high white blood cell count, not acutely unwell, what is the likely cause?
Chronic lymphocytic leukaemia
Auer rods are characteristic of which blood cancer?
Auer rods? My rods
CML
Carbot rings occur in the blood film of people who have suffered poisoning from what?
Lead poisoning, Carbot rings may be microtubules gone awry
Which virus triggers an aplastic crisis in sickle cell patients, and what happens when this occurs?
Parvovirus B19
Reduction in RBCs
Drug causes of spherocytosis (Coombs +ve)?
MNOPQ
Methyldopa NSAIDs Oh IFN! Penicillin Quinine
Low Hb, raised bilirubin
Blood film shows: Spherocytosis, Coombs +ve
Not worsened by cold
What could be the cause and Rx?
Warm haemolytic anaemia, spherocytes = spherical cells
Coombs = autoimmune, Warm = IgG
Idiopathic
CLL, Lymphoma
Autoimmune, SLE
High dose prednisolone
Infectious causes of Coombs +ve cold autoimmune haemolytic anaemia (what type of Ig mediates it?)
IgM- mycoplasma pneumoniae, EBV
A patient has been on holiday and over the last few days has had rigors, developed jaundice and reports black urine.
FBC shows progressive anaemia. Cause? Rx?
Blackwater fever (malaria) due to massive intravascular haemolysis, possibly from autoimmune reaction. Rx: antimalarials
Things someone with Glucose 6 Phosphate Dehydrogenase should avoid and disease inheritance?
X linked, G6PD is an enzyme protecting from oxidative stress
Aspirin, sulphonamides
Broad beans
Henna, dapsone (coeliac rash)
Cause of severe haemolytic uraemic syndrome? Possible complications?
E Coli 0157, produces Shiga toxin that stimulates cytokines promoting clot formation and inactivates ADAMTS13 protease (needed to cleave vWF to prevent multimers + clots)
Kidney failure, due to clots in small vessels + bloody diarrhoea
In someone with von willebrand’s disease, what FBC and clotting studies would be expected?
vWF binds factor 8 (intrinsic cascade) to carry it, promoting adhesion of platelets to damaged endothelium.
Reduction in levels leads to prolonged bleeding time
APTT may be prolonged (as less factor 8 carried)
Hb or MCV may be low if bleeding leads to iron deficiency
How do platelet disorders and coagulation disorders present differently in regards to symptoms?
Platelet disorders (which includes vWF disease) = epistaxis and menorrhagia Coagulation disorders are more severe = haemoarthrosis and muscle haematomas
68 year old patient has splenomegaly, lymphadenopathy and IgM paraprotein level of 40g/L. Normal calcium and creatinine.
Likely diagnosis?
Waldenstrom’s macroglobulinaemia
IgM paraprotein raise= less likely to be multiple myeloma (IgG, IgD would be more likely)
And other findings are not suggestive
Monoglonal gammopathy requires paraprotein of no more than 30g
Those with polycythaemia vera typically get intense itching when? What other conditions are associated?
When warm or humid (ie getting out the shower, due to histamine release by the RBCs)
Gout and DVTs
What findings indicate tumour lysis syndrome, how can you prevent it?
Hyperkalaemia (cell breakdown)
Hyperuricaemia (purine breakdown)
Hyperphosphataemia (nucleotide breakdown)
Allopurinol
Mutation causing polycythaemia rubra vera?
JAK2 (in 90%)
RBCs are more responsive to GFs like erythropoietin and do not apoptose if EPO is absent
Cause of polycythaemia?
Primary- JAK2 mutation (= rubra vera)
Secondary, hypoxia- altitude, COPD, cyanotic heart disease, smoking
Secondary, too high EPO- renal or hepatocellular carcinoma
Which infections are associated with non-hodgkins lymphomas?
EBV- Burkitts
HTLV- T cell
HCV, HHV8- Castleman’s
H Pylori- MALT
What are the diagnostic criteria of multiple myeloma?
Name 4 things
- Serum/urine electrophoresis- monoclonal band
- Marrow biopsy- more plasma cells
- End organ damage: hypercalcaemia, renal insufficiency, anaemia
- Bone lesions via xray or PET
What is the pathogenesis of primary amyloidosis?
Myeloma- proliferation of plasma cell clone
> Amyloidogenic monoclonal Ig
> Extracellular fibrillar light chain deposits
> Resistant to degradation
> Organ failure
What are the different types of amyloidosis?
AL amyloid (primary) due to Ig chains from myeloma
AA amyloid due to acute phase protein from chronic inflammation
Familial due to mutations in ‘prealbumin’
IHx for amyloidosis?
Biopsy and Congo Red stain: birefringent light makes it apple green
Which blood clotting test does haemophilia effect?
Prolonged aPTT
As haemophilia A (F 8) and haemophilia B (F 9) both affect the intrinsic pathway (factors 12, 11, 9 and 8)