Theme 7: Haematology Flashcards

1
Q

Which physiological developmental process gives rise to the cellular components of blood?

A

Haemopoiesis

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2
Q

Which type of cell replication increases the stem cell pool, generating no differentiated progeny?

A

Symmetrical self-renewal

Stem cell divides forming 2 daughter stem cells

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3
Q

With reference to haemopoietic stem cells, what is asymmetric self-renewal?

A

Division maintains the stem cell pool (no increase, no decrease). This is achieved by forming one stem cell and one differentiated cell.

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4
Q

With reference to haemopoietic stem cells, what is lack of self-renewal?

A

Can mean 2 things:

  • maintain the stem cell pool, no division takes place
  • deplete the stem cell pool, each division generates 2 differentiated daughter cells
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5
Q

Which haemopoietic stem cell lineage produces erythrocytes?

A

Myeloid

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6
Q

Which haemopoietic stem cell lineage produces platelets?

A

Myeloid

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7
Q

Which haemopoietic stem cell lineage produces B-lymphocytes?

A

Lymphoid

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8
Q

Which haemopoietic stem cell lineage produces basophils?

A

Myeloid

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9
Q

Which haemopoietic stem cell lineage produces T-lymphocytes?

A

Lymphoid

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10
Q

Haemopoiesis starts at day 27 in the __1__ region, expands rapidly at day 35 and disappears at day __2__.

A

1) aorta gonad mesonephros (AGM)

2) 40

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11
Q

The disappearance of the aorta gonad mesonephros region at day 40 coincides with what event?

A

Migration of the haemopoietic stem cells into the foetal liver, which becomes the new site of haemopoiesis

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12
Q

Reduced erythrocyte count is called what?

A

Anaemia

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13
Q

What term describes a state of raised RBC count?

A

Polycythaemia

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14
Q

What term describes a state where the % of RBC in the blood is raised because the volume of plasma is reduced?

A

Relative polycythaemia

The RBC count isn’t raised, but as the plasma volume has dropped it appears to be high in comparison with the plasma

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15
Q

What is the most common white cell in adults?

A

Neutrophils

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16
Q

Which of the following facts about neutrophils is incorrect?

a) They are phagocytic
b) They live for only a few hours in the blood
c) Malignant conditions (such as myelodysplasia) can increase the number of lobes on the nucleus
d) Numbers are usually raised in response to a bacterial infection

A

c) Malignant conditions (such as myelodysplasia) can increase the number of lobes on the nucleus

Neutrophil have multi-lobed nuclei. The number of lobes can be:

  • reduced in malignant conditions (e.g. myelodysplasia); or,
  • increased (hypersegmented neutrophils) in anaemia, B12 deficiency, folate deficiency.
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17
Q

What term means:

Raised number of neutrophils

A

Neutrophilia

Causes include bacterial infection and inflammation

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18
Q

What term means:

Decreased number of neutrophils

A

Neutropenia

Causes include drug side effects

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19
Q

Which WBCs are granulocytes?

A

Neutrophils, eosinophils, basophils

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20
Q

Which granulocyte has a bi-lobed nucleus?

A

Eosinophil

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21
Q

What effect might a parasitic infection have on numbers of eosinophils?

A

Cause eosinophilia (increased number)

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22
Q

Which granulocyte has a dark cytoplasm with lots of granules?

A

Basophils

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23
Q

What effect can chronic myeloid leukaemia have on numbers of basophils?

A

CML can cause basophilia: increased numbers

Raised basophil count is diagnostic of CML. This is because it is the only condition that causes basophilia. It also increases the numbers of the other myeloid lineage, but other things can also cause that.

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24
Q

What is the difference between a monocyte and a macrophage?

A

They are the same cells, but monocytes are in circulation and become known as macrophages or histiocytes when they enter the tissues.

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25
Q

What are Kupffer cells?

A

Macrophages in the liver

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26
Q

What are Langerhans cells?

A

Macrophages in the skin

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27
Q

What effect might TB have on numbers of monocytes?

A

Monocytosis: increased numbers of monocytes

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28
Q

Which WBCs are phagocytic & antigen-presenting cells?

