Haematology Flashcards
Why is ferritin high in anaemic of chronic disease?
The body stores iron outside the blood as it wants to keep it away from bacteria or blood loss
What is the size of the RBCs in haemolytic anaemia?
normal sized
What size are the RBCs in iron deficiency anaemia?
microcytic
Which condition is positive for the philadelphia chromosome?
chronic myeloid leukaemia
What are the features of chronic myeloid leukaemia?
Anaemia, weight loss, splenomegaly
What is the first line treatment of CML?
Imatinib
What is the management of hyperkalaemia?
Insulin and dextrose solution
What is the mechanism behind an acute haemolytic transfusion reaction?
RBC destruction by IgM-type antibodies - causes fever, abdopain, hypotension (ABO incompatibility)
What are the presenting features of myeloma?
CRABBI.
Hypercalcaemia, renal damage, anaemia, bleeding, bones, infection
What is myeloma?
A cancer of plasma cells
What investigations do you use to test for myeloma?
- blood film - rouleaux formation
- protein electrophoresis - Bence Jones proteins, raised IgA/IgG in serum
What are the features on blood test in beta-thalassaemia major?
HbA2 (variant haemoglobin with delta chains) and HbF (foetal haemoglobin) raised and HbA (normal haemoglobin) absent
What is the management of beta-thalassaemia major?
- repeated transfusion with iron chelation therapy (desferrioxamine)
What test is used to confirm an acute haemolytic reaction?
Direct coombs test
What does rhesus positive mean?
The RBCs have the rhesus antigen on them
What rhesus statuses are bad in pregnancy?
Mum - negative
Baby - positive
What happens if a mum is rhesus negative and the baby is positive?
In first child - nothing
In second - anti-D antibodies cross the placental membrane, they attack baby’s RBCs and cause haemolytic disease of the newborn
Which mother’s need routine antinatal anti-D prophylaxis?
Rhesus negative mothers who are not sensitised
What is a blood product vs a blood constituent?
Product = pharmaceutically produces
Constituent = part of blood
Which type of blood is the universal donor?
O negative
Which iron study results would show iron deficiency anaemia?
low ferritin
raised total iron-binding capacity
low serum iron
raised transferrin (low transferrin saturation)
What is the management of iron deficiency anaemia?
- treat underlying cause
- oral ferrous sulfate (for 3 months after deficiency has been corrected)
- iron-rich diet
What are the features of a transfusion-associated graft-versus-host disease?
- fever, rash, diarrhoea, abdominal pain, pancytopenia and abnormal LFTs - occurs after the transfusion can be days usually 1-6 weeks
What can be done to blood products to prevent transfusion-associated graft-versus-host disease?
Irradiation
What does irradiation do to blood?
It deactivates the white cells - mainly lymphocytes
Which blood products do not require irradiation?
Fresh frozen plasma, cryoprecipitate
Which groups of patients require CMV negative components?
pregnant women, neonates, granulocyte components
What rate should you give blood transfusions in children?
5ml/kg/hr
What is the Hb transfusion threshold in adults?
70g/L
Why must RBCs for transfusions be less than 5 days old for IUT and neonates?
risk of hyperkalaemia
What blood group platelets should be given if ABO compatibility cannot be tested?
Group A
What should the Rhesus status of platelets given be?
negative - in children and those with childbearing potential
What is the normal platelet count range?
150 to 400 x10^9/L
What are the components of fresh frozen plasma?
Coagulation factors, Immunoglobulins, proteins
What rate should red blood cells be transfused in an adult in a non-emergency situation?
4mL/kg
What commonly causes transfusion related acute lung injury?
HLA antibodies in the donor. Causes acute dyspnoea, hypoxia and bilateral pulmonary infiltrates, within 6hrs
What are delayed transfusion reactions?
Occur > 24hours after transfusion
= purpura, delayed haemolytic, Transfusion associated graft-versus host disease
Which observations are done as standard during a transfusion?
- temperature, pulse, resp rate, BP
- 60 mins before transfusion, 15 mins after starting, 60 mins after
When should you stop warfarin peri-operatively?
