Haematology Flashcards

1
Q

Which chromosome is present in more than 95% of patients with chronic myeloid luekaemia?

A

Philadelphia (9 and 22)

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2
Q

Other than lethargy and weight loss, what are two other presenting features of CML?

A

Abdominal discomfort (splenomegaly)
Excessive sweating (night sweats)

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3
Q

What would be seen on the blood results of a patient with CML?

A

Anaemia
Leucocytosis
+/- thrombocytosis

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4
Q

What would be seen on a blood film of CML?

A

Increased granulocytes at different stages of maturation.

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5
Q

What is the management of CML?

A

Imatinib (tyrosine-kinase inhibitor specifically BCR-ABL)
Hydroxyurea
Interferon-alpha
Allogenic bone marrrow transplant

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6
Q

Pruritis, splenomegaly and hypertension are 3 features of Polycythaemia Vera, name 3 more.

A

Hyperviscosity
Haemorrhage
Low ESR

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7
Q

What investigations should be performed for PV?

A

FBC - raised haematocrit, basophils, neutrophils and platelets in most patients.
JAK2
Serrum ferritin
Renal and liver function tests

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8
Q

How is PV managed?

A

Aspirin - reduces risk of thrombotic events
Venesection - firstline treatment to keep the Hb in the normal range.
Chemotherapy - hydroxyurea and phosphorus-32 therapy.

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9
Q

What can PV progress to?

A

5-15% progress to Myelofibrosis
5-15% progress to AML

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10
Q

Autoimmune disease and idiopathic are 2 of the causes of warm autoimmune haemolytic anaemia, name 2 more.

A

Neoplasia: lymphoma and CLL
Drugs: methylodopa, levodopa, cephalosporins and nitrofuratoin.

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11
Q

What is the management of warm autoimmune haemolytic anaemia?

A

Treatment of underlying disorder
Steroids (+/- rituximab) firstline.

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12
Q

Anaemia and reticulocytosis are 2 features of AIHA, name 3 more.

A

Low haptoglobin
Raised LDH
Raised unconjugated bilirubin

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13
Q

What would be seen on a blood film of a patient with AIHA?

A

Spherocytes
Reticulocytes

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14
Q

What is the reversal agent for dabigatran?

A

Idarucizumab

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15
Q

What is the reversal agent for rivaroxaban and apixaban?

A

Andexanet alfa

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16
Q

What is the most common type of lymphoma in the UK?

A

Diffuse large B cell (non-hodgkins lymphoma)

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17
Q

What is Monoclonal Gammaopathy of undetermined significance?

A

Production of a specific paraprotein without other features of myeloma or cancer.

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18
Q

What is the risk of progression from MGUS to myeloma?

A

1% per year.

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19
Q

What are the 4 most common features of myeloma?

A

Elevated Calcium
Renal impairment
Anaemia
Bone lesions and pain

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20
Q

What results in the bone pain seen in myeloma?

A

Cytokines released from abnormal plasma cells = Increase in osteoclast activity and decrease in osteoblast activity = bone metabolism becomes imbalanced = bone resorption.

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21
Q

The skull and spine are two common sites for myeloma bone disease, name 2 more.

A

Long bones
Ribs

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22
Q

What kind of bony lesions are seen in myeloma?

A

Osteolytic lesions

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23
Q

What is a plasmacytoma?

A

Individual tumour formed by cancerous plasma cells, they can occur in the bones and replace normal bone tissue, or in the soft tissues.

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24
Q

Paraproteins being deposited in the kidneys is one of the reasons for the renal impairment seen in myeloma, name 4 other reasons.

A

Hypercalcaemia affecting kidney function
Dehydration
Glomerulonephritis
Medications used to treat the myeloma

