Haemotology: Passmed Flashcards

1
Q

What do malignancies increase the risk for?

A

Venous thromboembolism

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2
Q

Which autoimmune conditions can cause lymphadenopathy?

A

Systemic lupus and rheumatoid arthritis

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3
Q

Which drugs cause lymphadenopathy?

A

Phenytoin and allopurinol

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4
Q

How is the clotting cascade regulated?

A

Positive feedback mechanism
-> Clotting factors promote the recruitment of other clotting factors

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5
Q

What are the features of Reed-Sternberg cells?

A

Large multinucleated cells with prominent eosinophils.

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6
Q

What cancer is associated with chromosomal translocation between 11 and 14?

A

Mantle cell lymphoma

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7
Q

What cancer is associated with chromosomal translocation between 14 and 18?

A

Follicular lymphoma, caused by an increased BCL-2

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8
Q

What cancer is associated with chromosomal translocation between 8 and 14?

A

Burkitt’s lymphoma, due to MYC oncogene

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9
Q

What cancer is associated with chromosomal translocation between 15 and 17?

A

Acute promyelocytic leukaemia, caused by a fusion of PML-RAR genes

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10
Q

What are the treatments for CML?

A

Imatinib
Alternatively, hydroxyurea, interferon-alpha and Allogenic bone marrow transplant

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11
Q

What are Hassall’s corpuscles?

A

Concentric ring of epithelial cells in the medulla of thymus.

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12
Q

Cancer causing a neoplasm of the plasma cells?

A

Multiple myeloma, characterised by raised PTH-rep due to malignancy assoicated with paraneoplastic syndrome.

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13
Q

Which cancers commonly metastasise?

A

Prostate cancer
Breast cancer
Lung cancer
Thyroid cancer
Renal cancer

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14
Q

What is the common site of metastases?

A

Spine
Pelvis
Ribs
Skull
Long bones

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15
Q

What is the common site of metastasis for the prostate?

A

Baston’s plexus of the prostate is a valveless structure associated with metastasis to the bone in prostate cancer, and bladder and breast.

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16
Q

How do the immune checkpoint inhibitors work?

A

Immune checkpoint inhibitors reactivate and increase the body’s own T cell population. These act by blocking cytotoxic T lymphocytes associated protein (CTLA) and programmed death ligand (PDL).

Side effects from the drugs related to boosting the number of immune cells, causing dry skin, nausea, reduced appetite and diarrhoea

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17
Q

What is CLL caused by?

A

Chronic lymphocytic leukaemia is caused by an abnormal monoclonal proliferation of B lymphocytes. It is characterised by smudge cells and immunophenotyping with CD5, CD19, CD20 and CD23.

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18
Q

What is Rouleaux formation caused by?

A

Clumps of RBC which can occur due to:
Stasis of blood
Infection
Increase in fibrinogen and globulin
Presence of plasma proteins

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19
Q

What can trigger Tumour lysis syndrome?

A

Chemotherapy and use of steroids.

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20
Q

What is the body’s stores of B12?

A

2-3mg for up to 2-4 years

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21
Q

What is a complication of cancer therapy?

A

Neutropenia sepsis that occurs 7-14 days after chemotherapy where there is a high temperature, lethargy and myalgia and is typically caused by staphylococcus epididermis.

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22
Q

What is the management of neutropenic sepsis?

A

NICE therapy recommends immediate antibiotics and urgent admission to hospital.

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23
Q

What is a complication for blood transfusions with thalassemia?

A

Iron overload and organ failure, therefore to prevent iron overload, Desferrioxamine is prescribed.

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24
Q

Which conditions increase the risk of venous thromboembolism?

A

Polycythaemia, sickle cell disease
Malignancy
Thrombophlebitis
Heart failure
Anti phospholipid syndrome
Antipsychotics like olanzapine

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25
Q

What increases the risk of of anaphylaxis for blood transfusions?

A

IgA deficiency

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26
Q

What is the cause of transfusion-related acute lung injury?

