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Haem Flashcards

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1
Q

What is Hb like in neonates?

A

HIGH

to compensate for low oxygen concentration in utero

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2
Q

Why does Hb in neonates progressively fall?

A

Due to decreased RBC production

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3
Q

What are normal Hb ranges for neonate / infant / child?

A

neonate: >140
infant (1m-1y): >100
Child (1y-12y) >110

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4
Q

What are mechanisms of anaemia split into

A

Reduced production

  • ineffective erythropoiesis (e.g. iron deficiency)
  • red cell aplasia (NO PRODUCTION)

Increased destruction (haemolysis)

Blood loss

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5
Q

What are causes for red cell aplasia

A

Parvovirus B19

Diamond-Blackfan

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6
Q

What are causes for ineffective erythropoiesis

A

Iron deficiency
folic acid deficiency
Chronic inflammation, chronic renal failure

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7
Q

What are causes of haemolysis

A

RBC membrane disorder (e.g. hereditary spherocytosis)
RBC enzyme disorder (e.g. G6PD deficiency)
Haemoglobinopathy (thalassaemia, sickle cell)
Immune (haemolytic disease of newborn, AI haemolytic anaemia)

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8
Q

What are main causes of iron deficiency anaemia in neonates?

A

inadequate intake
malabsorption
blood loss

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9
Q

What are sources of iron for infants

A

breast milk
infant formula
cows milk
solids e.g. cereal

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10
Q

what are clinical fts of iron deficiency anaemia in the newborn

A 
Asymptomatic until Hb<60 
Fatigue 
Slow feeding 
Pale 
Pica (inappropriate eating of non-food e.g. soil)
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11
Q

What are investigations for iron deficiency anaemia

A

Blood film: microcytic hypo chromic anaemia (low MCV, low MCH)

Iron studies (ferritin low, serum iron low, TIBC high)

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12
Q

What is management for iron deficiency anaemia?

A

Dietary advice

Ferrous sulphate PO

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13
Q

How do you monitor iron deficiency anaemia

A

Check Hb after 4 weeks - levels should rise by 2g/100
Otherwise check compliance

Once Hb levels are normal, continue iron tx for 3 months to replenish stores

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14
Q

What are specific tests for hereditary spherocytosis

A

Dye binding assay

Osmotic fragility test

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15
Q

How do you manage hereditary spherocytosis:

A

Supportive
RBC transfusion
Folic acid supplementation

Consider phototherapy / exchange transfusion if baby also has jaundicer

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16
Q

What is G6PD deficiency

A

G6PD > rate limiting step in pentose phosphate pathway > necessary to prevent oxidative damage to RBC

Deficiency means they are susceptible to oxidants > haemolysis

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17
Q

What are triggers for G6PD deficiency haemolysis

A

Antimalarials
Antibiotics
Alnalgesics (aspirin)
Chemicals (Naphtaline, fava beans)

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18
Q

What is pattern of inheritance of G6PD

A

X linked recessive

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19
Q

What are clinical features of G6PD

A

NEONATAL JAUNDICE
Trigger
Pallor
Dark urine with haemoglobin AND urobilinogn

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20
Q

How do you diagnose G6PD

A

measure G6PD activity in blood

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21
Q

What is haemolytic disease of the newborn

A

Haemolysis due to antibodies against blood group antigens (ABO/RhD)

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22
Q

How do you identify haemolytic disease of the newborn

A

COOMBS TEST +

Essentially picks up if there are any RBC circulating with antibodies on them

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23
Q

What are main causes of blood loss in the foetus / newborn

A

maternal occult haemorrhage

TTTS

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24
Q

Why does anaemia of prematurity occur

A

Inadequate EPO production
Low RBC lifespan
Frequent blood sampling
Iron / folate deficiency

