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Pancytopenia Flashcards

1
Q

What does ‘pan’ mean?

A

Generalised

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2
Q

What does ‘cyto’ mean?

A

Prefix denoting a cell

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3
Q

What does ‘penia’ mean?

A

Lack/deficiency (in blood)

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4
Q

What is pancytopenia?

A

A deficiency of blood cells of ALL lineages (but generally excludes lymphocytes).

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5
Q

** What is pancytopenia? **

A

A deficiency of blood cells of ALL lineages (but generally excludes lymphocytes).

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6
Q

What does pancytopenia NOT always mean?

A

Bone marrow failure

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7
Q

For steady state, cell production = cell destruction

A

T

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8
Q

In erythropoiesis, what cells are seen in the blood and not the bone marrow?

A
  • Reticulocyte

* Erythrocyte

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9
Q

Neutrophils are properly segmented

A

T

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10
Q

What is the immediate precursor to platelets?

A

Megakaryocyte

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11
Q

Megakarocytes are p________

A

Polypoid

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12
Q

In what cells does ‘the nucleus divides very quickly without the cytoplasm having divided’

A

Megakarocyte

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13
Q

What is the life span of red cells?

A

120 days

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14
Q

What is the lifespan of platelets?

A

7-10 days

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15
Q

What is the lifespan of neutrophils?

A

7-8 hours

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16
Q

If bone marrow fails, in order of first to drop and last to drop, list the cells.

A
  • Neutrophils
  • Platelets
  • Red cells
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17
Q

What are the 2 main groups of causes of pancytopenia?

A
  1. REDUCED production

2. INCREASED destruction

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18
Q

What is the basic cause of reduced production of cells, leading to pancytopenia?

A

Bone marrow failure

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19
Q

Name the 2 main things that lead to bone marrow failure.

A
  1. Inherited syndromes

2. Acquired conditions (where ‘production machinery’ comes to a halt)

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20
Q

Name the 2 main things that lead to bone marrow failure.

A
  1. Inherited syndromes

2. Acquired conditions (where ‘production machinery’ comes to a halt) - primary or secondary

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21
Q

Describe primary vs secondary acquired bone marrow failure.

A

Primary

(something has gone wrong in the bone marrow)

Secondary

(something else is going on, and even though bone marrow cells are healthy, they’re not able to produce healthy blood cells)

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22
Q

What are the 3 main groups of characteristics of inherited marrow failure syndromes?

A
  1. Cancer predisposition
  2. Impaired haempoiesis
  3. Congenital anomalies
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23
Q

What do these inherited marrow syndromes arise due to?

A

Defects in DNA repair/ribosomes

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24
Q

Inherited bone marrow failure conditions are very common

A

F - they are very rare

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25
Q

What is the most common inherited bone marrow failure syndrome?

A

Faconi’s anaemia

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26
Q

What does faconis anaemia lead to clinically?

A
  • Short stature – skeletal abnormalities.
  • Skin pigment abnormalities – café au lait spots.
  • Radial ray abnormalities
  • Hypogenitilia
  • Endocrinopathies
  • GI defects
  • Cardiovascular
  • Renal
  • Haematological
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27
Q

What is the mean age of presentation of inherited bone marrow failure syndromes?

A

7 years old

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28
Q

What do these haematological abnormalities of an inherited bone marrow failure syndrome occur due to?

A

An inability to correct inter-strand cross-links (DNA damage)

So, there patients are unable to correct their own DNA damage

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29
Q

In terms of cell deficiencies, what happens in inherited bone marrow failure syndromes?

A

Macrocytosis, followed by thrombocytopenia, then neutropenia

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30
Q

In patients with haematological abnormalities of inherited bone marrow failure syndromes, what are they unable to do?

A

Correct their own DNA damage

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31
Q

What is there a 52% risk of by 40 years in those with inherited bone marrow failure syndromes?

A

Leukaemia

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32
Q

In primary acquired bone marrow failure, there is no obvious cause

A

T

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33
Q

What type of defect is usually seen in people with a primary acquired bone marrow defect?

A

A stem cell defect

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34
Q

In acquired PRIMARY bone marrow failure, there is what type of problem?

A

An INTRINSIC marrow problem

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35
Q

Name 3 conditions associated with primary acquired bone marrow failure.

A
  • Idiopathic aplastic anaemia
  • Myelodysplastic syndromes (MDS)
  • Acute Leukaemias
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36
Q

What does idiopathic aplastic anaemia occur due to?

