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Hee-matte-ology > Pancytopenia > Flashcards

Pancytopenia Flashcards

1
Q

What is pancytopenia?

A

Deficiency of blood cells of all lineages (generally excludes lymphocytes)

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2
Q

Does Pancytopenia always = bone marrow failure?

A

No

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3
Q

What is the general path of neutrophil production?

A 
Promyelocyte
Myelocyte
Blast
Band
Metamyelocyte
Segmented neutrophil
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4
Q

What are the 2 branching causes of pancytopenia?

A

Reduced production

Increased destruction

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5
Q

What can cause reduced production?

A

Bone marrow failure

Inherited or acquired

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6
Q

What are the 3 characteristics of inherited marrow failure syndromes?

A

Cancer predisposition
Impaired haemopoiesis
Congenital anomalies

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7
Q

What is an example of inherited marrow failure syndrome?

A

Fanconi’s anaemia

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8
Q

What are the symptoms of Fanconi’s anaemia?

A 
Short stature
Skin pigment
Radial ray 
Hypogenitalia 
Endocrinopathies
GI defects 
Cardiovascular
Renal 
Haematological
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9
Q

What is the average age of someone with Fanconi’s Anaemia?

A

7 Years

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10
Q

What is the problem with fanconis anaemia?

A

Unable to correct inter-strand cross links (DNA damage)

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11
Q

What are examples of acquired primary bone marrow failure?

A

Idiopathic anaplastic anaemia
Myelodysplastic syndromes
Acute leukaemia

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12
Q

What happens in anaplastic anaemia?

A

IFNy and TNFa react to HSC

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13
Q

What are 4 types of myelodysplastic syndromes?

A

Dysplasia
Hypercellular marrow
Increased apoptosis of progenitor and mature cells
Propensity for evolution into AML

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14
Q

Why does acute leukaemia cause pancytopenia?

A

Proliferation of abnormal xcells form leukaemic stem cells
Failure to mature into normal cells
Prevent normal HSC development

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15
Q

What can cause secondary bone marrow failure?

A

Drug induced
B12/Folate deficiency
Infiltrative-non-haemopoietic malignant

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16
Q

What can cause increased destruction?

A

Hypersplenism

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17
Q

What is the spleen?

A

Secondary lymphoid organ

Encapsulated

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18
Q

How much does the spleen usually weight?

A

150–200g

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19
Q

What supplies the spleen?

A

Splenic artery

20
Q

What drains the spleen?

A

Splenic vein

21
Q

What is included in the parenchyma?

A

Red pulp and white pulp

22
Q

What does red pulp contain?

A

Sinusoids and cords

23
Q

What are sinusoids?

A

Fenestrated
Lined by endothelial cells
Supported by hoops of reticulin

24
Q

What do cords contain?

A

Macrophages and fibroblasts and cells in transit

25
Q

What is the function of the spleen?

A

Filter for blood
Detects and eliminates unwanted/foreign material
Facilitates immune responses to blood borne antigens

26
Q

What happens in hypersplenism?

A

Increased splenic pool
Increased destruction that exceeds bone marrow capacity
Enlarged spleen

27
Q

What is the splenic red cell mass of a normal spleen?

A

5%

28
Q

What is the splenic red cell mass of a hypersplenic spleen?

A

40%

29
Q

Is red cell transit fast or slow in hypersplenism?

A

Slow

30
Q

Is the splenic platelet poor higher or lower in hypersplenism?

A

Higher

31
Q

What can splenomegaly result in?

A

Hypersplenism

32
Q

What can cause Hypersplenism?

A 
Splenic congestion
-Portal Hypertension
Systemic diseases
(RA)
Splenic lymphoma
33
Q

What are secondary causes of Acquired pancytopenia?

A 
Drug induced
Vit B12/Folate deficiency
Infections
Metastatic cancer
Storage disorders
34
Q

What are the 3 contributing factors to pancytopenia?

A

Anaemia
Neutropenia
Thrombocytopenia

35
Q

What are three symptoms of anaemia?

A

Fatigue
SOB
CV comprimise

36
Q

What are clinical features of neutropenia?

A

Infections

37
Q

What are symptoms of thrombocytopenia?

A

Bleeding
Purpura
Petechiae
Wet bleeds

38
Q

What investigations are done for Pancytopenia?

A 
History
FBC
B12/folate
LFTs 
Virology
Autoantibodies
Bone marrow
39
Q

What is the marrow like in aplastic anaemia?

A

Hypocellular

40
Q

When is the bone marrow hypercellular?

A

Myelodysplastic syndromes
B12/Folate deficiency
Hypersplenism

41
Q

What is the treatment of pancytopenia?

A

Supportive

Specific (dependant on cause)

42
Q

What is the supportive treatment for pancytopenia?

A 
Red cell transfusions
Platelet transfusions 
Neutrophil transfusions
Antibiotics prophylaxis/treatment
Antibacterial 
Antifungals
43
Q

What is the treatment for congenital bone marrow disorder?

A

bone marrow transplant

44
Q

What is the treatment for idiopathic anaplastic anaemia?

A

Immunosuppression

45
Q

What is the treatment for secondary bone marrow disorder?

A

Drug reaction -STOP
Viral - Treat infection
Replace B12/folate

46
Q

What is the treatment for hypersplenism?

A

Treat cause

Consider splenectomy

Hee-matte-ology (17 decks)

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