It should be stopped 5 days before surgery, can be swapped to LMWH which would be stopped 24hours before surgery
What are the features of Hodgkin’s lymphoma?
Reed-Sternberg cells, pain in node with alcohol, B symptoms (fever, weight loss, night sweats)
Which staging system is used for lymphoma?
Lugano
stages 1-4. 1 (1 node), 2 (2 or more nodes on one side of diaphragm), 3 (both sides), 4 (everywhere)
A (no symptoms), B (symptoms), E (extranodal disease), S (splenic involvement), X (bulky disease)
What are the features of CML?
anaemia, splenomegaly, increased granulocytes at different stages of maturation. Philadelphia chromosome. Increased neutrophils and platelets
What is the management of CML?
Imatinib
What is polycythaemia vera?
myeloproliferative disorder - proliferation of stem cells causing high wcc, platelets and rbcs
What is the definition of neutropenic sepsis?
neutrophil count < 0.5 with septic features
What is the first line management of neutropenic sepsis?
piperacillin with tazobactam
Myelodysplastic syndromes are at risk of progressing to which type of cancer?
Acute myeloid leukaemia
What types of conditions cause a metabolic acidosis with a normal anion gap?
diarrhoea, renal tubular acidosis, addisons, drugs
What conditions cause a metabolic acidosis with a raised anion gap?
lactate - shock, hypoxia, diabetic ketoacidosis, alcohol, renal failure, acid poisoning
What blood results are seen in von willebrand factor disease?
Prolonged bleeding time, normal platelets, APTT is prolonged
What is the inheritance pattern of von willebrand factor disease?
Autosomal dominant - causes reduction in VWF - does not affect platelets
What is the long term management of sickle cell anaemia?
hydroxyurea - increases HbF levels
pneumococcal vaccine every 5 years
Which factors are affected in haemophilia A and B?
both cause high APTT, normal PT - causes bleeding
haemophilia A (8)
haemophilia B (9)
What are the features of G6PD deficiency?
X-linked recessive, causes increased rbc susceptibility to oxidative stress. neonatal jaundice, causes intravascular haemolysis. heinz bodies on blood films, bite and blister cells
How do you diagnose G6PD?
G6PD enzyme assay, 3 months after acute episode of haemolysis
What are some early signs of haemochromatosis?
fatigue, erectile dysfunction and arthralgia
later: bronze skin, diabetes, liver disease, dilated cardiomyopathy, hypogonadism, arthritis
What is the pathophysiology of haemochromatosis?
autosomal recessive disorder of iron absorption and metabolism causing iron accumulation
How do you treat hyperkalaemia?
- IV calcium gluconate (to stabilise cardiac membrane - no effect on K+)
- Insulin/dextrose, nebulised salbutamol to move K+ into ICF
- calcium resonium, loop diuretics and dialysis for longer term management
What investigations do you do for myeloma?
Serum protein electrophoresis, urinary Bence-Jones protein
Which condition would you see smear/smudge cells?
CLL
How do you diagnose sickle cell?
definitive: haemoglobin electrophoresis
Smear: Howell-Jolly bodies (smiley face)
What is the management of a major haemorrhage in patients on warfarin?
prothrombin complex, vit K, stop warfarin
What is the management of hypokalaemia?
IV NaCl plus MgSO4 - must correct magnesium levels first
IV NaCl plus KCl next
What are the features of an acute haemolytic reaction?
ABO incompatibility - causes fever, abdo pain and hypotension
What are the features of transfusion-associated lung injury and transfusion-related acute lung injury?
TACO - excessive rate of transfusion, HF. Causes pulmonary oedema and hypertension
TRALI - non-cardiogenic pulmonary oedema, causes fever and hypotension
What is the management of a minor allergic reaction to a blood transfusion?
temporarily stop transfusion, antihistamine, monitor
How do you differentiate Hodgkins and non-hodgkins lymphoma?
Hodgkins - alcohol induced pain, B symptoms earlier, Reed Sternberg cells
What do you do in suspected DVT when the scan is negative but D dimer is positive?