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25
What is another word for bone marrow failure
APLASTIC ANAEMIA
26
What are 2 inherited causes of aplastic anaemia
Fanconi | Schwachmai-Diamond Syndrome
27
Explain Fanconi Anaemia
TRIAD - congenital abnormalities - defective haematopoesis - high risk AML/Tumours
28
What are the congenital abnormalities in Fanconi Anaemia
NORD NEURO - micropthalmia, - microcephaly, - developmental delay ORTHO: - short stature, hip dislocation, scoliosis, SHORT THUMB RENAL - renal aplasia/hypoplasia - horseshoe kidney - double ureter DERM - cafe au last spots - hypo/hyperpigmented
29
How do you diagnose Fanconi anaemia
increased chromosomal breakage of peripheral blood lymphocytes
30
How do you manage Fanconi
BM transplant
31
What is the triad in Schwachman-Diamond Syndrome
TRIAD Schwachman-Diamond Syndrome: - BM failure - pancreatic failure - skeletal abnormality
32
Give examples of inherited bleeding disorders
haemophilia | vWF deficiency
33
Give examples of congenital clotting disorders
protein C deficiency Protein S deficiency Antithrombin deficiency FV leiden
34
What is mode of inheritance for haemophilia
X linked recessice
35
What is hallmark feature of haemophilia
Recurrent DEEP bleeding into joints, muscles
36
What is a bad consequence of haemophilia
arthritis
37
How do you manage haemophilia
Recombinant F8 for HA Recombinant F9 for HB Also give prophylactic F8 if severe HA to reduce risk of joint damage Also Desmopressin (DDAVP) in mild haemophilia A
38
What is hallmark feature of vWD
SUPERFICIAL BLEEDING | epistaxis, menorrhagia
39
How do you manage vWD
Type 1 (poor quality): DDAVP T2/3: plasma derived F8 concentrated
40
What must you avoid in vWD/haemophilia
IM injections aspirin NSAIDS
41
What is a dangerous consequence of vitamin K deficiency in babies
Haemorrhagic disease of the newborn
42
What is vitamin K necessary for
F2, 7, 9, 10 | Protein C, S production
43
What is the cause of ITP
Antiplatelet IgG antibodies | Cause destruction of circulating platelets
44
How does ITP present
In children, few weeks after viral infection Children develop petechiae, purpura, superficial bruising Epistaxis, mucosal bleeding *** beware of intracranial bleeding
45
How do you manage mild ITP
self resolving | manage at home with corticosteroids or IVIG
46
How do you manage severe ITP
IVIG, corticosteroids, platelet transfusion
47
Explain how MAHA occurs
MECHANISM NOT DISEASE clot forms in blood vessel > blood cannot flow nicely > sheared into small fragments > schistocytes on blood film + anaemia, jaundice
48
Explain how HUS occurs
E coli 0157:H7 produces shiga-like toxin Toxin damages endothelium of glomerular vessel causes platelet aggregation > thrombocytopenia shearing of RBC > MAHA less end organ perfusion > RENAL failure
49
What is the triad of HUS + other symptom to remember
MAHA + THROMBOCYTOPOENIA + RENAL FAILURE + diahrroea
50
How does TTP occur
antibodies against ADAMTS13 causes vWF to stick together > clots form > platelets all stuck together cause same as above + diminished end organ perfusion to brain as well, causing perfusion
51
What is PENTAD of TTP
MAHA + RENAL FAILURE + THROMBOCYTOPOENIA + FEVER + ALTERED MENTAL STATE
52
What is the cause of DIC
sudden increase in exposure to tissue factor (due to sepsis, tumour, pancreatitis, pregnancy, trauma) Causes activation of coagulation cascade > clotting body attempts to reverse the clotting via normal anti clot mechanism causes massive consumption of all clotting factors
53
How does DIC present
in VERY UNWELL patient | Everything is wrong: high PT, APTT, low platelets
54
EXPLAIN aetiology of DIC
Increase in tissue factor Causes activation of coagulation cascade > clotting body attempts to reverse the clotting via normal anti clot mechanism causes massive consumption of all clotting factors
55
what does ITP stand for
Immune / Idiopathic Thrombocytopenic Purpura
56
