Haematology (White blood cells) Flashcards

1
Q

What is the normal concentration range of WBC in the blood?

A

4-10 x 10^9/L

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2
Q

What is the only immature WBC that leaves the bone marrow?

A

T lymphocytes

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3
Q

Therefore, what does the presense of immature WBCs (other than T cells) in the blood indicate?

A

Disease

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4
Q

Name 2 diseases resulting from granulocyte defects?

A

1) Acute myeloid leukemia
- increase in the number of myeloblasts in bone marrow or peripheral blood

2) Actute promyelocytic leukaemia
- Increase number of promyelocytes
- Not associated with increases in more differentiated types

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5
Q

What is a Leukaemoid Reaction?

A

Defined as a leucocyte count in excess of 50 x 10^9/L with all stages of myeloid maturation (including myeloblasts)

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6
Q

Leukaemoid reaction can be difficult to distinguish from what disease?

A

Chronic myeloid leukaemia (CML)

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7
Q

What is a main difference between leukaemoid reaction and true leukaemia?

A

Cells of leukaemoid reaction are polyclonal

True leukemia cells usually monoclonal (derived for a single cell type)

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8
Q

What are some causes of leukaemoid reaction?

A
  • Corticosteroids
  • Haemorrhage
  • Haemolysis
  • Alcohol intoxication
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9
Q

What is neutropenia in response to acute tissue damage/infection?

A

Neutrophils migrate in large numbers from the bone marrow to the periphery, depleting the marrow reserves

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10
Q

What is neutrophilia?

A

A neutrophil count in excess of the normal range
- immature neutrophils in peripheral blood, contain granules with high concs of acid mucosubstances capable of fighting infection

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11
Q

What is neutrophilia usually a result of?

A

Blood borne infectons, known as sepsis

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12
Q

What does Pelger-Huet anomaly result in?

A

Hyposegmented ( 2 nuclear lobes or fewer) neutrophils with a singular or bilobed nucleus

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13
Q

What is Kostmann Syndrome?

A
  • Congenital severe combined neutropenia commonly diagnosed in first year of life
    (0. 2 x 10^9/L)
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14
Q

60% of Kostmann syndrome patients have mutations in what?

A

Gene encoding neutrophil elastase (ELA2)

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15
Q

30% of Kostmann syndrome patients also have defects in what?

A

HAX1, the gene encoding HS-1 associated protein X (HAX)

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16
Q

What is the result of HAX mutations?

A

Plays an important role in apoptosis

- cells deficient in HAX undergo premature apoptosis in the bone marrow

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17
Q

Patients with Kostmann syndrome are at risk of developing what?

A
  • Myelodysplastic syndrome or AML
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18
Q

Describe the process of granule release from neutrophils in patients with sepsis

A

Approx 300 different protein types selectively distributed between primary, secondary and tertiary granules

Released at different times to induce toxic response and inflammation to foreign objects

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19
Q

What are hypersegmented neutrophils and when can they be seen?

A
  • Average of 5 lobes or more in the nucleus
  • Occurs in many infections
  • Iron deficiency
  • Uraemic patients
20
Q

What is megaloblastic anaemia? What is the cause and consequence?

A

Megaloblastic anaemia develops as a result of vitamin B12 deficiency

  • DNA replication compromised, cell cycle arrest during S phase
  • Results in macropolycytes - enlarged cells with double DNA
21
Q

What are the 3 main proteins found in neutrophil granules and which are they found in (primary, secondary etc)

A

1) Myeloperoxidase (MPO)
- haem containing enzyme involved in non-specific pathogen elimination
- Primary granules

2) Lactoferrin
- sequencers iron, prevents bacterial growth
- secondary granules

3) Gelatinase - metalloprotease that digests collagen, allowing movement through basement membrane of blood vessels (diapedesis)

22
Q

How are eosinophils involved in mediating hypersensitivity?

A

Counteract the effects of basophils and mast cells by secreting prostaglandins

23
Q

What is another key role of eosinophils?

A

Destruction of helminths (e.g worms)

24
Q

What count is considered eosinophilia? What is it caused by?

A
  • 0.5 x 10^9/L
  • allergy or inflammation
  • helminth infection
  • hodgkin lymphoma
  • chronic eosinophilic leukemia
25
Q

What occurs in prolonged eosinophilia?

A

Severe tissue damage

26
Q

Eosinophils in severe allergy have secondary granules that stain orange with eosin due to the presence of what?

A

Bacteriocidal arginine-rich basic proteins

27
Q

What count is basophilia?

A

0.1 x 10^9/L

28
Q

When does basophilia occur?

A
  • Acute hypersensitivity reactions
  • Haematological malignancies e.g CML
  • Advancing CML is associated with progressive basophilia
29
Q

What can occur to macrophages during severe chronic inflammation?

A

May undergo endomitosis

- replication of the nucleus in the absense of cell division to produce giant cells

30
Q

What is a raised peripheral blood lymphocyte count called?

A

lymphocytosis

31
Q

What can cause lymphocytosis?

A

Viral infections
Bacterial infections
Haematological maligancies

32
Q

What is the name for a reduced lymphocyte count?

A

Lymphopenia

33
Q

What can cause lymphopenia?

A

Trauma or infection

Acute or chronic immunosuppression

34
Q

What is an indicator of NK cell leukaemia?

A

Large numbers of circulating NK cells

35
Q

What is the difference between normal NK cells and NK cells in leukemia?

A

Within an NK cell population, leukemia has more NK cell with large cytoplasms with cytoplasmic granules

36
Q

What is Infectious mononucleosis? What is it caused by?

A
  • A benign lymphoproliferative disorder
  • Caused by infection of B cells with Epstein-Barr virus (EBV)
  • Binds to CD21 receptor on B cell membrane
37
Q

How to B cells respond to EBV infection?

A

Produce anti-EBV antibodies

38
Q

What % of adults worldwide are seropositive? (possess antibodies against EBV)

A

80-90%

39
Q

What are the symptoms of IM?

A
Fever
Malaise
Chills
Sore throat
Enlarged lymph nodes
40
Q

Name some characteristics of the full blood count in IM

A

1) Leukocytosis (12-25 x 10^9/L)
2) Neutropenia and thrombocytopenia may be evident
3) Scalloping of the membrane around neighboring red cells

41
Q

How is IM diagnosed?

A

Patients with IM have heterophile antibodies (also called Paul-Bunnell antibodies)

Antibodies detected by standard latex agglutination test

42
Q

Name a more specific test for EBV infection

A

Antibodies against:

  • viral capsid antigen (VCA)
  • nuclear antigen (EBNA)
43
Q

Where are plasma cells usually restricted too?

A

The bone marrow and lymphoid organs

44
Q

What can cause the appearance of plasma cells in the peripheral blood (plasma cell leukemia)?

A

Acute infections or haematologial malignancies

45
Q

How can plasma cells in the peripheral blood be identified?

A
  • Eccentric (off centre) shaped nucleus

- deeply basophilic (blue) cytoplasm

46
Q

What is the structure of a plasma cell nucleus?

A
  • clumped chromatin

- surrounded by a prominent golgi zone known as the perinuclear halo