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Flashcards in Pancytopenia Deck (19)
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1
Q

What is pancytopenia?

A

A deficiency of blood cells of all lineages

2
Q

What can cause pancytopenia due to reduced production?

A

Bone marrow failure - inherited syndromes e.g. Fanconi’s anaemia, acquried (primary and secondary)

3
Q

What are the clinical features of Fanconi’s anaemia?

A

Short stature, skin pigment abnormalities e.g. cafe au lait patches, radial ray abnormalities, hypogenitalia, endocrinopathies, GI defects, cardiovascular, renal, haematological i.e. multi-system disease

4
Q

When does Fanconi’s anaemia typically present and what is the disease mechanism?

A
  • Median age - 7 years
  • Unable to correct inter-strand cross links (DNA damage)
  • Macrocytosis followed by thrombocytopenia, then neutropenia
  • Bone marrow failure risk - 84% by 20 y/o
  • Leukaemia risk - 52% by 40 y/o
5
Q

What are some causes of acquired bone marrow failure?

A
  • Idiopathic aplastic anaemia - autoimmune attack against haemopoietic stem cell
  • Myelodyplastic syndrome
  • Acute leukaemia
6
Q

What is myelodysplastic syndrome?

A
  • Dysplasia, hypercellular marrow, increased apoptosis of progenitor and mature cells
  • Propensity for evolution into AML
7
Q

Why can acute leukaemia cause pancytopenia?

A
  • Proliferation of abnormal cells from leukaemia stem cells
  • Failure to differentiate or mature into normal cells
  • Prevent normal haemopoietic stem/progenitor development by hijacking/altering the haemopoietic niche and marrow microenvironment
8
Q

What can cause secondary bone marrow failure?

A
  • Drug induced e.g. chemo, chloramphenicol, alcohol - causes aplasia B12/folate deficiency (nuclear maturation can affect all lineages)
  • Infiltrative - non-haemopoietic malignant infiltration, lymphoma
  • Viral - HIV
9
Q

What can cause pancytopenia due to increased destruction?

A

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

10
Q

What can cause hypersplenism?

A
  • Splenic congestion - portal HPT, congestive cardiac failure
  • Systemic disease - rheumatoid arthritis
  • Haematological diseases - splenic lymphoma
11
Q

What are the clinical features of pancytopenia?

A

Anaemia + neutropenia + thrombocytopenia

12
Q

What can the clinical features of pancytopenia suggest?

A
  • The lack of circulating blood cells
  • The cause of the pancytopenia
13
Q

How is the cause of pancytopenia established?

A
  • History, exam
  • FBC, blood film
  • Bone marrow exam
  • Routine tests - B12/folate, LFTs, virology, autoantibody tests
  • Specialised tests - cytogenetics
14
Q

What needle is used for bone marrow trephine biopsy?

A

Jamshidi needle

15
Q

How does the marrow cellularity vary depending on cause of pancytopenia?

A
  • Hypocellular in aplastic anaemia
  • Hypercellular in myelodysplastic syndromes, B12/folate deficiency and hypersplenism
16
Q

What is the supportive treatment for pancytopenia?

A
  • Red cell transfusions, platelet transfusions
  • Neutrophil transfusions are NOT routine
  • Antibiotic prophylaxis/treatment
17
Q

How is pancytopenia treated due to a primary bone marrow disorder?

A
  • Malignancy - consider chemo
  • Congenital - consider bone marrow transplantation
  • Idiopathic aplastic anaemia - immunosuppression
18
Q

How is pancytopenia treated due to secondary bone marrow disorder?

A
  • Drug reaction - stop the drug
  • Viral - e.g. treat HIV
  • Replace B12/folate
19
Q

How is pancytopenia treated due to hypersplenism?

A

Treat cause if possible, consider splenectomy