Haematology Flashcards
What is the most commonly inherited clotting disorder and its mode of inheritance?
Von Willebrand’s factor
Mostly autosomal dominant
What would coag results look like in haemophilia?
1) Prolonged APTT
2) BT, TT and PT all normal
What do blood parameters look like in DIC?
↓ platelets
↓ fibrinogen
↑ PT & APTT
↑ fibrinogen degradation products
High D-dimer
What would coag results look like in Von Willebrand’s disease?
1) Prolonged bleeding time
2) APTT may be prolonged
Normal platelets
What is the most common inherited thrombophilia?
Factor V Leiden
What would blood tests show in tumour lysis syndrome?
High potassium
High phosphate
Low calcium
What is the medical management for Tumour lysis syndrome prophylaxis?
allopurinol or rasburicase
Why use irradiated blood?
To avoid transfusion-associated graft versus host disease
FBC results in sickle cell anaemia?
Low Hb
Normal MCV
Raised reticulocytes
What is the single most important factor in determining whether cryoprecipitate should be given?
A low fibrinogen level
What are signs of hyposplenism on a blood film?
target cells
Howell-Jolly bodies
What are the signs of IDA on blood film?
target cells
What is the sign of myelofibrosis on blood film?
‘tear-drop’ poikilocytes
What is the sign of microangiopathic haemolytic anaemia/ intravascular haemolysis on blood film?
Schistocytes
What is the sign of megaloblastic anaemia on blood film?
hypersegmented neutrophils
What is the blood film feature for multiple myeloma?
Rouleaux formation
What are blood film signs of Acute Myeloid Leukaemia?
Auer rods
What is in cryoprecipitate?
Factor VIII
Factor XIII
VWF
Fibrinogen
What is the management of antiphospholipid syndrome for pregnancy?
1) Low-dose aspirin once urine pregnancy test confirmed
2) LMWH once fetal heart rate on ultrasound
What are the causes of warm AIHA?
1) idiopathic
2) autoimmune disease: e.g. systemic lupus erythematosus*
3) neoplasia (lymphoma,
chronic lymphocytic leukaemia)
4) drugs: e.g. methyldopa
What is the management of warm AIHA?
1) treatment of any underlying disorder
2) steroids (+/- rituximab) are generally used first-line
What are the causes of cold AIHA?
1) neoplasia: e.g. lymphoma
2) infections: e.g. mycoplasma, EBV
What are the blood test features of haemolytic anaemia?
1) Anaemia
2) reticulocytosis
3) Low haptoglobin
4) raised LDH
5) Blood film: spherocytes and reticulocytes
Specific feature to autoimmune: positive direct antiglobulin test (Coombs’ test).
What is the management of Beta-Thalassaemia major?
repeated transfusion
(note can lead to Fe overload so iron chelation therapy important e.g. desferrioxamine)
What is the sign on blood film for megaloblastic anaemia?
hypersegmented neutrophils
What is the management of a Non-haemolytic febrile reaction?
Slow or stop the transfusion
Paracetamol
Monitor
What is the management of a Minor allergic reaction?
Temporarily stop the transfusion
Antihistamine
Monitor
What is the management of anaphylaxis during blood transfusion?
Stop the transfusion
IM adrenaline
ABC support
oxygen
fluids
What is the management of an Acute haemolytic reaction?
1)Stop transfusion
2)Confirm diagnosis
- check the identity of patient/name on blood product
- send blood for direct Coombs test, repeat typing and cross-matching
3)Supportive care
- fluid resuscitation
What is the management of Transfusion-associated circulatory overload (TACO)?
Slow or stop transfusion
Consider intravenous loop diuretic (e.g. furosemide) and oxygen
What is the management of Transfusion-related acute lung injury (TRALI)?
Stop the transfusion
Oxygen and supportive care
What virus is associated with Burkitt’s lymphoma?
Epstein-Barr virus (EBV) is strongly implicated in the development of the African form
What are microscopy findings for Burkitt’s lymphoma?
‘starry sky’ appearance: lymphocyte sheets interspersed with macrophages containing dead apoptotic tumour cells
What are the complications of tumour lysis syndrome?
hyperkalaemia
hyperphosphataemia
hypocalcaemia
hyperuricaemia
acute renal failure
What is Richter’s transformation?
Ritcher’s transformation occurs when leukaemia cells enter the lymph node and change into a high-grade, fast-growing non-Hodgkin’s lymphoma.
E.g. CLL-> non-hodgkin’s
What are the blood test features for chronic lymphocytic anaemia?
1) lymphocytosis
2) anaemia: due to bone marrow replacement or
AIHA
3) thrombocytopenia
4) Blood film: smudge cells
What is the management of chronic myeloid leukaemia?
1st line: imatinib
Also:
hydroxyurea
interferon-alpha
allogenic bone marrow transplant
What is the management of hereditary spherocytosis?
1) acute haemolytic crisis:
- treatment is generally supportive
- transfusion if necessary
2) longer term treatment:
- folate replacement
- splenectomy
What is the staging system used for patients with Hodgkin’s lymphoma?
Ann-Arbor staging
What is the management of Hodgkin’s lymphoma?
1st line: chemotherapy
Also:
- Radiotherapy
- Combined modality therapy
- Hematopoietic cell transplantation
What is the management of ITP?
Oral prednisolone
What are the causes of macrocytic anaemia?
Megaloblastic:
1) vitamin B12 deficiency
2) folate deficiency
3) e.g. secondary to methotrexate
Normoblastic:
alcohol
liver disease
hypothyroidism
pregnancy
reticulocytosis
myelodysplasia
drugs: cytotoxics
What are the causes of microcytic anaemia?
1) iron-deficiency anaemia
2) thalassaemia*
3) congenital sideroblastic anaemia
4) anaemia of chronic disease (more commonly a normocytic, normochromic picture)
5)lead poisoning
What are the features of myeloma?
Calcium (HIGH)
Renal (impairment)
Anaemia (fatigue and pallor)
Bleeding (and bruising)
Bones (pain)
Infection (susceptibility to infections)
What are the investigations for myeloma?
Bloods
1) Anaemia
2) rouleaux formation on blood film
3) renal failure
4) hypercalcaemia
Protein electrophoresis
1) serum: raised concentrations of monoclonal IgA/IgG proteins
2) urine: Bence Jones proteins
Bone marrow aspiration (confirms diagnosis)
Imaging
1) whole body MRI
2) X-Ray: ‘rain-drop skull’
- raised no of plasma cells
What are the causes of normocytic anaemia?
1) anaemia of chronic disease
2) chronic kidney disease
3) aplastic anaemia
4) haemolytic anaemia
5) acute blood loss
How is Paroxysmal nocturnal haemoglobinuria diagnosesd?
flow cytometry of blood to detect low levels of CD59 and CD55
What is the management of Paroxysmal nocturnal haemoglobinuria?
1) blood product replacement
2) anticoagulation
3) stem cell transplantation
What is the management of Polycythaemia vera?
1) Venesacion =1st line- keep Hb in range
2) Aspirin
3) Chemotherapy
What is the long-term management of sickle cell anaemia?
1) Hydroxyurea (increases HbF so is prophylactic)
2) pneumococcal polysaccharide vaccine every 5 years
What are the types of sickle cell crises?
1) Thrombotic crises
2) Acute chest syndrome
3) Aplastic crises
4) Sequestration crises
What is the management of Von Willebrand’s disease?
1) tranexamic acid for mild bleeding
2) desmopressin (DDAVP)
3) factor VIII concentrate
