Haematology Flashcards
Describe the clinical presentation of an acute haemolytic reaction
Fever, abdominal pain, hypotension during a blood transfusion
What blood results are asociated with CML?
Increase in granulocytes (neutrophils, eosinophils, and basophils) at different stages of maturation +/- thrombocytosis
What is Richter’s transformation?
Transformation of CLL to high-grade lymphoma - patients suddenly deteriorate
Name a medication that can precipitate renal failure in patients with multiple myeloma
NSAIDs
Describe the presentation of transfusion-associated circulatory overload
- Pulmonary oedema, elevated JVP
- Afebrile
- S3 present
- Hypertension
- More common in patients with pre-existing HF
Describe the management of transfusion-associated circulatory overload
- Slow or stop transfusion
- Consider intravenous loop diuretic (e.g. furosemide) and oxygen
Patient has low platelets and is due surgery/invasive procedure. They are given a platelet transfusion.
What should the platelet levels be before the procedure?
- > 50×109/L for most patients
- > > 50-75×109/L if high risk of bleeding
- > 100×109/L if surgery at critical site
What blood results are associated with iron deficiency anaemia?
- Increased ferritin
- Increased TIBC
- Decreased serum iron
- Decreased transferrin saturation
Describe the presentation of transfusion related lung injury
Hypotension, pyrexia, normal/unchanged JVP
Describe the management of transfusion related lung injury
- Stop the transfusion
- Oxygen and supportive care
A 15-year-old girl presents with abdominal pain. She is normally fit and well and currently takes a combined oral contraceptive pill. The patient is accompanied by her mother, who is known to have hereditary spherocytosis. The pain is located in the upper abdomen and is episodic in nature, but has become severe today. There has been no change to her bowel habit and no nausea or vomiting. What is the most likely diagnosis?
Biliary colic
This patient has hereditary spherocytosis resulting in chronic haemolysis and gallstone formation
What is hereditary spherocytosis?
- Most common hereditary haemolytic anaemia in people of northern European descent
- Autosomal dominant defect of red blood cell cytoskeleton
- The normal biconcave disc shape is replaced by a sphere-shaped red blood cell
- Red blood cell survival reduced as destroyed by the spleen
Describe the clinical presentation of hereditary spherocytosis
- Failure to thrive
- Jaundice, gallstones
- Splenomegaly
- Aplastic crisis precipitated by parvovirus
- Infection
- Degree of haemolysis variable
- MCHC elevated
What metabolic abnormalities are seen in tumour lysis syndrome?
High potassium, high phosphate, and low calcium
What medication is used as prophylaxis for tumour lysis syndrome?
Allopurinol (low risk), IV rasburicase (high risk)
In multiple myeloma, what would bone marrow aspirate reveal?
Plasma cells
What metabolic abnormality can be caused by transfusion of packed red cells?
Increased serum potassium
What investigation is used for definitive diagnosis of sickle cell disease?
Haemoglobin electrophoresis
Describe the presentation of myelofibrosis
- Elderly person with symptoms of anaemia e.g. fatigue (the most common presenting symptom)
- Massive splenomegaly
- Hypermetabolic symptoms: weight loss, night sweats etc
What findings on blood film are associated with myelofibrosis?
‘Tear-drop’ poikilocytes
Sickle cell crises: describe presentation of thrombotic crises (painful crises, vaso-occlusive crises)
Infarcts occur in various organs including the bones (e.g. avascular necrosis of hip, hand-foot syndrome in children, lungs, spleen and brain
Sickle cell crises: describe presentation of acute chest syndrome
Dyspnoea, chest pain, pulmonary infiltrates on chest x-ray, low pO2
Sickle cell crises: describe the management of acute chest syndrome
- Pain relief
- Respiratory support e.g. oxygen therapy
- Antibiotics: infection may precipitate
- Transfusion: improves oxygenation
Sickle cell crises: describe the presentation of aplastic crises
- Sudden fall in haemoglobin
- Bone marrow suppression causes a reduced reticulocyte count
Sickle cell crises: describe the presentation of sequestration crises
Sickling within organs such as the spleen or lungs causes pooling of blood with worsening of the anaemia
Associated with an increased reticulocyte count
What is the Ann-Arbor staging?
The Ann-Arbor staging system was historically the main way patients with Hodgkin’s lymphoma were staged:
- I: single lymph node
- II: 2 or more lymph nodes/regions on the same side of the diaphragm
- III: nodes on both sides of the diaphragm
- IV: spread beyond lymph nodes
What is the universal donor of FFP?
AB RhD negative blood
What test is used to confirm autoimmune haemolytic anaemia?
Direct Coombs test
What is the blood transfusion threshold for patients with ACS?
80 g/L
When is platelet transfusion indicated?
Patients with a platelet count < 30 x 109 and clinically significant bleeding
After a complicated revision of a total hip replacement, an 80-year-old lady receives two units of packed red cells. She has a history of heart failure and currently takes bisoprolol, ramipril and furosemide.
Which should be prescribed between the units?
Stat dose of furosemide
Reduce the risk of transfusion overload
What is the management of anaphylactic reaction to blood transfusion?
Permanent transfusion termination, IM adrenaline, antihistamine, corticosteroids, bronchodilators, supportive care
What medication is prescribed to reduce the risk of thrombotic events in polycythaemia vera?
Aspirin
Describe the presentation of polycythaemia vera
Hyperviscosity, facial plethora, pruritus and splenomegaly,
What drugs are contraindicated in G6PD deficiency?
Sulph- drugs: sulphonamides, sulphasalazine and sulfonylureas can trigger haemolysis
What blood results are assocaited with haemophilia?
- Prolonged APTT
- Bleeding time, thrombin time, prothrombin time normal
What factor is deficienct in haemophilia A?
Factor VIII
What factor is deficienct in haemophilia B?
Factor IX
What type of haemophilia is the most common?
A
What blood results are associated with sideroblastic anaemia?
- Hypochromic microcytic anaemia
- High ferritin iron & transferrin saturation
- Basophilic stippling of red blood cells
A 34-year-old male with no previous medical history presents to general practice with difficulty breathing, fatigue, and dark urine.
Blood tests show a decreased haptoglobin levels.
What is the most likely diagnosis?
Autoimmune haemolytic anaemia
A decrease in haptoglobin levels can be seen in intravascular haemolysis
What blood results are associated with von Willebrand’s disease?
Prolonged bleeding time and increased APTT
What blood results are seen in acquired haemophilia?
Prolonged APTT with normal PT
Describe the features of antiphospholipid syndrome
- Recurrent thromboembolism and bleeding in a young woman
- Other features may include foetal loss, venous and arterial thrombosis and thrombocytopenia
- A Lupus anticoagulant may be present and the APTT is prolonged
Give two differentials for anaemia with reticulocytosis
Haemlysis or bleeding
Haemolysis is associated with (high/low) haptoglobin
Low
What is the diagnostic test for hereditary spherocytosis?
EMA binding test