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Paediatrics YR4 > Case 10 - pallor > Flashcards

Case 10 - pallor Flashcards

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1
Q

Causes of haemolytic anaemia in children

A
  • Glucose 6 phosphatase dehydrogenase - G6PD
  • Sickle cell anaemia
  • Beta thalassaemia
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2
Q

Haemolytic anaemia with negative direct coombs test cause

A

This means it is non immune mediated - not IgG or IgM:
* DIC
* Enzyme defects - eg G6PD deficiency, pyruvate kinase
* Haemolytic uraemic syndrome - bloody diarrhoea, contaminated shiga toxin from e.coli
* Abnormal Hb - sickle cell, thalassaemia
* Red cell membrane defects - spherocytosis, elliptocytosis
* Thrombotic thrombocytopaenic purpura
* Micro-angiopathic anaemia - prosthetic valves, haemangiomas, DIC

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3
Q

Causes of macrocytic anaemia in children

A
  • Medications - anticonvulsants, zidovudine, immunosupressant meds
  • Vitamin B12 deficiency
  • Hypothyroidism
  • Blackfan-Diamond anaemia
  • Liver disease
  • Aplastic anaemia
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4
Q

Differentials for microcytic anaemia in a child

A
  • Beta thalassaemia
  • Iron deficiency
  • Lead poisoning
  • Sideroblastic anaemia
  • Chronic disease - usually normocytic but can become micro
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5
Q

Causes of direct coombs test +ve haemoltyic anaemia

A
  • Rhesus incompatibility
  • ABO incompatability
  • Autoimmune - warm, cold
  • Paroxysmal cold haemoglobulinuria
  • SLE, Rheumatoid arthiritis
  • Infections - mycoplasma, CMV, EBV
  • Drugs, malignancy
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6
Q

Megaloblastic macrocytic anaemia (B12/folate deficiency) characteristics

A
  • Glossitis
  • Angular cheillitis
  • Neurological symptoms
  • Jaundice - ineffective erythropoesis
  • Hypersegmented neutrophils
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7
Q

Blackfan-Diamond anaemia

A
  • Presents in first year of life
  • Pure red cell aplasia
  • Congenital abnormalities of face, hands, kidney and heart
  • Low reticulocytes
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8
Q

Characteristics of macrocytic anaemia caused by hypothyroidism

A
  • Reduced height velocity
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9
Q

Aplastic anaemia characteristics of macrocytic anaemia

A
  • Neutropenia
  • Thrombocytopenia
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10
Q

Characteristics of iron deficiency microcytic anaemia

A
  • Pallor
  • Irritable
  • Koilonychia
  • Pica
  • Brittle hair
  • Delayed motor development
  • Low MCV, MCH
  • Raised RDW
  • Low red cell count
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11
Q

Beta thalassaemia trait microcytic anaemia characteritsics

A
  • Asymptomatic
  • Low MCV
  • Normal RDW
  • Raised red cell count
  • Target cells on blood film
  • High Hb A2
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12
Q

Characteristics of sideroblastic anaemia causing microctyic anaemia

A
  • Low MCV
  • Raised RDW
  • Target cells
  • Siderocytes
  • Dimorphic blood film
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13
Q

Characteristics of lead poisoning cause of anaemia on blood film

A
  • Basophilic stippling on red cells
  • Haemolytic anaemia
  • Dense metaphyseal lines on radiographs
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14
Q

Iron studies and anaemia they mean

A
  • Iron deficiency - low serum Fe, high TIBC, 10% saturation, low ferritin (low iron, able to bind)
  • Chronic disease - low Fe, low TIBC, normal saturation, normal/increased ferritin (ferritin hogs, iron is bound, low available)
  • Thalassaemia - normal/increased Fe, normal TIBC, normal saturation, normal ferritin
  • Sideroblastic anaemia - increased Fe, increased TIBC, 100% saturation, increased ferritin (everything up)
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15
Q

Cause of target cells

A
  • Post splenectomy
  • Haemoglobinopathy
  • Severe iron deficiency
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16
Q

Cause of anisocytosis

A
  • Iron deficiency anaemia
  • Beta thalassaemia
  • Megaloblastic anaemia
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17
Q

