haematology Flashcards
signs and symptoms of anaemia
tiredness pallor koilonychia dizziness angina/claudication if concomitant arterial disease breathlessness tachycardia
investigations into red blood cell disorders
FBC - HCT, MCV, Hb serum iron ferritin TIBC reticulocytes haematinics (b12/folate)
causes of anaemia with low MCV, normal MCV, high MCV
low mcv - iron def, thalassaemia, anaemia of inflammation
normal mcv - renal anaemia, multiple myeloma, anaemia of inflammation, mixed haematinic and iron def, bleeding, haemolytic anaemia
high mcv - b12/folate, pregnancy, hypothyroid, liver disease, bleeding with increased retics, alcohol
how is high or low ferritin interpreted?
low ferritin is almost always iron deficiency
high ferritin is non-specific
what is pernicious anaemia?
inability to make or use intrinsic factor, which binds B12 to be absorbed
what is TIBC?
total iron binding capacity, surrogate measurement of transferin. combined with serum iron, can determine % blood iron saturation.
what is haemochromatosis?
abnormally high iron in the body causing multisystem disease
signs/symptoms of haemochromatosis?
heart failure, palpitations liver failure joint pains diabetes bronzed skin hypogonadism
investigations into haemochromatosis?
iron studies
genetic test for HFE gene
liver biopsy
MRI
2 broad categories of causes of anaemia?
high destruction
decreased production
causes of decreased production in anaemia?
haematinics deficiency
bone marrow failure
anaemia of inflammation
renal anaemia
what kind of anaemia would you get with renal anaemia?
normocytic
examples of anaemia caused by high destruction
haemolysis
haemorrhage
what is multiple myeloma and when should it be suspected?
cancer of BM causing over production of antibodies which causes destruction of BM and anaemia.
suspect in normocytic anaemia
signs/symptoms of multiple myeloma
CRAB
high calcium
renal failure
anaemia
bone lytic lesions
diagnostic investigation of multiple myeloma?
serum/urine test for lightchains
what can cause haemolysis?
odd shaped RBCs e.g. spherocytosis
autoimmune haemolysis
what would haematinic levels be like in autoimmune haemolysis?
normal
how to investigate autoimmune haemolysis?
blood film
reticulocytes
direct agglutanin test (ab)
LFTs: bilirubin raised
what can cause changes in white cells?
high - infection, leukaemia
low - drugs, disease, sepsis
what is the risk in thrombocytosis
stroke, clotting events
what is the risk in low platelets?
bleeding
what can cause high platelet count?
reactive to infection
bone marrow dysfunction e.g. myeloproliferative neoplasm
how to investigate abnormal platelet counts?
redo the FBC
serial platelet monitoring
markers of inflammation
what can cause low platelets?
platelet clumping during testing haemtinic deficiency acute leukaemia sepsis DIC ITP
what is polycythaemia vera?
primary cause of polycythaemia, genetically linked
how to investigate polycythaemia?
rule out secondary causes
genetic test for primary cause
FBC, ferritin, EPO, blood film
what is the gene involved in polycythaemia vera?
JAK2
what are secondary causes of polycythaemia?
alcohol
smoking
exogenous testosterone/EPO/steroids
EPO secreting tumour
signs and symptoms of polycythemia?
itching
flushing
splenomegaly
clotting event
treatment for polycythaemia
aspirin
venesection
hydroxycarbamide
adverse effect of hydroxycarbamide?
leg ulcers
what is essential thrombocytosis?
primary cause of raised platelets
treat like polycythaemia
what is myelofibrosis?
cancer of bone marrow causing fibrosis and progressive damage
signs and symptoms of myelofibrosis?
sweats massive splenomegaly cachexia fatigue bone pain gout pancytopaenia
investigations into myelofibrosis?
blood film - poikilocytes
bone marrow biopsy
look for splenomegaly
what causes chronic myeloid leukaemia?
associated with philadelphia gene - 9:22 translocation causing combination of BCR/ABL genes - upregulate tyrosine kinase causing overproliferation of cells
features of CML?
high wcc and platelets
splenomegaly
investigations into CML
blood film
BM biopsy
genetic/chromosome test
treatment of CML
tyrosine kinase inhibitors
stem cell transplant
complication of tyrosine kinase inhibitors?
pleural effusion
what neurological symptoms do you get with b12/folate deficiency?
paraesthesia
what vaccinations must be given before splenectomy?
Haemophilus influenza
meningococcal
pneumococcal
what kind of anaemia would also present with jaundice?
haemolytic anaemia
what will reticulocyte % be like in haemolytic anaemia?
raised %
what haematological condition do you give hydroxycarbamide?
polycythaemia, essential thrombocytosis
describing the classical description of the coagulation pathway and which tests it corresponds to
Tissue factor activates F7, F7a activates F10, F10a converts prothrombin into thrombin. Thrombin converts fibrinogen into fibrin which key ingredient in forming stable clots
F7 is extrinsic pathway (PT), F8, F9, F11 and (F12) is intrinsic pathway (APTT).
some examples of inherited bleeding disorders
haemophilia A and B
VW disease
other factor disorders
examples of acquired bleeding disorders
DIC liver failure renal failure - platelets anticoagulant and antiplatelet drugs vitamin K deficiency
questions to ask in history taking of bleeding d/o
history of abnormal bleeding
menstrual bleeds
family history of bleeding
bruises
investigations used in bleeding disorders?
coagulation screen - PT and APTT
specific factor levels
VWF levels
genetic testing
what is haemophilia A?
deficiency in F8. X linked recessive disease
how does haemophilia A usually present
childhood, boys, around age start to be mobile
abnormal bleeding, bruising, haemarthrosis
what is the function of F8 in clotting?
key factor in forming thrombin.
what will coagulation screen look like in haemophilia A
prolonged APTT. low or none F8 levels
if APTT comes back prolonged, what can be done to correct it to investigate further?
addition of individual Factors to find deficient factor
treatment of haemophilia A
lifelong F8 replacement
what is haemophilia B
less severe version of haemophilia A. deficiency in F9. usually present in adulthood
what is von willebrand disease
deficiency in VWF
what is the function of VWF?
binds platelets to endothelium
binds platelets to platelets
binds to F8 and brings it to site of coagulation