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Paeds FOP/TAS > Haem Onc > Flashcards

Haem Onc Flashcards

1
Q

Define anaemia

A

WHO definition - Number of RBCs and O2 carrying capacity is insufficient to meet the body’s physiological needs

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2
Q

Categorise the causes of anaemia

A

Reduced red cell production

Increased red cell loss

Increased red cell destruction

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3
Q

3 phases of iron deficiency

A

Depletion
- Normal Hb, low ferritin, low t sats

Deficient erythropoeisis
- low ferritin, t sats and serum iron

Anaemia
- Low Hb, t sats and serum iron

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4
Q

What is serum ferritin a marker of?

A

Measure of iron stores in liver and bone marrow.
Also an acute phase protein so increased in inflammatory states.

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5
Q

Risk factors for iron deficiency

A

<37 weeks + / - LBWT
Multiple pregnancy
Breast fed babies
Early weaning to Cow’s milk
Adolescent girls - menstruation
Restrictive diets - vegetarians
Pica
Chronic disease
Malabsorption - coeliac disease, h. pylori, IBD
Hookworm

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6
Q

Blood film findings in IDA

A

Hypochromic, microcytic anaemia

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7
Q

What form does iron mostly exist in in the body?

A

Haeme

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8
Q

Non-haematological effects of iron deficiency

A

Reduced CNS higher functions
Diminished T cell function and cell-mediated immunity
Diminished muscle performance

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9
Q

What foods is B12 found in?

A

Present in animal products (eggs, meat, fish, dairy)

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10
Q

Blood film findings in B12 deficiency?

A

Oval macrocytes, hyper segmented neutrophils

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11
Q

What else can be checked if b12 indeterminate but high clinical suspicion?

A

Check MMA (methylmalonic acid)

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12
Q

What else can be checked if b12 indeterminate but high clinical suspicion?

A

check MMA (methylmalonic acid)

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13
Q

Foods containing folate

A

Fortified cereals and foods
Green leafy vegetables
Fruit
Yeast

Note - folate is destroyed by prolonged cooking

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14
Q

Causes of folate deficiency

A

Dietary deficiency
Chronic infections - HIV, malaria
Drugs

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15
Q

Blood film findings in folate deficiency

A

Hyper segmented neutrophils, oval macrocytes, can develop pancytopenia

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16
Q

Presentation of copper deficiency

A

Ataxia
Proprioception difficulties

17
Q

What type of anaemia can pyridoxine (Vit B6) deficiency cause?

A

Sideroblastic anaemia (rare)

18
Q

What condition is basophilic stripping seen in?

A

Lead toxicity

19
Q

Bone marrow changes seen in Anorexia Nervosa?

A

Gelatinous changes (due to fat cell atrophy)

20
Q

Mechanisms of neutropenia

A

Inadequate production (Ineffective granulopoiesis, bone marrow repalcement)

Destruction at source (haemophagocytic syndromes)

Defective release (myelokathexis)

Splenic pooling (Felty’s syndrome)

Shortened intravascular lifespan (immune mediated destruction)

Rapid egress from tissues (sepsis in neonates)

21
Q

What is NAIT

A

NeoNatal Allo Immune Neutropenia

22
Q

Inheritance pattern of hereditary spherocytosis

A

Autosomal dominant (75%)
Autosomal recessive (25%)

23
Q

Most common non-haematological complication in patients with hereditary spherocytosis

A

Gallstones

24
Q

5 genes which can be affected in hereditary spherocytosis

A

ANK1
SLC4A1
SPTA1
SPTB
EPB42

All the above genes code for proteins that are part of the red blood cell membrane

25
Q

Red cell membrane proteins affected in hereditary spherocytosis

A

Spectrin
Anykrin
Protein 4.2

These are the major components of the cytoskeleton responsible for red blood cell shape

26
Q

Pathophysiology of why spherocytic red blood cells are destroyed prematurely in the spleen in hereditary spherocytosis

A

The decrease in the deformability of the spherocytic red blood cells causes impaired cell passage from the splenic cords to the splenic sinuses

27
Q

Mx of hereditary spherocytosis

A

Steroids
Folate supplementation
Red cell transfusions
Splenectomy (>6 yrs, mod-severe disease only, will then also need Abx prophylaxis and additional vaccines)

28
Q

Which infection can cause sudden anaemia in hereditary spherocytosis

A

Parvovirus

29
Q

What is Shwachman-Diamond syndrome

A

Genetic condition (autosomal recessive) causing bone marrow failure and neutropenia

Associated with exocrine pancreas dysfunction

Paeds FOP/TAS (12 decks)

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