Cause of Howell-Jolly bodies

A
  • Beta thalassaemia
  • Megaloblastic
  • Post splenectomy
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18
Q

Cause of poikilocytosis

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A
  • Beta thalassaemia
  • Severe iron deficiency
19
Q

Cause of heinz bodies

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A

Red cell enzyme defects (eg G6PD)

20
Q

Cause of spherocytes

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A
  • Hereditary spherocytosis
  • Immune haemolytic aneamia
  • Severe burns
  • Post transfusion
21
Q

Cause of basophilic stippling

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A
  • Lead poisoning
  • Beta thalassaemia
22
Q

Post splenectomy blood film features

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A
  • Howell jolly bodies
  • Acanthocytes
  • Target cells
  • Schistocytes
23
Q

What can hydroxycarbamide be used for in children with sickle cell anaemia?

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A

Increase the production of fetal Hb

24
Q

Initial investigations for child with anaemia

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A
  • FBC
  • Blood film
  • Reticulocyte count
  • Iron studies
  • B12 and folate
  • Billirubin
  • Direct Coombs test
  • Haemoglobin electrophoresis
25
Conditions that increase risk of leukaemia
* Down syndrome * Kleinfelter syndrome * Noonan syndrome * Fanconi syndrome
26
What to do if suspect leukaemia in child?
FBC within 48hrs
27
Cause of ITP
* Type II hypersensivity reaction * Antibodies that destroy platelets
28
Sickle cell - what is it
autosomal recessive condition affecting the gene for beta-globin on chromosome 11. One abnormal copy of the gene results in sickle-cell trait.
29
Triggers for sickle cell crisis
* Dehydration * Infection * Stress * Cold weather Or spontaneously
30
Types of sickle cell crisis
* Vaso-occlusive - sickle cells block capillaries --> distal ischaemia, can have fever * Splenic sequestration - blocked flow within spleen, enlarged and painful spleen, pooling --> anaemia and shock * Aplastic - absence of creation of new RBCs, triggered by parvovirus * Acute chest syndrome - blood vessels supplying lungs get clogged, cxr shows infiltrates
31
Triggers for acute chest syndrome
* Vaso-occlusive crisis * Fat embolism * Infection
32
Management of sickle cell
* Avoid crisis triggers * Prophylactic penicillin V * Treatment of crisis promptly * Vaccines * Hydroxycarbamide (for HbF) * Crizanlizumab - MAB targeting p selectin, prevents RBCs sticking to wall
33
Hereditary spherocytosis - key points
* RBC sphere shaped * Can have haemolytic crisis * Aplastic crisis cna be triggered by parvovirus
34
Management of spherocytosis
* Folate supplements * Splenectomy * Removal of gall bladder if gall stones a problem
35
Thalassaemia - what is it?
* Genetic defect * Protein chains - normally 2 alpha and 2 beta - defects lead to thalassaemia
36
What causes iron overload in thalassaemia?
* Faulty RBCs * Recurrent tranfsuions * Increased absorption of iron in response to anemia * - monitor ferritin
37
Management of alpha thalassaemia
* Monitor FBC and for complications * Blood tranfsuions * Splenectomy potentially * BM transplant can cure
38
3 types of beta thalassaemia
Thalassaemia minor, intermedia and major
39
Thalassaemia minor
* One abnormal and one normal gene * Mild microcytic anaemia * Monitor, no treatment needed
40
Thalassaemia intermedia
* Two abnormal copies of beta globin genes * Two defective or one defective and one deletion * More significant anaemia, blood transfusions and iron chelation
41
Thalassaemia major
* Homozygous for deletion gene * No functioning beta globin * Most severe - failure to thrive * --> severe anaemia, splenomegaly, bone deformities
42
Management of thalassaemia major
* Regular trasnfusions * Iron chelation * Bone marrow transplant
43
Management of G6PD deficiency
* Avoid fava beans * Avoid medications that trigger haemolysis - eg primaquine, nitrofurantoin, cipro, trimethoprim etc
44

Paediatrics YR4 (35 decks)

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