What is the function of desmopressin in haemophilia/VWD
stimulates release of F8 and vWF
57
How do you differentiate ineffective erythropoiesis from red cell aplasia on blood test
LOW RETICULOCYTES in red cell aplasia HIGH RETICULOCYTES in ineffective erythropoesis
58
What should you suspect if reticulocytes are low?
Red cell anaemia > parvovirus B19 OR Diamond Blackfan
59
What investigations should you get if low reticulocytes
Parvovirus serology | BM aspirate
60
What is sideroblastic anaemia
Bone marrow produces ringed sideroblasts instead of healthy RBCs This causes a microcytic anaemia
61
Why does sideroblastic anaemia occur
Body has iron but cannot incorporate it properly into Hb
62
What do sideroblasts look like
nucleated erythrocytes (precursors) with iron granules surrounding nucleus
63
What are causes of sideroblastic anaemia
CONGENITAL - genetic AQUIRED: - Myelodysplastic syndrome - AML - Alcohol use - B6 deficiency - Lead poisoning, copper poisoning
64
What do you see on blood film for sideroblastic anaemia
- basophillic stippling | - target cells
65
What THREE TYPES of anaemia can you categorise
Microcytic (MCV<80) Normocytic (MCV 80-100) Microcytic (>100)
66
What are causes of MICROCYTIC anaemia
Iron deficiency Thalassaemia Anaemia of chronic disease Sideroblastic anaemia
67
How can you further subdivide NORMOCYTIC anaemia
LOW reticulocytes | NORMAL /HIGH reticulocytes
68
What are causes of NORMOCYTIC anaemia with LOW reticulocytes
Aplastic anaemia (Fanconi, Schwachen-DIamond) Red cell aplasia (Parvovirus 19, Diamond-Blackfan) Malignancy Renal disease
69
What are causes of NORMOCYTIC anaemia with NORMAL reticulocytes (AKA HAEMOLYTIC CAUSES)
INTRINSIC CAUSES - Sickle cell - Membrane defect (spherocytosis, elliptocyt, paroxysmal nocturnal haemoglobinuria) - Enzyme deficiency (G6PD, PK deficiency) - immune (ABO/RhD incompatibility, warm/cold AIHA) EXTRINSIC CAUSES - Infection e.g. Malaria - MAHA
70
What are causes of MACROCYTIC anaemia
MEGALOBLASTIC - B12/ Folate deficiency NON-MEGALOBLASTIC - liver diserase - alcohol - drugs
71
Explain beta thalassamia major
MOST SEVERE FORM | NO HbA - due to NO functioning beta-globin gene
72
How do you manage beta thalassamia major
FATAL without regular blood transfusions Give BLOOD TRANSFUSIONS + IRON CHELçATION BONE MARROW TRANSPLANT IS CURE
73
What is HYDROPS Fetalis
oedema in min 2 fluid compartments + ascites
74
What are causes for Hydrops
``` foetal anaemia (iron deficiency, parvovirus B19( Maternal infection (CMV, syphilis9 TTTS ```
75
How do you treat Hydrops foetal is
EXCHANGE TRANSFUSION
76
What is the whole spectrum of disease caused by Parvovirus B19
FIFTH DISEASE OR erythema infectiosum OR "slapped cheek syndrome". Prodrome of headache, fever, nausea Bright red rash on cheeks (periorbital pallor) Rash can stretch to trunk and extremities
77
what are examples of iron chelation regimens available for beta thalassaemia
SC desferrioxamine | Oral deferasirox
78
How do you manage DIC
1. Treat underlying cause (usually sepsis) 2. Supportive care 3, Replacement therapy (platelet transfusion for platelets, FFP for coag factors, cryoprecipitate transfusions) Consider Protein C concentrate esp in purpura fulminans
79
How do you manage hereditary spherocytosis
supportive care, RBC transufion Folic acid supplement Consider splenectomy + encapsulated bacteria vaccine cholecystectomy
80
What prophylaxis do you give for sickle cell
immunise against encapsulated organisms Daily oral penicillin Daily oral folic acid Avoid triggers
81
How do you treat acute crisis of SCD
``` Oral, IV analgesia Good hydration Antibiotics for infection Oxygen if reduced sats Exchange transfusion ```
82
What management can you consider for chronic problems in sickle cell
- hydroxycarbamide (if recurrent admissions) - splemnectomy + imms against encapsulated bacteria - BM transplant if sevre

Y5 Paediatrics (30 decks)

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