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25
Hyperviscosity syndrome is seen in patients with myeloma and can cause bleeding, name 3 other complications that can occur.
Visual sx - retinal haemorrhages Neurological sx - stroke Heart failure
26
Older age and fhx are two risk factors for multiple myeloma, name 3 more.
Male Black ethnic origin Obesity
27
Other than renal impairment, bone pain, anaemia and hypercalcaemia state 4 more features that should raise suspicion of myeloma.
Pathological fractures Unexplained fatigue Unexplained weight loss Fever of unknown origin
28
What is meant by Bence Jones protein?
Free light chains in the urine
29
What investigations are carried out for suspected myeloma?
FBC Calcium ESR - increased Plasma viscosity U+Es Serum protein electorphoresis Serum-free light-chain assay Urine protein electrophoresis
30
What is required to confirm the diagnosis of multiple myeloma?
Bone marrow biopsy
31
Which imaging modality is preferred for myeloma?
MRI (whole body)
32
How might the bone disease seen in myeloma be managed?
Bisphosphonates (suppress osteoclast activity) Radiotherapy Orthopaedic surgery (stabilise bones) Cement augmentation (this may improve spinal stability and pain)
33
Infection, bone pain and fractures are common complications of myeloma and its treatment. State 6 more.
Renal failure Hypercalcaemia Peripheral neuropathy Spinal cord compression Hyperviscosity syndrome Venous thromboembolism
34
What is myelofibrosis?
Proliferation of a single cell line leading to bone marrow fibrosis, where bone marrow is replaced by scar tissue due to the cytokines released from the proliferating cells.
35
Teardrop-shaped red blood cells are a feature of myelofibrosis on blood films, name 2 more.
Anisocytosis (varying sizes of RBC) Blasts (immature red and white cells)
36
What are the mx options for primary myelofibrosis?
No active treatment Supportive mx of sx Chemotherapy (hydroxyurea) control of disease Targeted therapies (JAK2 inhibitors) Allogeneic stem cell transplantation
37
Which haematological cancer does myelofibrosis have the potential to transform into?
AML
38
What is the pathophysiology behind Immune thrombocytopenic purpura?
Antibodies are created against the platelets = thrombocytopenia
39
What are the management options for ITP?
Prednisolone IV immunoglobulins Thrombopoietin receptor agonists Rituximab Splenectomy (removes the primary site of PLT destruction) - less common
40
What are the 3 most common cancers that cause bone mets?
Prostate Breast Lung
41
Older age, white ethnicity and fhx are 3 risk factors for the development of non-hodgkins lymphoma, name 5 more.
History of viral infection (especially EBV) Certain chemicals (solvents and pesticides) Chemo or radiotherapy hx Immunodeficiency Autoimmune disease
42
What is the diagnostic investigation for Non-hodgkins lymphoma?
Excisional node biopsy
43
What is the name of the staging for lymphoma?
Lugano staging
44
Bone marrow infiltration can cause anaemia, thrombocytopaenia or neutropaenia, name 3 more complications of non-hodgkins lymphoma?
SVCO Metastasis Spinal cord compression
45
When do B symptoms occur in non-hodgkins lymphoma?
Later on.
46
Which grade of non-hodgkins lymphoma has a better prognosis?
Low grade better prognosis but high grade higher cure rate.
47
What are the key components of mx for a sickle cell crisis?
Analgesia + oxygen + IV fluids
48
How are women with antiphospholipid syndrome managed during pregnancy?
LMWH + aspirin
49
Which cytotoxic agent can cause liver fibrosis, myelosuppression and oral mucositis?
Methotrexate
50
Why are irridiated blood products used?
Depleted number of T-lymphocytes minimises the risk of graft vs host disease.
51
How often do patients with sickle cell need the pneumococcal vaccine?
Every 5 years
52
In what patients is HbA2 rasied?
Beta-thalassaemia major
53
What is the firstline treatment for autoimmune haemolytic anaemia?
Corticosteroids
54
What are the characteristic features of hyposplenism on blood film?
Howell-jolly bodies Target cells
55
What is basophillic stippling on blood film indicative of?
Sideroblastic anaemia
56
At what point should a patient with clinically signifcant bleeding be given a platelet transfusion?
<30 x 10^9
57
When should the threshold for platelet transfusion be higher - <100 x 10^9?
Severe bleeding Bleeding at critcial sites (e.g. CNS)
58
B symptoms are one of the signs of poor prognosis with Hodgkin's lymphoma, name 5 more.
increasing age male sex stage IV disease lymphocyte depleted subtype
59
What is the immediate management steps for an acute haemolytic reaction?
STOP transfusion IV fluids
60
In a non-emergency setting how quickly should a unit of red blood cells be transfused?
90-120 mins (1.5-2 hours)
61
What is the treatment for periductal mastitis?
Co-amoxiclav
62
How can hodgkins lymphoma present on an FBC?
Eosinophilia Normocytic anaemia
63
Other than a cough and dyspnoea, state 3 more features of an acute chest syndrome.
Chest pain Hypoxia Pulmonary infiltrates (CXR)
64
What is haptoglobin and what happens to haptoglobin levels when there is haemolysis?
Haptoglobin binds to free Hb in the blood. When there is haemolysis Hb is released into the blood = low haptoglobin.
65
What is the main treatment option for patients with beta thalassaemia major?
Life-long blood transfusion
66
What is the key electrolyte disturbance that can occur during a transfusion? Why is it dangerous?
Hyperkalaemia Arrhythmias
67
What is the universal donor of fresh frozen plasma?
AB negative
68
What is Richter's transformation?
When CLL transforms into Non-hodgkins lymphoma.
69
What does a prolonged APTT and a normal PT indicate?
Problem with the intrinsic pathway (e.g. haemophilia B - factor VIII deficiency)
70
What type of anaemia is indicative of alcoholism? What other abnormality is observed on FBC?
Macrocytic anaemia Thrombocytopaenia
71
What medication is given to patients with polycythaemia vera to decrease the risk of clotting?
Aspirin
72
What drugs can trigger haemolysis in a patient with G6PD deficiency?
anti-malarials: primaquine ciprofloxacin sulph- group drugs: sulphonamides, sulphasalazine, sulfonylureas
73
Over what time should a non-urgent blood transfusion (asymptomatic) be administered over? When would you consider increasing this time?
90-120 mins If the patient had heart failure as they are at risk of fluid overload.
74
What is the most likely reason for pancytopenia after chemo or radiotherapy?
Myelodysplastic syndrome
75
What condition is livedo reticularis a cutaneous manifestation of?
Antiphospholipid syndrome
76
What are the poor prognostic factors for ALL?
age < 2 years or > 10 years WBC > 20 * 109/l at diagnosis T or B cell surface markers non-Caucasian male sex