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Haematology > Pancytopenia > Flashcards

Pancytopenia Flashcards

1
Q

secondary bone marrow failure causes

A

drug induced

B12/folate deficiency

Infiltrative- non-haemopoietic malignant infiltration, lymphoma

Misc.: Viral (eg HIV)/storage diseases

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2
Q

pancytopenia definition

A

a deficiency of blood cells of all lineages (but generally excludes lymphocytes)

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3
Q

neutrophil lifespan

A

7-8 hours

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3
Q

red cell lifespan

A

120 days

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4
Q

increased destruction cause

A

hypersplenism

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5
Q

causes of hypersplenism

A

Splenic Congestion
- Portal Hypertension

Systemic diseases
- Rheumatoid Arthritis

Haematological diseases
- Splenic lymphoma

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6
Q

pancytopenia is _______ + ___________ + __________

A

anaemia
neutropenia
thrombocytopenia

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7
Q

what supportive treatment options are there for pancytopenia

A

Red cell transfusions
Platelet transfusions
(Neutrophil transfusions not routine)

Antibiotics prophylaxis/treatment
- antibacterials
- antifungals

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8
Q

pancytopenia due to secondary bone marrow disorder treatment

A

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

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9
Q

establishing the cause of pancytopenia (6 points)

A
  • history, including family history
  • clinical findings
  • FBC, blood film
  • additional routine tests guided by above (B12/folate, LFTs, virology, autoantibody tests)
  • bone marrow examination
  • specialised tests guided by above (cytogenetics, e.g. chromosome fragility testing in Fanconi’s syndrome, Next generation sequencing. Whole genome sequencing)
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10
Q

platelet lifespan

A

7-10 days

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11
Q

reduced production cause of pancytopenia

A

bone marrow failure
- inherited syndromes
- acquired: primary or secondary

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12
Q

marrow cellularity in pancytopenia

A

variable depending on the cause:

hypocellular in aplastic anaemia

hypercellular in:
- myelodysplastic syndromes
- B12/folate deficiency
- hypersplensim

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13
Q

should you wait for microbiology results before starting treatment for neutropenic fever?

A

no just start antibiotics promptly based on local unit antibiotic policy

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14
Q

specific treatment for pancytopenia: malignancy

A

consider treatment (chemotherapy)

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15
Q

specific treatment for pancytopenia: congenital

A

consider allogeneic stem cell transplantation

16
Q

specific treatment for pancytopenia: idiopathic aplastic anaemia

A

immunosuppression

17
Q

specific treatment for pancytopenia: Myelodysplastic syndromes

A

depends on risk of leukaemic transformation

erythropoiesis - stimulating agents in low risk, demethylating agents or chemotherapy in higher risk

18
Q

secondary bone marrow disorder causes of cytopenia treatments

A
  • drug reaction: stop
  • viral: e.g. treat HIV
  • replace B12/folate
19
Q

hypersplenism cause of pancytopenia treatment

A

treat cause if possible
consider splenectomy (not appropriate in all cases)

20
Q

Inherited marrow failure syndromes can be a cause of pancytopenia.
Give an example of an inherited marrow failure syndrome (very rare)

A

Fanconi’s anaemia

21
Q

Features of Fanconi’s anaemia

A
  • short stature
  • skin pigment abnormalities
  • MSK - radial ray abnormalities
  • hypogenitilia
  • endocrinopathies
  • GI defects
  • Cardiovascular
  • Renal
  • Haematological
22
Q

Fanconi’s anaemia average age of onset

A

7

23
Q

Fanconi’s anaemia - what is the problem

A

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

24
Q

pancytopenia - acquired primary bone marrow failure examples

A
  • idiopathic aplastic anaemia
  • Myelodysplastic syndromes (MDS)
  • Acute leukaemia (total white cell count can be high due to an excess of circulating blasts but patients can present with pancytopenia)
25
Q

idiopathic aplastic anaemia

A

Aplastic Anemia (AA) is also known as idiopathic aplastic anemia (IAA) and the production of new blood cells ceases in AA, which leads to an abnormal hematological syndrome such as pancytopenia and suppression of hypo-cellular bone marrow.

autoimmune attack against haemopoietic stem cells

26
Q

myelodysplastic syndromes - hyper or hypocellular marrow?

A

hypercellular

27
Q

myelodysplastic syndromes have propensity for evolution into _______

A

acute myeloid leukaemia (AML)

28
Q

what’s the problem in myelodysplastic syndromes

A

increased apoptosis of progenitor and mature cells (ineffective haemopoiesis)

29
Q

pancytopenia - increased destruction: hypersplenism

A

increased splenic pool (too many cells getting stuck in the spleen??)

increased destruction that exceeds bone marrow capacity, usually associated with significantly enlarged spleen

Haematology (36 decks)

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