A

Host neutrophil activation against donated blood products, presenting with hypoxia, fever and hypotension.

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27
Q

What are the Vitamin K dependent substances?

A

Protein C
Factor 2, 7, 9 and 10

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28
Q

What is the management of CML?

A

Imatinib
Hydroxyurea
Interferon-alpha
Allogenic bone marrow transplant

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29
Q

What does heparin inhibit?

A

Factor 2,9, 10 and 11.

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30
Q

What is the role of protein C?

A

It is synthesised in the liver and is important in anticoagulation. Protein C is activated by thrombin binding to thrombomodulin and Protein S acts as a co-factor for anti-coagulation activity.

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31
Q

What are the NICE guidelines for providing blood transfusion?

A

Threshold for blood transfusion is 7g/dl

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32
Q

Why does metastatic calcification occur?

A

Due to hypercalcaemia

33
Q

What is the risk of large of massive transfusions?

A

Assoicated with reduced levels of factor 5 and 8 and risk of thrombocytopenia.

34
Q

What is the most common thrombophilia?

A

Activated protein C resistance by factor V which leads to excessive blood clotting. It is the most common inheritable cause of DVT.

35
Q

What is an example of an acquired thrombophilia?

A

Antiphospholipid syndrome, an autoimmune disease which leads to a hyperocagulable state.

36
Q

What do dacrocytes RBCs indicate?

A

Teardrop cells indicate Myelofibrosis

37
Q

What are Howell jolly bodies?

A

Erythrocytes contain basophils remnants due to an issue with maturation in the bone marrow. This occurs due to hyposplenism.

38
Q

What are Heinz bodies?

A

RBCs containing denatured haemoglobin due to:
glucose-6-phosphate deficiency which begins with a bite cell, abnormally shaped RBC due to removal of denatured haemoglobin
Alpha-thalassemia.

39
Q

What causes pencil shaped RBCs?

A

Iron deficiency anaemia

40
Q

What causes hypersegmneted neutrophils?

A

Megaloblastic anaemia

41
Q

What is basophilic stippling?

A

Basophilic granules in the RBCs, typically due to impaired haemoglobin synthesis with:
Sideroblastic anaemia
Lead poisoning
Thalassemia
Myelodysplasia

42
Q

What cause RBCs to appear as target cells?

A

Excess RBC membrane that occurs with:
Iron-deficiency anaemia
Occurs after
Liver disease
Sickle cell anaemia

43
Q

What is the role of haptoglobin?

A

Binds to free haemoglobin released from broken down RBCs to prevent the potential oxidative damage from haem, produced by the liver.

It reduces during intravascular haemolysis.

44
Q

What is the most common cause of B12 deficiency?

A

Pernicious anaemia

45
Q

Which cells are predominant in chronic inflammation?

A

Mononuclear cells such as macrophages, lymphocytes and plasma cells.

46
Q

Which clotting factors are most commonly consumed in disseminated intravascular coagulation?

A

Factor V and VIII.

47
Q

What are the features of lead poisoning?

A

Abdominal pain, constipation with motor peripheral neuropathy and blue lines on the gym margin.

48
Q

Which chromosomal translocation causes Burkitt’s lymphoma?

A

Chromosomal translocation between 8 and 14.

49
Q

Which chromosomal translocation is assoicated with follicular lymphoma?

A

Translocation between 14 and 18, causing increased BCL2.

50
Q

What is the most common adult leukaemia?

A

Acute myeloid leukaemia is the most aggressive and Chronic lymphoid leukaemia.

51
Q

Which antibody is found on the surface of B lymphocytes?

A

IgD.

52
Q

What is the genotype for moderate sickle cell disease?

A

HbScc where there is sickle cell disease with beta thalassemia.

53
Q

What are the antibodies in anti phospholipid syndrome?

A

Lupus anticoagulant
Anti-cardiolipin
Anti-beta 2 glycoprotein

54
Q

Which substances in the body reduce platelet activation?

A

Prostacyclin
Nitric oxide
Endothelial ATPase

55
Q

What is the cause of thrombotic thromocytopenic purpura?