A

Monocytes

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29
Q

Which of the following is likely to lead to lymphopenia?

a) Glandular fever
b) Bone marrow transplant
c) Chronic lymphocytic leukaemia
d) Pertussis

A

b) Bone marrow transplant

It can take years for lymphocyte numbers to normalise following a bone marrow transplant as they generate quite slowly.

The other conditions would all lead to lymphocytosis.

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30
Q

Which type of lymphocyte(s) form part of the innate immune system?

A

Natural killers (NK)

These lymphocytes identify self and non-self; launching an attack on non-self.

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31
Q

Which lymphocytes include multiple subtypes, such as CD4 and CD8?

A

T-lymphocytes

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32
Q

Are T-lymphocytes part of the innate immune system or the adaptive immune system?

A

Adaptive

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33
Q

Are B-lymphocytes part of the innate immune system or the adaptive immune system?

A

Adaptive

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34
Q

Platelets are derived from __?__ in the bone marrow

A

Megakaryocytes

Fragments of these large cells break off and enter circulation as platelets.

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35
Q

What results are included with a FBC?

A
  • Haemoglobin concentration
  • Red cell parameters: mean cell volume (MCV) and mean cell Hb (MCH)
  • WCC
  • Platelet count
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36
Q

What three things are assayed as part of the coagulation screen?

A

Prothrombin time
Thrombin time
Activated partial thromboplastin time

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37
Q

Which test involves hollow-needle biopsy from the posterior iliac crest of the pelvis under local anaesthetic?

A

Bone marrow aspirate and trephine

Liquid marrow is aspirated and then a core is removed using a trephine (a cylindrical hole saw used in surgery to remove a circle of tissue or bone)

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38
Q

What term is used to describe the proportion of abnormal results correctly classified by a test?

A

Sensitivity

Sensitivity (also called the true positive [sick] rate) measures the proportion of actual positives that are correctly identified as such (e.g., the percentage of sick people who are correctly identified as having the condition)

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39
Q

What is meant by specificity of a test?

A

Specificity (also called the true negative rate) measures the proportion of actual negatives that are correctly identified as such (e.g., the percentage of healthy people who are correctly identified as not having the condition)

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40
Q

Which type of anaemia is associated with a low MCV and low MCH?

A

Microcytic hypochromic anaemia

The RBCs will appear small and pale.

Low MCV = small size
Low MCH = low Hb level = pale

Examples: iron deficiency, thalassaemia, anaemia of chronic disease, lead poisoning, sideroblastic anaemia

41
Q

Which type of anaemia is associated with a normal MCV and normal MCH?

A

Normocytic normochromic anaemia

RBCs appear normal, but their numbers are depleted.

Examples: Haemolytic anaemia, anaemia of chronic disease, acute haemorrhage, renal disease, mixed deficiencies, bone marrow failure (inc. chemo, malignancy)

42
Q

Which type of anaemia is associated with a high MCV?

A

Macrocytic

Two types: megaloblastic and non-megaloblastic

  • Megaloblastic: B12/folate deficiency
  • Non-megaloblastic: alcohol abuse, liver disease, myelodysplasia, aplastic anaemia
43
Q

What is the number one cause of macrocytic anaemia?

A

Alcohol abuse

44
Q

What is the characteristic appearance of erythrocytes in iron-deficiency anaemia?

A

Small and pale (low MCV, low MCH)

Varying shape and size: pencil cells - long and thin

45
Q

Which type of anaemia results in hypersegmented neutrophils and oval macrocytes?

A

B12 deficiency

46
Q

At what temperature are RBCs stored?

A

4 degrees C

47
Q

At what temperature are platelets stored?

A

22 degrees C (room temp)

48
Q

At what temperature is FFP stored?

A

-30 degrees C

49
Q

How are platelets collected?

A

Two ways:

1) Pooled platelets: 1 unit of platelets is removed from a unit of whole blood. 4-6 of these units are pooled together from different donors into a single pack
2) Apheresis platelets: Donor’s blood passes through apheresis machine which removes the platelets and returns all other blood products to the donor. This method takes a full pack of platelets from a single donor (equivalent to 4-6 units of pooled platelets)

50
Q

What is the adult therapeutic dose for platelets?

