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
What bacteria are the most common cause of neutropenic sepsis?
Coagulase-negative, Gram-positive bacteria such as Staphylococcus epidermidis
How do you diagnose of Richeter’s syndrome?
Lymph node biopsy
Low SpO2 + polycythaemia. What is the most likely underlying diagnosis?
COPD
Name 4 causes of thrombophilia
- Protein S deficiency
- Antithrombin III deficienc
- Protein C deficiency
- Activated protein C resistance
What is the most common cause of inherited thrombophilia?
Factor V Leiden (activated protein C resistance)
What blood gas result is associated with nitrates (including amyl nitrate - poppers)?
Methaemoglobinaemia (raised MetHb)
What is the mangement of B12 deficiency?
- If no neurological involvement 1 mg of IM hydroxocobalamin 3 times each week for 2 weeks, then once every 3 months
- If a patient is also deficient in folic acid then it is important to treat the B12 deficiency first to avoid precipitating subacute combined degeneration of the cord
What type of anaemia is caused by B12 deficiency?
Macrocytic anaemia
High uric acid + renal impairment following chemotherapy. What is the diagnosis?
Tumour lysis syndrome
What is the stereotypical history of G6PD deficiency?
Greek boy develops pallor and jaundice after having a lower respiratory tract infection. He has a history of neonatal jaundice
What is the management of a vaso-occlusive sickle cell crisis?
- The main components for managing sickle cell crisis should be analgesia, oxygen, and IV fluids
- You can also consider antibiotics if you suspect an infection, and transfusion if the Hb is low
What is the first line treatment for polycythaemia vera?
Venesection
Grossly elevated APTT + normal factor VIIIc activity points to what diagnosis?
Haemophilia B
What is the first line treatment for autoimmune haemolytic anaemia?
Corticosteroids
What is the platelet transfusion threshold for patients with severe bleeding or bleeding at critical sites e.g. CNS?
Maximum < 100 x 10^9
What is the first-line treatment for ITP?
Oral prednisolone
What are the diagnostic criteria for tumour lysis syndrome?
Increased serum creatinine, a cardiac arrhythmia or a seizure to have occurred
Which type of Hodgkin’s lymphoma carries the worst prognosis?
Lymphocyte depleted
What is the mechanism of action of dabigatran?
Direct thrombin inhibitor
What blood results are associated with sickle cell anaemia?
Normocytic anaemia with raised reticulocyte count
DVT investigation: if the scan is negative, but the D-dimer is positive, what do you do next?
Stop anticoagulation and repeat scan in 1 week
What is the first line management for flares of acute intermittent porphyria?
IV haem arginate
What are the complications of CLL?
- Anaemia
- Hypogammaglobulinaemia leading to recurrent infections
- Warm autoimmune haemolytic anaemia
- Richter’s transformation
What is the most common type of Hodgkin’s lymphoma?
Nodular sclerosing
Describe the presentation of lead poisoning
Abdominal pain, constipation, neuropsychiatric features, basophilic stippling
What is the treatment of beta-thalassaemia major?
Life-long blood transfusions
What is the single most important factor in determining whether cryoprecipitate should be given?
Low fibrinogen level
What proportion of patients with multiple myeloma have negative serum protein electrophoresis?
10%
What is the platelet transfusion threshold for patients not bleeding or having an invasive procedure?
10 x 10^9
Patient meets the threshold for platelet transfusion. Name 4 conditions which would be a contraindication
- Chronic bone marrow failure
- Autoimmune thrombocytopenia
- Heparin-induced thrombocytopenia
- Thrombotic thrombocytopenic purpura
What is the RBC transfusion threshold for patients without ACS?
70 g/L
Describe the presentation of G6PD deficiency
- Neonatal jaundice is often seen
- Intravascular haemolysis
- Gallstones are common
- Splenomegaly may be present
What findings on blood film are associated with G6PD deficiency?
Heinz bodies, bite and blister cells
What is the management of a patient due for surgery who is found to have iron deficiency anaemia?
Oral iron if > 6 weeks
IV iron if < 6 weeks
What is the first line imaging in suspected multiple myeloma?
Whole body MRI
Men of any age with a Hb below what level should be referred for upper and lower GI endoscopy as a 2ww?
110g/L
Sickle cell anaemia: sudden anaemia and low reticulocyte count. What is the diagnosis?
Parovirus
What blood film results are associated with CLL?
Lymphocytosis, smudge cells
What is the management of lntiphospholipid syndrome in pregnancy?