A

An autoimmune attack against haemopoietic stem cell(s).

37
Q

Who do myelodysplastic syndromes occur more commonly in?

38
Q

What is white cell count like in acute leukaemia?

A

It can be variable.

May start off with pancytopenia as white cells haven’t yet spilled into blood

May also start off high due to lots of primitive cells

39
Q

What cells fail to be produced in aplastic anaemia?

A
  • Erythrocytes
  • Platelets
  • Granulocytes

Due to auto-immune attack on haematopoietic stem cells

40
Q

What cell is attacked in aplastic anaemia?

A

Haematopoietic stem cells

41
Q

Haematopoietically active marrow = red marrow

42
Q

What is seen on histology of someone with aplastic anaemia?

A

In aplastic anaemia, all the haematopoietic islands are replaced by fat and fibrosis etc

43
Q

‘clone of autoreactive T cells which are detected’ is seen in what condition?

A

Aplastic anaemia

44
Q

What cells (that damage stem cells) are increased in aplastic anaemia?

A

IFN and TNF

45
Q

Describe what happens in bone marrow in myelodysplastic syndrome.

A
  • Dysplasia.
  • HYPERCELLULAR marrow.
  • Increased APOPTOSIS of progenitor and mature cells (ineffective haemopoiesis).
46
Q

What is a myelodysplastic syndrome?

A

Myelodysplastic syndromes (MDS) are a group of cancers in which immature blood cells in the bone marrow do not mature and therefore do not become healthy blood cells.

47
Q

Why is the bone marrow in myelodysplastic syndromes HYPERCELLULAR?

A

Because the abnormal production of cells occurs at a fast rate. (greater than rate of apoptosis).

48
Q

The bone marrow in myelodysplastic syndromes is _____ cellular

A

HYPERCELLULAR

49
Q

What does hyper cellular bone marrow have a propensity to evolve into?

50
Q

Why can leukaemia cause pancytopenia?

A

Basically HSC need a nice bone marrow to live in (their niche), if LSC (leukaemic stem cells ) come along they ruin the nice niche environment for the HSP so they no longer can make the nice normal blood cells (failure of differentiation of normal cells)

This prevents abnormal HSC development

A random mutation of a normal HSC makes an abnormal LSC

51
Q

** Outline the 2 main causes of secondary bone marrow failure. **

A
  • Drug induced e.g chemotherapy, chloramphenicol, alcohol– these all cause aplasia thus are hypocellular
  • B12/folate deficiency (nuclear maturation can affect all lineages) -remember hypercellular
52
Q

B12/folate deficiency results in a HYPO/HYPERcellular bone marrow

A

HYPERCELLULAR

53
Q

Drug induced secondary bone marrow failure e.g chemo, alcohol all cause HYPO/HYPERcellular bone marrow failure.

A

HYPOCELLULAR

54
Q

Why does B12/folate deficiency result in secondary bone marrow failure?

A

Results in nuclear maturation defects which can affect all lineages

55
Q

Why is bone marrow hyper cellular in b12/folate deficiency?

A
  • Hypoxia leads to an increase in epo
  • Increased proliferation of primitive compartments.

But throughput to more premature compartments decreases as don’t have the resources to make mature cells properly

56
Q

What drugs can cause secondary bone marrow failure? Via what mode of action?

A
  • Chemo
  • Chloramphenicol
  • Alcohol

They all cause bone marrow aplasia

57
Q

Give 2 examples of infiltrative causes of secondary bone marrow failure.

A
  • Non-haemopoietic malignant infiltration.

* Lymphoma.

58
Q

What is increased destruction of bone marrow associated with?

A

Hypersplenism - the spleen is causing the cells to be phagocytosed, the bone marrow tries to compensate (hypercellularity) but can’t = pancytopenia

59
Q

Describe the structure of the spleen (mention red pulp and sinusoids).

A

Red pulp has sinusoids.

Sinusoids are much bigger than capillaries and have a fenestrated basement membrane.

60
Q

Describe the BM of sinusoids.

A

Fenestrated

61
Q

The spleen is a very vascular organ. Describe the blood supply to the spleen.

A
  • Supplied by splenic artery (branch of coeliac axis)

* Drained by splenic vein (with SMV forms portal vein)

62
Q

What is hypersplenism?