A

4-6 units from random donors (pooled platelets) or a single donation from apheresis

51
Q

A single dose of platelets would be expected to increase patient’s count by how much?

A

20-60x10^9 per L

52
Q

Which product contains all clotting factors at physiological levels?

A

Fresh frozen plasma (FFP)

53
Q

Which product contains high concentration of vitamin K-dependent factors?

A

Prothrombin complex concentrate (factor IX complex)

Contains high concentrations of factors II, VII, IX, and X

54
Q

What is cryoprecipitate?

A

Blood product extracted from FFP during the freezing process.

Contains fibrinogen, vWF, factor VIII, and factor XIII

55
Q

Which of the following is most appropriate for a patient deficient in factor IX?

a) FFP
b) Whole blood
c) Cryoprecipitate
d) Prothrombin complex concentrate

A

d) Prothrombin complex concentrate

They don’t need FFP or whole blood as they only have a single deficiency. Cryoprecipitate contains fibrinogen, vWF, factor VIII, and factor XIII - therefore would not improve factor IX. PCC contains factors II, VII, IX, and X.

56
Q

Why should FFP only be thawed immediately before use?

A

6 hours after thawing, levels of the labile factors (V and VIII) begin to diminish.

57
Q

What term is being described:

The Hb concentration at or below which RBCs are usually ordered for transfusion of asymptomatic patients with anaemia.

A

Transfusion threshold

58
Q

True or false: RBC transfusion is a suitable option for treatment of chronic anaemia caused by iron or vitamin deficiency.

A

False.

Red cell transfusion should be avoided in patients with chronic anaemia due to Fe or vitamin deficiency, unless it is so severe there is an imminent risk of death

59
Q

Why might you transfuse platelets?

A

Treatment of bleeding due to severe thrombocytopenia or platelet dysfunction

Prevention of bleeding in patients with severe thrombocytopenia or platelet dysfunction

60
Q

According to NICE guidelines, what are the target values when transfusing patients in the following groups:

a) Haemorrhaging patient
b) Anaemia with severe symptoms
c) Acute anaemia with mild symptoms
d) Chronic anaemia

A

a) Haemorrhaging patient:
Maintain normovolaemia, transfuse based on Hb

b) Anaemia with severe symptoms:
Until symptoms resolve (but not >100g/L)

c) Acute anaemia with mild symptoms:

<70g/L if no CV disease
< 80g/L if CV disease

d) Chronic anaemia:
Individualised plan, commonly 80-100g/L

61
Q

What alternatives can be considered instead of RBC transfusion?

A

Correction of treatable causes of anaemia:

  • Fe deficiency
  • B12/folate deficiency
  • EPO treatment for renal disease

Correction of coagulopathy:

  • Discontinue anti-platelets
  • Administer anti-fibrinolytics
62
Q

Which of the following is an appropriate situation for transfusion of FFP?

a) treat single factor deficiency
b) correct abnormal clotting prior to invasive procedure
c) reverse warfarin
d) correct abnormal clotting in patients that are not bleeding

A

b) correct abnormal clotting prior to invasive procedure

63
Q

Which of the following product requires ABO testing of patient’s blood:

a) Albumin
b) Cryoprecipitate
c) Clotting factors
d) Prothrombin complex concentrate

A

b) Cryoprecipitate

Requires ABO testing:

  • RBC
  • Platelets
  • FFP
  • Cryoprecipitate

No need to ABO test:

  • Prothrombin complex concentrate
  • Clotting factors
  • Albumin
64
Q

What treatment is used to replace clotting factors in patients with multiple factor deficiencies (acquired coagulopathies), i.e. liver disease, disseminated intravascular coagulation etc

A

FFP transfusion

65
Q

What are the acute transfusion reactions?

