Bone marrow failure Flashcards

1
Q

Symptoms of bone marrow failure

A

Anaemia - fatigue, tachycardia, SOB
Thrombocytopenia - bruising, petechiae, ecchymoses
Neutropenia - infection

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2
Q

Examples of bone marrow failure syndromes with failure of cell production

A

Agranulocytosis (single line)
Aplastic anaemia (all 3 lines)
Acquired

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3
Q

Causes of acquired bone marrow failure with failure of cell production

A

Infection - EBV, HIV, Hep C, parvovirus
Drug induced - NSAIDs, methotrexate, carbamazepine
Immune
Radiation
Vitamin deficiency - B12, folate
Infiltration - mets, myelofibrosis, Gaucher’s disease, myeloma

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4
Q

Pathophysiology of aplastic anaemia

A

Insult to bone marrow stem cell which triggers immune response
Damage to haemopoeitic stem cells
Resulting pancytopenia

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5
Q

Investigations for aplastic anaemia

A
History - symptoms, assess infection risk
FBC
Reticulocyte count
B12, folate
Infection screen 
LFTs
U+Es
TFTs
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6
Q

How to diagnose aplastic anaemia

A

2 bone marrow assessments (at a different time and from a different place)
Aspirate and trephine

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7
Q

Treatment for aplastic anaemia

A
Reduce infection risk
Aggressively treat any infections
Transfuse blood products if appropriate
Bone marrow transplant if young 
ATG and ALG immunotherapy
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8
Q

What conditions can aplastic anaemia transform to if untreated

A

20% Anaemia paroxysmal nocturnal haemoglobinuria

15% AML

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9
Q

Prognosis of untreated aplastic anaemia

A

<6 months

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10
Q

What are myelodysplastic syndromes

A

Bone marrow failure syndromes with ineffective cell production

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11
Q

Demographic affected by myelodysplastic syndromes

A

Incidence increases with increasing age

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12
Q

Primary vs secondary myelodysplastic syndromes

A

Primary usually idiopathic

Secondary usually due to a bone marrow insult e.g chemotherapy or radiotherapy

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13
Q

WHO classification of myelodysplastic syndromes

A

Refractory cytopenia with unilineage dysplasia
Refractory cytopenia with multi lineage dysplasia
Refractory anaemia with ring sideroblasts
Refractory anaemia with excess blasts (RAEB)-1
RAEB-2

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14
Q

FAB classification of myelodysplastic syndromes

A

Refractory anaemia
Refractory anaemia with ring sideroblasts
Refractory anaemia with excess blasts
Refractory anaemia with excess blasts in transformation (to AML)

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15
Q

Diagnosis of myelodysplastic syndromes

A
FBC
U+Es
LFTs
B12 
Folate
Infection risk 
Bone marrow aspirate and trephine
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16
Q

Treatment of myelodysplastic syndromes

A

Reduce infection risk
Transfuse blood products if appropriate
Chemo
BMT in young patients