Stop anticoagulation and repeat scan in 1 week
What is the mechanism of action of rivaroxaban/apixaban?
Direct factor Xa inhibitor
What are the features of acute sickle chest syndrome?
vaso-occlusion of pulmonary microvasculature. dyspnoea, chest pain, low O2, pulmonary infiltrates
In patients with both folate and B12 deficiency, which do you treat first?
B12, to prevent subacute combined degeneration of the spinal cord
What type of anaemia does beta-thalassaemia cause?
hypochromic, microcytic anaemia (very small RBCs)
HbA2 raised
What is the diagnostic investigation of choice for lymphoma?
Excisional node biopsy
Deficiency of which immunoglobulin increases the risk of anaphylactic blood transfusion reactions?
IgA
What can cause macrocytic anaemia?
folate, B12, alcohol, liver disease, hypothyroid, pregnancy
Which medication can be used to reduce the risk of tumour lysis syndrome?
Rasburicase
What are the features of burkitt’s lymphoma?
HIV/EBV associated. ‘starry sky’ on microscopy
What is the threshold of haemoglobin for transfusion?
70
Which medication can be used to reverse Rivaroxaban/apixaban?
Andexanet alfa
What is the first line management of CML?
Imatinib - inhibits tyrosine kinase (to do with philadelphia chromosome)
What condition are smudge cells associated with?
CLL (crushed little lymphocytes)
What is a Coomb’s test?
direct antiglobulin test - positive in haemolysis
What is the management of warm autoimmune haemolytic anaemia?
Steroids +/- rituximab
What should be taken by patients having regular blood transfusions?
Desferrioxamine (iron chelation - to prevent iron overload)
What is the management of anyone on chemo with any kind of infection?
IV Tazocin
What blood results are seen in DIC?
low platelets, low fibrinogen, high PT, APTT, schistocytes
What is Richter’s transformation?
When CLL can turn into Non-hodgkins lymphoma
What can be used to reverse the action of dabigatran?
idarucizumab
dabigatran is a direct thrombin inhibitor
Which cells come from a myeloid progenitor?
granulocytes - neutrophils, basophils, eosinophils
Which cells come from a lymphoid progenitor?
Lymphoctyes
What is the management of a DVT?
if haemodynamically stable: DOAC
if unstable: alteplase, then embolectomy if not helped
What do you see on the blood film in multiple myeloma?
Rouleaux formation
What is the management of CML?
Imatinib - tyrosine kinase inhibitor
What is the management of Non-hodgkin’s lymphoma?
R-CHOP
Rituximab, cyclophosphamide, doxyrubicin, vincristine, prednisolone
What causes a low Hb with a low reticulocyte count in sickle-cell anaemia?
Parvovirus B19, usually reticulocyte count is high in sickle-cell. parvovirus causes reticulocyte suppression
What is the most common type of non-hodgkin lymphoma?
B cell lymphoma
What conditions can polycythaemia rubra vera turn into?
AML or myelofibrosis
What causes polycythaemia vera?
JAK2 gene mutation
What is the action of hydroxyurea?
Reduces the proliferation of bone marrow cells
Why does rasburicase help in tumour lysis syndrome?
High uric acid levels - rasburicase is a urate oxidase enzyme and decreases levels fast
Which blood component is most associated with bacterial contamination?
Platelets as they are stored at room temperature
Which scan is used to stage lymphoma?
PET-CT
What are the features of lead poisoning?
Abdominal pain, constipation, neuropsychiatric features, basophilic stippling (blue lines on gums)
What type of anaemia does sickle cell cause?
Normocytic with a raised reticulocyte count
What is the first line management of polycythaemia vera?
Aspirin and venesection
Which medication can be used in sickle cell to reduce the incidence of vaso-occlusive crises?
Hydroxycarbamide/hydroxyurea - increases the synthesis of foetal haemoglobin
What are the features of haemophilia A?
Bleeding, bruising, haemoarthroses, prolonged APTT. Normal PT. Deficiency of factor 8.
What are the features of heparin induced thrombocytopaenia?
Immune mediated against heparin. 5-10 days after start of treatment. Low platelets, but prothrombotic state