A

Immunological:

  • Acute haemolytic transfusion reaction due to incompatibility
  • Allergic/anaphylactic reaction
  • Transfusion-related acute lung injury (TRALI)

Non-immunological:

  • Bacterial contamination
  • Transfusion-associated circulatory overload (TACO)
  • Febrile non-haemolytic transfusion reaction (FNHTR)
66
Q

Which transfusion product contains fibrinogen, vW, factor VIII and factor XIII?

A

Cryoprecipitate

67
Q

What are the most common reactions following a transfusion?

A

Febrile (FNHTR)

  • 10-30% after platelets
  • 1-2% after RBC

Anaphylaxis (0.5-1%)

68
Q

According to NICE guidelines, what are the target values when transfusing patients in the following groups:

a) Haemorrhaging patient
b) Anaemia with severe symptoms
c) Acute anaemia with mild symptoms
d) Chronic anaemia

A

a) Haemorrhaging patient:
Maintain normovolaemia, transfuse based on Hb

b) Anaemia with severe symptoms:
Until symptoms resolve (but not >100g/L)

c) Acute anaemia with mild symptoms:

<70g/L if no CV disease
< 80g/L if CV disease

d) Chronic anaemia:
Individualised plan, commonly 80-100g/L

69
Q

What alternatives can be considered instead of RBC transfusion?

A

Correction of treatable causes of anaemia:

  • Fe deficiency
  • B12/folate deficiency
  • EPO treatment for renal disease

Correction of coagulopathy:

  • Discontinue anti-platelets
  • Administer anti-fibrinolytics
70
Q

Which test involves mixing patient’s plasma with aliquots of donor red cells to see if agglutination or haemolysis occurs?

A

Crossmatching

71
Q

Which of the following product requires ABO testing of patient’s blood:

a) Albumin
b) Cryoprecipitate
c) Clotting factors
d) Prothrombin complex concentrate

A

b) Cryoprecipitate

Requires ABO testing:

  • RBC
  • Platelets
  • FFP
  • Cryoprecipitate

No need to ABO test:

  • Prothrombin complex concentrate
  • Clotting factors
  • Albumin
72
Q

Which of the following treatments require crossmatching:

a) Red blood cell transfusion only
b) RBC and FFP
c) RBC, FFP and cryoprecipitate
d) RCP, FFP, cryoprecipitate and platelets

A

a) Red blood cell transfusion only

If the question asked which required a group and save or ABO testing, the answer would be d.

73
Q

What are the delayed transfusion reactions?

A

Immunological:

  • Graft vs Host disease (GvHD)
  • Post-transfusion purpura
  • Immunomodulation
  • Immunosuppression

Non-immunological:

  • Transfusion transmitted infection (TTI)
  • Haemochromatosis
74
Q

What are the most common reactions following a transfusion?

A

Febrile (FNHTR)

  • 10-30% after platelets
  • 1-2% after RBC

Anaphylaxis (0.5-1%)

75
Q

Primary myelofibrosis is frequently associated with which genetic change?

A

JAK2 mutation

The JAK2 gene is mutated in approximately half of cases of myelofibrosis. It is also mutated in 55% of cases of essential thrombocythaemia and almost all cases of polycythaemia vera.

76
Q

A 49-year-old woman is admitted to hospital with severe anaemia. The haemoglobin concentration is 43 g/l, white cell count 28 × 10^9/l, platelet count 220 × 10^9/l.

92% of the white cells are mature looking lymphocytes and some smear cells are noted. Further examination of the blood film showed polychromasia and spherocytes. The bilirubin is elevated at 67 μmol/l (normal 3‒17). The patient is known to have chronic lymphocytic leukaemia.

The most likely cause of the anaemia is?

A

Autoimmune haemolytic anaemia

About 10% of patients with chronic lymphocytic leukaemia (CLL) develop autoimmune haemolytic anaemia. The diagnosis could be confirmed by finding a positive Coombs’ test.

Bone marrow failure can cause anaemia in patients with CLL but you would also expect thrombocytopenia and there would not be polychromasia (implying a reticulocytosis). Infection with parvovirus causes red cell aplasia in some susceptible patients. In such patients, you would not find polychromasia. The reticulocyte count would be very low.

There is no evidence of iron deficiency and in bleeding you would not expect to find spherocytes in the blood film.

