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Haematology > Pancytopenia > Flashcards

Pancytopenia Flashcards

1
Q

What is pancytopenia?

A

A deficiency of blood cell of ALL lineages (but generally excludes lymphocytes)
Commonly see reduced Hb, platelets and neutrophil count

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2
Q

Is pancytopenia a diagnosis?

A

No

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3
Q

Does pancytopenia always mean bone marrow failure?

A

No

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4
Q

What is the life span of a red cell?

A

120 days

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5
Q

What is the life span of a neutrophil?

A

7-8 hours

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6
Q

What is the lifespan of a platelet?

A

7-10 days

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7
Q

Generally, what can cause pancytopenia?

A

Reduced production

Increased destruction

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8
Q

What are the causes of reduced production in pancytopenia?

A

Bone marrow failure; inherited or acquired (primary or secondary)

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9
Q

What are the characteristics of inherited marrow failure syndromes?

A

Cancer predisposition
Impaired hematopoiesis
Congenital anomalies
Arises due to defects in DNA repair/ ribosomes

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10
Q

What is an example of an inherited bone marrow failure syndrome?

A 
Fanconi's Anaemia: 
Short stature
Skin pigment abnormalities 
Radial ray abnormalities
Hypogenitalia
Endocrinopathies
GI defects
CV
Renal 
Haem
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11
Q

What are the haematological abnormalities seen in fanconi’s anaemia?

A

Macrocytosis followed by thrombocytopenia then neutropenia
Bone marrow failure (aplasia): 84% by 20 years
Leukaemia risk: 52% by 40 years

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12
Q

What is the pathogenic basis of the haematological abnormalities seen in fanconi’s anaemia?

A

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

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13
Q

What are examples of acquired primary bone marrow failure (an intrinsic marrow problem)

A 
Idiopathic aplastic anaemia (autoimmune attack against haemopoietic stem cells) 
Myelodysplastic syndromes (MDS) 
Acute leukaemia
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14
Q

What cytokines are involved in aplastic anaemia?

A

IFN-gamma

TNF- alpha

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15
Q

Describe myelodysplastic syndromes?

A

Dysplasia (disordered development)
Hypercellular marrow
Increased apoptosis of progenitor and mature cells (ineffective haemopoiesis)

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16
Q

What cancer is linked with myelodysplastic syndromes?

A

Can progress into AML

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17
Q

How is aplastic anaemia diagnosed?

A

Bone marrow biopsy

18
Q

What are the causes for aplastic anaemia?

A 
Fanconi's anaemia 
Autoimmune 
Drugs 
Virus; parvovirus B19, hepatitis 
Irradiation
19
Q

Why can acute leukaemia cause pancytopenia?

A

Proliferation of blasts from leukemic stem cell
Failure to differentiate or mature into normal cells
Prevents normal haemopoietic stems from developing by hijacking the haemopoietic niche and marrow microenvironment

20
Q

What can cause secondary bone marrow failure?

A

Drug induced (chemo, chloramphenicol, alcohol)
B12 / folate deficiency (nuclear maturation can affect all lineages)
Infiltrative; non-haemopoietic malignant infiltration, lymphoma
Misc; viral; HIB, parvovirus B19
Storage diseases

21
Q

How will the bone marrow look in B12/ folate deficiency?

A

Hypercellular

22
Q

What can result in pancytopenia via increased destruction?

A

Hypersplenism

23
Q

What does the red pulp of the spleen contain?

A

Sinusoids and cords

24
Q

What does the cord of red pulp contain?

A

Macrophages
Fibroblasts
Cells in transit (RBC, WBC, platelets, CD8 T cells)

25
Q

What is the function of the spleen?

A

Detects, retains and eliminates unwanted foreign or damaged material
Facilitates immune responses to blood borne antigens

26
Q

Why does hypersplenism result in increased destruction of cells?

A

Increased splenic pool

Increased destruction that exceeds bone marrow capacity, usually assoc with significant splenomegaly

27
Q

Does splenic size correlate with hypersplenism?

A

No; any cause of splenomegaly could result in hypersplenism but splenic size alone is not predictive of the degree of hypersplenism

28
Q

What can cause hypersplenism?

A

Splenic congestion; portal hypertx, CCF
Systemic; RA (felty’s)
Haemo: splenic lymphoma

29
Q

What can the clinical features of pancytopenia reflect?

A

Lack of circulating blood cells

Cause of pancytopenia

30
Q

What are the consequences of lack of circulating cells in pancytopenia?

A

Anaemia
Neutropenia
Thrombocytopenia

31
Q

What are the features of anaemia?

A

Fatigue
SOB
CV compromise; dizziness, palpitations

32
Q

What are the features of neutropenia?

A

Infections; determine severity and duration

33
Q

What are the features of thrombocytopenia?

A

Bleeding; purpura, petechiae, wet bleeding including visceral bleeds

34
Q

How is the cause of pancytopenia established?

A

Hx including FMHx
Clinical findings
FBC, blood film
Additional tests; B12/folate, LFTs, virology, autoantibody tests
Bone marrow aspirate
Specialised tests; chromosome fragility testing for fanconi’s syndrome

35
Q

What will cause hypocellular marrow cellularity in pancytopenia?

A

Aplasitc anaemia

36
Q

What will cause hypercellularity in marrow cellularity in pancytopenia?

A 
Myelodysplastic syndromes (proliferation and apoptosis) 
B12/ folate deficiency (late maturation failure, early proliferation and apoptosis) 
Hypersplenism
37
Q

What is the treatment for pancytopenia?

A

Supportive

Specific; dependent on cause (pancytopenia due to B12/folate deficiency)

38
Q

What is the supportive treatment for pancytopenia?

A

Red cell transfusions
Platelet transfusions
Antibiotic prophylaxis; antibacterials, antifungals

39
Q

Should you wait for microbial results before antibiotic treatment in neutropenic fever?

A

NO; give broad spectrum antibiotics that specifically cover gram negs

40
Q

What is the specific treatment for pancytopenia caused by a primary bone marrow disorder?

A

Malignancy; chemo
Congenital; bone marrow transplantation
Idiopathic aplastic anaemia; immunosuppression

41
Q

What is the specific treatment for pancytopenia caused by a secondary bone marrow disorder?

A

Drug reaction; STOP
Viral; treat HIV
Replace B12/ folate

42
Q

What is the specific treatment for pancytopenia caused by hypersplenism?

A

Treat cause if poss

Consider splenectomy

Haematology (31 decks)

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