A
  • Increased splenic pool.

* Increased destruction that exceeds bone marrow capacity, usually associated with a significantly enlarged spleen

63
Q

A pancytopaenia, associated with an increased size of the spleen.

64
Q

How does hypersplenism cause pancytopenia?

A

If spleen is trapping all of these cells that are being produced, the bone marrow will try to compensate. Will become hypercellular. If can’t compensate – will become pancytopenic

65
Q

Any cause of what can POTENTIALLY result in hypersplenism?

A

Splenomegaly

66
Q

A pancytopenia is associated with an increased size of the spleen, why?

A
  • All blood cells pass through the spleen
  • If transit slows down, there are macrocytes there that immediately starts to be phagocytosed the cells
  • It is important that circulation of cells through the spleen is at a certain speed
  • If the spleen is trapping all the cells passing through it, the bone marrow will try compensate by becoming hypercellular
  • If the bone marrow cannot compensate, it becomes pancytopenic
67
Q

Name the 3 different categories of causes of hypersplenism.

A
  • Splenic congestion
  • Systemic disease
  • Haematological disease
68
Q

Name 2 causes of splenic congestion (which result in hypersplenism).

A
  • Portal hypertension

* Congestive heart failure

69
Q

Name a systemic disease (which results in hypersplenism).

A
  • Rheumatoid arthritis – Felty’s syndrome
70
Q

Name a haematological disease (which results in hypersplenism).

A
  • Splenic lymphoma
71
Q

What are the 5 manifestations of Felty’s syndrome.

A
  • Splenomegaly
  • Anaemia
  • Neutropenia
  • Thrombocytopenia
  • Arthritis (rheumatoid)
72
Q

The clinical features of pancytopenia can reflect either …

A
  • Lack of circulating blood cells

* The cause of pancytopenia

73
Q

Pancytopenia =

A

Anaemia + thrombocytopenia + neutropenia

74
Q

** Pancytopenia =

A

Anaemia + thrombocytopenia + neutropenia

75
Q

Describe neutropenia and state what type of infection people with this are most likely to get.

A
  • Infections – of great severity and duration

Neutrophils often form the barriers of the mucosal membranes (endothelial cells) where there is contact with germs

People with neutropenia are predisposed to gram -ve infections (more than gram +ve infections) from the bowel

76
Q

List 4 signs of thrombocytopenia.

A
  • Bleeding
  • Purpura
  • Petechiae
  • ‘Wet’ bleeds including visceral bleeds
77
Q

How is Faconi’s anaemia detected?

A

Chromosome fragility testing  used to check for inability to correct DNA damage

78
Q

** How is Faconi’s anaemia detected? **

A

Chromosome fragility testing  used to check for inability to correct DNA damage

79
Q

** Outline the tests you would do to find the cause of a pancytopenia. **

A
  • History
  • Family history
  • Clinical findings
  • FBC, blood film
  • Bone marrow aspirate
  • Core biopsy via Jamshidi needle
  • Specialist test
80
Q

** Teat neutropenic fever promptly based on local unit antibiotic policy without waiting for (microbiology) results **

A

T - always ask about fever in the OSCE

81
Q

Name a hypocellular cause of pancytopenia.

A

Aplastic anaemia

82
Q

Name 3 causes of hypercellular pancytopenia.

A
  1. Myelodysplastic syndromes – proliferation and apoptosis.
  2. B12/folate deficiency – late maturation ‘failure’ + early proliferation + apoptosis.
  3. Hypersplenism.
83
Q

Outline the treatment for pancytopenia.

A
  1. Supportive

2. Specific - depending on the cause

84
Q

Outline the supportive treatment of pancytopenia.

A
  • Red cell transfusion
  • Platelet transfusion
  • Antibiotics (tx or prophylaxis)
85
Q

Outline the treatments for primary bone marrow disorders.

A
  • Malignancy – chemotherapy
  • Congenital – bone marrow transplant
  • Idiopathic aplastic anaemia – immunosuppression (since this is an autoimmune attack on the cells)
86
Q

Treatment for hypersplenism?

A
  • Treat cause if can.

* Consider splenectomy (although not always appropriate).

87
Q

If someone is having a drug reaction that is causing a secondary bone marrow failure, what should you do?

88
Q

If someone is B12/folate deficient which is causing secondary bone marrow failure, what should you do?

A

REPLACE B12/folate

Haematology (26 decks)

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