77
Q

A 60-year-old woman presents with severe anaemia and jaundice. She is thought to have an autoimmune haemolytic anaemia.

To support this diagnosis, what are the the most likely abnormalities on the blood film?

A

Polychromasia, spherocytes and nucleated red cells

The blood film of someone with an autoimmune haemolytic anaemia typically shows polychromasia, spherocytes and, if severe, nucleated red blood cells. A direct antiglobulin test (DAT or Coombs’ test) would be positive in most patients. The red cells may be macrocytic but this would not be the most likely blood film comment. Patients with myelofibrosis or metastatic malignancy involving the marrow often have polychromasia, tear drop red cells and leuco-erythroblastic changes.

78
Q

A patient who has had a splenectomy for trauma requires vaccination against which diseases?

A

Haemophilus influenzae type b, Meningococcus, Pneumococcus

The patient is at increased risk of malaria but vaccination is not the solution. The patient is at increased risk of Haemophilus influenzae type b, pneumococcus and meningococcus and available vaccinations should be given. The patient is not at increased risk of hepatitis B.

79
Q

A 58-year-old woman with severe rheumatoid arthritis is feeling increasingly tired. She has been taking non-steroidal anti-inflammatory drugs (NSAID) and recently started a tumour-necrosis factor (TNF) inhibitor.

Her haemoglobin concentration is 90 g/l, mean red cell volume 76 fl (normal range 77 – 98) and mean cell haemoglobin 26.5 pg (normal range 28 – 32). Her ferritin is 155 μg/l and her estimated glomerular filtration rate is >60 ml/min.

The most likely cause of her anaemia is?

A

Anaemia of chronic disease

If a patient has both iron deficiency anaemia and the anaemia of chronic disease the ferritin is usually low in the normal range. The value in this patient suggests that this is anaemia of chronic disease without iron deficiency. The anaemia of chronic disease is most commonly normochromic, normocytic but when severe is hypochromic microcytic.

80
Q

__?__ is a neoplastic disorder of lymphoid tissue usually associated with t(14;18)

A

Follicular lymphoma

81
Q

Widespread activation of the clotting cascade resulting in the formation of clots throughout the small blood vessels?

A

Disseminated intravascular coagulation

82
Q

A genetic blood disorder resulting in a rigid abnormally shaped red blood cell

A

Sickle cell anaemia

83
Q

Which organ is essential to the maintenance of normal levels of coagulation factors?

A

Liver

84
Q

Chromosomal abnormality seen in CML

A

Philadelphia Chromosome t(9;22)

85
Q

Clopidogrel is an __1__ agent that inhibits __2__.

A

1) anti-platelet

2) P2Y12

86
Q

What is the most common heritable bleeding disorder?

A

von Willebrand disease

87
Q

The brand name of the small molecule specifically designed to block the active site in the Bcr-Abl tyrosine kinase

A

Gleevec

88
Q

The process of mixing a patient’s plasma with aliquots of donor red cells to see if a reaction occurs

A

Crossmatching

89
Q

Increase in the number of red blood cells

A

Polycythaemia

90
Q

Neoplasm where the proliferation of abnormal haematopoietic stem cells leads to scarring of the marrow

A

Myelofibrosis

91
Q

Type of lymphoma characterised by the presence of Reed-Sternberg cells

A

Hodgkin Lymphoma

92
Q

Possible severe complication of intensive chemotherapy in acute leukaemia

A

Neutropenic sepsis

93
Q

Type of anaemia associated with atrophic gastritis

A

Pernicious

94
Q

Tyrosine kinase inhibitor that is effective in CML

A

Imatinib

95
Q

Malignant clonal proliferation of plasma cells

A

Myeloma

96
Q

What is a common cause of subacute combined degeneration of the cord?

A

B12 deficiency

97
Q

Type of anaemia commonly resulting in a microcytic hypochromic picture

A

Iron deficiency anaemia

98
Q

A type of anaemia associated with folic acid deficiency

A

Megaloblastic

99
Q

An X-linked recessive disorder resulting in factor VIII deficiency

A

Haemophilia