Pancytopenia Flashcards

1
Q

What are the features of Fanconi’s anaemia?

A

Short stature

Skin pigment abnormalities

Radial ray abnormalities

Hypogenitilia

Endocrinopathies

GI defects

Cardiovascular

Renal

Haematological

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2
Q

What is the pathology in Fanconi’s anaemia?

A

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

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3
Q

What haematological cell abnormalities occur in Fanconi’s anaemia?

A

Macrocytosis followed by thrombocytopenia, then neutropenia

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4
Q

What is the risk of developing bone marrow aplasia with Fanconi’s anaemia?

A

84% by 20 years

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5
Q

What is the risk of developing leukaemia with Fanconi’s anaemia?

A

52% by 40

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6
Q

What are some of the causes of acquired primary bone marrow failure?

A

Intrinsic bone marrow problems:

  • aplastic anaemia
  • myelodysplastic disorders
  • acute leukaemia
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7
Q

What are some of the causes of secondary bone marrow failure?

A

Drugs e.g. chemotherapy, chloramphenicol

B12 or folate deficiency

Malignant: non-haemopoietic infiltration, lymphoma

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8
Q

What is aplastic anaemia?

A

Autoimmune attack against the haemopoetic stem cell

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9
Q

What can cause increased destruction of haematological cells causing pancytopenia?

A

Hypersplenism

Autoimmune

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10
Q

What is the difference in the splenic pool in a normal spleen and in hypersplenism?

A

Increased splenic pool

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11
Q

What is pancytopenia?

A

Anaemia, thrombocytopenia and neutropenia

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12
Q

How will the marrow cellularity appear in aplastic anaemia?

A

Hypocellular

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13
Q

What things will cause a hypercellular picture in bone marrow?

A

Myelodysplastic syndromes

B12/folate deficiency (maturation ‘failure’)

Hypersplenism

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14
Q

How is neutropenic sepsis managed?

A

Barrier nursing

GCSF

Piperacillin

Tazobactam

Gentamicin if severe

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15
Q

What is CD20 a marker of?

A

B cells (non-Hodgkins)

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16
Q

What is CD3 a marker of?

A

T cells

17
Q

Which drug targets CD20?

A

Retuximab

18
Q

Which drug targets CD30 and what for?

A

Brentuximab - Hodgkin’s lymphoma

19
Q

What will cause a predominant B cell response in lymph nodes?

A

Autoimmune disease

Infections

20
Q

What will cause a predominant phagocytic response in lymph nodes?

A

Tumour drainage

21
Q

What will cause a predominant T cell response in lymph nodes?

A

Viral infections

Drugs e.g. phenytoin

22
Q

What is the triad of hypersplenism?

A
  1. splenomegaly
  2. fall in one or more cellular components of blood
  3. correction of cytopenias by splenectomy