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
Q

Hyperviscosity syndrome is seen in patients with myeloma and can cause bleeding, name 3 other complications that can occur.

A

Visual sx - retinal haemorrhages
Neurological sx - stroke
Heart failure

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26
Q

Older age and fhx are two risk factors for multiple myeloma, name 3 more.

A

Male
Black ethnic origin
Obesity

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27
Q

Other than renal impairment, bone pain, anaemia and hypercalcaemia state 4 more features that should raise suspicion of myeloma.

A

Pathological fractures
Unexplained fatigue
Unexplained weight loss
Fever of unknown origin

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28
Q

What is meant by Bence Jones protein?

A

Free light chains in the urine

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29
Q

What investigations are carried out for suspected myeloma?

A

FBC
Calcium
ESR - increased
Plasma viscosity
U+Es
Serum protein electorphoresis
Serum-free light-chain assay
Urine protein electrophoresis

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30
Q

What is required to confirm the diagnosis of multiple myeloma?

A

Bone marrow biopsy

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31
Q

Which imaging modality is preferred for myeloma?

A

MRI (whole body)

32
Q

How might the bone disease seen in myeloma be managed?

A

Bisphosphonates (suppress osteoclast activity)
Radiotherapy
Orthopaedic surgery (stabilise bones)
Cement augmentation (this may improve spinal stability and pain)

33
Q

Infection, bone pain and fractures are common complications of myeloma and its treatment. State 6 more.

A

Renal failure
Hypercalcaemia
Peripheral neuropathy
Spinal cord compression
Hyperviscosity syndrome
Venous thromboembolism

34
Q

What is myelofibrosis?

A

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
Q

Teardrop-shaped red blood cells are a feature of myelofibrosis on blood films, name 2 more.

A

Anisocytosis (varying sizes of RBC)
Blasts (immature red and white cells)

36
Q

What are the mx options for primary myelofibrosis?

A

No active treatment
Supportive mx of sx
Chemotherapy (hydroxyurea) control of disease
Targeted therapies (JAK2 inhibitors)
Allogeneic stem cell transplantation

37
Q

Which haematological cancer does myelofibrosis have the potential to transform into?

A

AML

38
Q

What is the pathophysiology behind Immune thrombocytopenic purpura?

A

Antibodies are created against the platelets = thrombocytopenia

39
Q

What are the management options for ITP?

A

Prednisolone
IV immunoglobulins
Thrombopoietin receptor agonists
Rituximab
Splenectomy (removes the primary site of PLT destruction) - less common

40
Q

What are the 3 most common cancers that cause bone mets?

A

Prostate
Breast
Lung

41
Q

Older age, white ethnicity and fhx are 3 risk factors for the development of non-hodgkins lymphoma, name 5 more.

A

History of viral infection (especially EBV)
Certain chemicals (solvents and pesticides)
Chemo or radiotherapy hx
Immunodeficiency
Autoimmune disease

42
Q

What is the diagnostic investigation for Non-hodgkins lymphoma?

A

Excisional node biopsy

43
Q

What is the name of the staging for lymphoma?

A

Lugano staging

44
Q

Bone marrow infiltration can cause anaemia, thrombocytopaenia or neutropaenia, name 3 more complications of non-hodgkins lymphoma?

A

SVCO
Metastasis
Spinal cord compression

45
Q

When do B symptoms occur in non-hodgkins lymphoma?

A

Later on.

46
Q

Which grade of non-hodgkins lymphoma has a better prognosis?

A

Low grade better prognosis but high grade higher cure rate.

47
Q

What are the key components of mx for a sickle cell crisis?

A

Analgesia + oxygen + IV fluids

48
Q

How are women with antiphospholipid syndrome managed during pregnancy?

A

LMWH + aspirin

49
Q

Which cytotoxic agent can cause liver fibrosis, myelosuppression and oral mucositis?

A

Methotrexate

50
Q

Why are irridiated blood products used?

A

Depleted number of T-lymphocytes minimises the risk of graft vs host disease.

51
Q

How often do patients with sickle cell need the pneumococcal vaccine?

A

Every 5 years

52
Q

In what patients is HbA2 rasied?

A

Beta-thalassaemia major

53
Q

What is the firstline treatment for autoimmune haemolytic anaemia?

A

Corticosteroids

54
Q

What are the characteristic features of hyposplenism on blood film?

A

Howell-jolly bodies
Target cells

55
Q

What is basophillic stippling on blood film indicative of?

A

Sideroblastic anaemia

56
Q

At what point should a patient with clinically signifcant bleeding be given a platelet transfusion?

A

<30 x 10^9

57
Q

When should the threshold for platelet transfusion be higher - <100 x 10^9?

A

Severe bleeding
Bleeding at critcial sites (e.g. CNS)

58
Q

B symptoms are one of the signs of poor prognosis with Hodgkin’s lymphoma, name 5 more.

A

increasing age
male sex
stage IV disease
lymphocyte depleted subtype

59
Q

What is the immediate management steps for an acute haemolytic reaction?

A

STOP transfusion
IV fluids

60
Q

In a non-emergency setting how quickly should a unit of red blood cells be transfused?

A

90-120 mins (1.5-2 hours)

61
Q

What is the treatment for periductal mastitis?

A

Co-amoxiclav

62
Q

How can hodgkins lymphoma present on an FBC?

A

Eosinophilia
Normocytic anaemia

63
Q

Other than a cough and dyspnoea, state 3 more features of an acute chest syndrome.

A

Chest pain
Hypoxia
Pulmonary infiltrates (CXR)

64
Q

What is haptoglobin and what happens to haptoglobin levels when there is haemolysis?

A

Haptoglobin binds to free Hb in the blood.
When there is haemolysis Hb is released into the blood = low haptoglobin.

65
Q

What is the main treatment option for patients with beta thalassaemia major?

A

Life-long blood transfusion

66
Q

What is the key electrolyte disturbance that can occur during a transfusion? Why is it dangerous?

A

Hyperkalaemia
Arrhythmias

67
Q

What is the universal donor of fresh frozen plasma?

A

AB negative

68
Q

What is Richter’s transformation?

A

When CLL transforms into Non-hodgkins lymphoma.

69
Q

What does a prolonged APTT and a normal PT indicate?

A

Problem with the intrinsic pathway (e.g. haemophilia B - factor VIII deficiency)

70
Q

What type of anaemia is indicative of alcoholism? What other abnormality is observed on FBC?

A

Macrocytic anaemia
Thrombocytopaenia

71
Q

What medication is given to patients with polycythaemia vera to decrease the risk of clotting?

A

Aspirin

72
Q

What drugs can trigger haemolysis in a patient with G6PD deficiency?

A

anti-malarials: primaquine
ciprofloxacin
sulph- group drugs: sulphonamides, sulphasalazine, sulfonylureas

73
Q

Over what time should a non-urgent blood transfusion (asymptomatic) be administered over? When would you consider increasing this time?

A

90-120 mins
If the patient had heart failure as they are at risk of fluid overload.

74
Q

What is the most likely reason for pancytopenia after chemo or radiotherapy?

A

Myelodysplastic syndrome

75
Q

What condition is livedo reticularis a cutaneous manifestation of?

A

Antiphospholipid syndrome

76
Q

What are the poor prognostic factors for ALL?

A

age < 2 years or > 10 years
WBC > 20 * 109/l at diagnosis
T or B cell surface markers
non-Caucasian
male sex