A

There is a failure to cleave the abnormally large vWF which causes platelets to clamp together.This occurs post-infection, with tumours and in pregnancy and lupus.

56
Q

Which antibody mediates cold haemolytic anaemia?

A

IgM and occurs due to neoplasia or infections.

57
Q

Which antibody mediates warm haemolytic anaemia?

A

IgG.

58
Q

Which type of haemolytic anaemia is associated with chronic lymphoid leukaemia?

A

Warm autoimmune haemolytic anaemia.

59
Q

What is used to diagnose hereditary spherocytosis?

A

Osmotic fragility test

60
Q

Which cancers are the BRCA1 mutation associated with?

A

Breast and ovarian cancer.

61
Q

Which clotting factor is not synthesised by hepatocytes?

A

Factor VIII, which are synthesised in the endothelial cells of the liver.

62
Q

Which clotting factors are affected in DIC?

A

Factor I, II, V, VIII and XI.

63
Q

Which clotting factors are inhibited by heparin?

A

Factor 2, 9, 10 and 11.

64
Q

Which blood product commonly causes an urticarial reaction?

A

Fresh frozen plasma

65
Q

Which condition can cause rupturing of the spleen?

A

Epstein-Barr Virus

66
Q

Which genetic mutation is associated with marginal cell lymphoma?

A

Chromosomal translocation between 11 and 18, which is an indolent non-Hodgkin’s lymphoma that commonly manifests as MALT in the gut, urogenital tract, breast and endocrine tissue.

67
Q

What are the features of mantle cell lymphoma?

A

Chromosomal translocation between 11 and 14 with translocation of the heavy chain immunoglobulin and cyclin D. It causes fever, weight loss, night sweats and enlarged lymph nodes.

68
Q

What is the risk with transfusing out of date products?

A

Hyperkalemia, due to lysis of RBCs.

69
Q

Which antibody is associated with hyperviscosity?

A

IgM.

70
Q

What is an indicator for clotting factors dependent on Vitamin K?

A

PT time, for the extrinsic pathway.

71
Q

How are clotting results affected in haemophilia?

A

Bleeding time is normal because platelets are still able to adhere and aggregate together in response to ADP, but the ability to form a fibrin mesh is lowered, therefore:
Increased time in apTT due to either Factor VIII or IX are involved in intrinsic pathway
Normal PT time

72
Q

How are clotting results affected in Vitamin K deficiency?

A

Normal bleeding time because ability for platelets to respond to ADP and bind and adhesive are not affected, however:
Increased PT time due to Factor VII
Increased PTT time due to Factor IX

73
Q

How are clotting results affected in Von willebrand disease?

A

Increased bleeding time due to reduced platelet adhesive
Increased time in PT time due to reduced Factor VII activation
Normal PTT time

74
Q

How is a febrile reaction managed?

A

Slowing transfusion and administering paracetamol

75
Q

How is transfusion-associated circulatory overload?

A

Loop diuretic and slowing/stopping transfusion

76
Q

How does acute haemolytic transfusion reaction occur?

A

IgM mediated intravascular haemolysis due to blood group mismatch. Management includes fluid resuscitation and infusion termination.

Complications of acute haemolytic transfusion is renal failure and disseminated intravascular coagulation.

77
Q

What is the action of hyodxyurea?

A

Inhibits ribonucleotide reductase, part of the DNA structure. It is used in the treatment of sickle cell disease and CML.

78
Q

What is acute intermittent porphyria?

A

It is an autosomal dominant condition caused by a defect in haem biosynthesis which has an onset typically in 30s to 40s and presents with urine turning red on standing and abdominal pain, motor neuropathy, depression and hypertensions.

Management is avoiding triggers and treatment of acute attacks is IV haem arginate.

79
Q

What are the general complications with transfusion reactions?

A

Overload, causing pulmonary oedema
Urticaria
Infection
Transfusion-associated lung injury
Graft vs hot disease
Thrombophilia and neutrophilia