Aspirin + LMWH (e.g. enoxaparin)
Prosthetic heart valves may result in what type of anaemia?
Haemolytic anaemia
What is the recommended treatment for post-thrombotic syndrome?
Compression stockings
Child with unexplained petechiae or hepatosplenomegaly. What next?
Refer for immediate specialist assessment
What Wells score indicates DVT is likely? What does this mean?
- 2 or more
- Proximal leg vein ultrasound within 4 hours
- If delay in US - interim therapeutic anticoagulation
What Wells score indicates DVT is unlikely? What does this mean?
- 1 or less
- Peform D dimer
What is the management of acute haemolytic transfusion reaction?
Stop the transfusion and give IV fluids
Initial blood tests show a macrocytic anaemia with hypersegmented neurophils. Which investigation will most likely confirm the diagnosis?
Haemanitics (including B12 and folated)
What findings on blood film are associated with iron deficiency anaemia?
Target cells, pencil poikilocytes
What findings on blood film are associated with intravascular haemolysis?
Schistocytes
How does factor V Leiden increase the risk of venous thromboembolic events?
Activated factor V is inactivated 10 times more slowly by activated protein C than normal
A 23-year-old woman presents with lethargy. Blood tests show a microcytic anaemia with a disproprotionately low microcytosis.
What is the most likely diagnosis?
Beta-thalassaemia trait
Describe the presentation of thrombotic thrombocytic purpura
Presents with a pentad of fever, neuro signs, thrombocytopenia, haemolytic anaemia and renal failure
A 32 year male with leukaemia attends the day unit for a blood transfusion. Five days after the transfusion he attends A&E with a temperature of 38.5, erythroderma and desquamation.
What is the most likely diagnosis?
Graft vs. host disease
What drugs can cause aplastic anaemia?
Cytotoxics, chloramphenicol, sulphonamides, phenytoin, gold
What biochemical pattern is seen in myeloma?
High calcium, normal/high phosphate and normal alkaline phosphate
Assuming no mets
What is the most common cause of an isolated thrombocytopenia?
ITP
What lymph nodes is ovarian cancer most likely to spread to?
Para-aortic lymphatics
What are the risk factors for lymphoma?
- Elderly
- Caucasians
- History of viral infection (specifically Epstein-Barr virus)
- Family history
- Certain chemical agents (pesticides, solvents)
- History of chemotherapy or radiotherapy
- Immunodeficiency (transplant, HIV, diabetes mellitus)
- Autoimmune disease (SLE, Sjogren’s, coeliac disease)
How quickly should a unit of RBC be transfused in a non-urgent scenario?
90-120 mins
What finding on haemoglobin electrophoresis is associated with beta thalassaemia major?
HbA2
Describe the presentation of acute intermittent porphyria
- Abdominal: abdominal pain, vomiting
- Neurological: motor neuropathy
- Psychiatric: e.g. depression
- Hypertension and tachycardia common
What findings on urinalysis is seen in acute intermittent porphyria?
- Classically urine turns deep red on standing
- Raised urinary porphobilinogen
Burkitt’s lymphoma is associated with what genetic change?
C-myc gene translocation
Why would you use irradiated blood products?
Irradiated blood products are used to avoid transfusion-associated graft versus host disease by destroying T cells
Myelodysplastic syndrome can progress to which form of leukaemia?
AML
What are the components of cryoprecipitate?
Factor VIII, fibrinogen, von Willebrand factor, and Factor XIII
What is the drug treatment for CML?
Imatinib (tyrosine kinase inhibitor)
What chromosome is present in more than 95% of patients with CML?
- It is due to a translocation between the long arm of chromosome 9 and 22 - t(9:22)(q34; q11)
- This results in part of the ABL proto-oncogene from chromosome 9 being fused with the BCR gene from chromosome 22
- The resulting BCR-ABL gene codes for a fusion protein that has tyrosine kinase activity in excess of normal
As a first line investigation, all people with iron deficiency anaemia should be screened for what condition?
Coeliac disease
What are the features of multiple myeloma?
CRAB: hyperCalcaemia, Renal failure, Anaemia (and thrombocytopenia) and Bone fractures/lytic lesions
Deranged coagulation in sepsis indicates what diagnosis?
DIC
Transmission of which type of infection is most likely to occur following a platelet transfusion?
Bacterial
Platelet transfusions are at particular risk of bacterial contamination as they are stored at room temperature
