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3MB Haematology > Pancytopenia > Flashcards

Pancytopenia Flashcards

1
Q

Define pancytopenia

A

Deficiency of blood cells of all lineages (excludes lymphocytes)

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2
Q

Pancytopenia can include either reduced production or increased destruction. Give examples of reduced production

A

Acquired (primary and secondary) bone marrow failure

Inherited syndrome

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3
Q

Characteristics of inherited marrow failure syndrome

A

Arise due to defects in DNA repair/ribsosomes/telomeres

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4
Q

What is an example of an inherited bone marrow failure

A

Fanconi’s anaemia

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5
Q

Short stature
Skin pigment abnormalities
Radial ray abnormalities
Café au lait spots
Hypogenitilia
Endocrine, GI, CVD, renal and haem abnormalities

A

Fanconi’s anaemia

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6
Q

Pathophysiology of inherited bone marrow failure

A

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

Consists of macrocytosis followed by thrombocytopenia, then neutropenia

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7
Q

Mode of transmission of Fanconi anaemia

A

Autosomal recessive

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8
Q

Fanconi anaemia imposes an increased risk of..

A

Acute myeloid leukaemia

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9
Q

Haematological problems seen in Fanconi anaemia

A

Aplastic anaemia

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10
Q

Give examples of acquired primary bone marrow failure

A

Idiopathic aplastic anaemia
Myelodysplastic syndromes
Acute leukaemia

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11
Q

Aplastic anaemia is characterised by..

A

Pancytopenia and a hypoplastic bone marrow

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12
Q

Normochromic, normocytic anaemia
Leukopenia, with lymphocytes relatively spared thrombocytopenia

A

Aplastic anaemia

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13
Q

Aplastic anaemia may be the presenting feature in..

A

Acute lymphoblastic or myeloid leukaemia

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14
Q

A minority of patients with aplastic anaemia later develop..

A

Paroxysmal nocturnal haemoglobinuria or myelodysplasia

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15
Q

Causes of aplastic anaemia

A

Fanconi anaemia, dyskeratosis congenita
Cytotoxics, chloramphenicol, sulphonamides, phenytoin, gold
Benzene
Parvovirus, hepatitis
Radiation

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16
Q

Pathophysiology of idiopathic aplastic anaemia

A

Auto-reactive T cells release inflammatory cytokines (IFN-ᵞ
TNF-α) leading to pancytopenia and a hypoplastic bone marrow

17
Q

Define myelodysplastic syndromes

A

Heterogeneous group of clonal hematopoietic stem cell disorders, characterised by ineffective hematopoiesis and peripheral blood cytopenias

18
Q

Myelodysplastic syndromes can progress to..

A

Acute myeloid leukaemia (AML)

19
Q

What are the secondary causes of hematopoietic stem cells and when do they develop?

A

Chemotherapy and radiotherapy

Develops around 5 years post-treatment

20
Q

The key pathophysiological feature of MDS is..

A

Ineffective hematopoiesis leading to peripheral cytopenias despite a typically hypercellular bone marrow

21
Q

Ix for MDS

A

Peripheral blood counts
Bone marrow biopsy
Cytogenetic analysis

22
Q

Dysplastic changes in hematopoietic cells and a varying degree of blasts

23
Q

Tx for MDS

A

Supportive treatment (e.g., blood or platelet transfusions)
Erythropoietin
Granulocyte colony-stimulating factor
Chemotherapy and targeted therapies (e.g., lenalidomide)
Allogenic stem cell transplantation

24
Q

Increased apoptosis of progenitor and mature cells

25
Q

Why can acute leukaemia cause pancytopenia?

A

Proliferation of abnormal cells (blasts) from leukaemic stem
cells
Failure to differentiate or mature into normal cells
Prevent normal haemopoietic stem/ progenitor (HSC) development by ‘hijacking’/ altering the haemopoietic niche and marrow microenvironment

26
Q

Give examples of causes of secondary bone marrow failure

A

Drugs (chemotherapy, alcohol, azathioprine, methotrexate
chloramphenicol)
B12/folate deficiency
Non-haemopoietic malignant
infiltration, lymphoma
Viral (eg HIV)/storage diseases

27
Q

Pancytopenia can include either reduced production or increased destruction. Give examples of increased destruction

A

Hypersplenism - increased splenic pool leads to Increased destruction that exceeds bone marrow capacity, usually associated with enlarged spleen

28
Q

What causes hyperplenism?

A

Splenic congestion - portal hypertension
Systemic diseases - RA
Haematological - splenic lymphoma

29
Q

Clinical features of pancytopenia

A

Anaemia
Neutropenia
Thrombocytopenia

30
Q

Hypocellularity in bone marrow is normally seen in..

A

Aplastic anaemia

31
Q

Hypercellularity in bone marrow is normally seen in..

A

Myelodysplastic syndromes
B12/folate deficiency
Hypersplenism

32
Q

Tx in pancytopenia

A

Supportive - red, platelet, neutrophils transfusions
Antibiotics prophylaxis/treatment

33
Q

Tx in congenital primary bone marrow disorder

A

Allogeneic stem cell transplantation

34
Q

Tx in idiopathic aplastic anaemia

A

Immunosuppression

35
Q

Tx in secondary bone marrow disorder

A

Drug reaction – STOP
Viral – eg treat HIV
Replace B12/folate

3MB Haematology (23 decks)

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