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3A Paediatrics > Paediatric haematology > Flashcards

Paediatric haematology Flashcards

1
Q

What is the definition of anaemia?

A

A condition in which there is a deficiency of red cells or of haemoglobin in the blood to meet the body’s needs.

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2
Q

Normal ranges for blood in children

A

slide 6 paediatric haematology

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3
Q

What is the pathogenesis of anaemia

A
  • Blood loss
    Acute haemorrhage
    Chronic gut bleeding leading to iron deficiency
  • Decreased Production
    Nutritional deficiency e.g. iron, folate, B12, Vitamin C
    Bone marrow failure e.g. DBA, TEC
    Infiltration e.g. Acute Leukaemia, Neuroblastoma, Lymphoma, Osteopetrosis, Storage Disease
  • Increased consumption
    Acquired e.g. immune, drugs, parasites, MAHA
    Inherited e.g. red cell membrane defects, enzyme defects
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4
Q

What are the blood parameters?

A

Hb (always check normal range for age and sex)
Is the abnormality isolated to a single cell line or part of multiple cell lines (?BM failure, infiltration, immune, hypersplenism)
MCV (microcytic, normocytic, macrocytic)

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5
Q

EG of microcytic RBC conditions

A

Fe Deficient
Thalassaemia
Sideroblastic anaemia
Chronic disease
Lead toxicity
Copper deficiency
Haemoglobin E trait
Severe malnutrition

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6
Q

Macrocytic RBC

A

Newborn
Aplastic anaemia
Hypothyroidism
Megaloblastic anaemia
Increased erythropoiesis
Fanconi anaemia
PNH
Drugs
Post splenectomy

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7
Q

Normocytic RBC

A

Acute blood loss
Infection
Renal failure
Early Fe deficiency
Bone marrow infiltration
Haemolysis
Hyperslpensim
Drugs

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8
Q

What are reticulocytes?

A

Immature red blood cells
Typically 1% of RBC in humans
Develop in bone marrow and then circulate in blood for 1 day prior to developing into mature RBC.
Useful in establishing if marrow producing RBC effectively

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9
Q

What are blood films?

A

Very helpful in the diagnosis of anaemia
It establishes if hypochromic, microcytic, normocytic, macrocytic
It shows specific morphological abnormalities e.g. spherocytes, sickle cells, elliptocytes, schistocytes etc.
Allows review of other cell lines also e.g. platelets, white blood cells

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10
Q

Most common anaemia of childhood and what can cause it

A

Fe defi
LBW, dietary- excessive cows milk intake, occult GI bleeding (e.g. hookworm), cow’s milk intolerance

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11
Q

Presentation of Fe deficiency

A

pallor, irritability, anorexia when Hb<50, tachycardia, cardiac dilatation, murmur, poss. splenomegaly

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12
Q

Blood results of Fe deficiency

A

microcytic, hypochromic, low-normal retics

Low ferritin and serum iron, Increased TIBC

High ZPP

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13
Q

Treatment of Fe

A

ORAL THERAPY – Mainstay

Oral iron dose is 6mg/kg/day of elemental iron

reticulocytosis in 72 hr, Hgb responds at ~10g/L per wk, iron stores replenished by 3 mo

treatment is needed for 3-6 months
constipation common

commonest cause of failure is non-compliance
address cause- usually diet

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14
Q

What is haemolysis?

A

Increased RBC turnover, shortened RBC lifespan
RBCs are fragile- especially abnormal ones
Spleen filters out and breaks down senescent RBCs, and must work overtime, and can result in effective asplenia (e.g. in sickle cell)
RBC degradation products must be handled

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15
Q

What are the intra corpuscular causes of RBC destruction

A

Haemoglobin
Enzyme
Membrane

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16
Q

What are extra corpuscular causes of destruction of RBC

A

Autoimmune
Fragmentation
Hyper splenism
Plasma factors

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17
Q

Presentation of haemolytic anaemias

A

Hydrops fetalis
Neonatal hyperbilirubinaemia
Neonatal ascites
Anaemia/failure to thrive
Splenomegaly
Cholecystitis/gall stones
Hyperbilirubinaemia
Leg ulcers
Aplastic crisis
Thromboembolism

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18
Q

Severe anaemia at birth features

A

Haemolytic disease of the newborn

Bleeding
umbilical cord
internal hemorrhage

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19
Q

What is haemolytic disease of the newborn?

A

Rh negative mother previously sensitised to Rh pos cells
Transplacental passage of antibodies
Haemolysis of Rh Pos fetal cells

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20
Q

S + S of haemolytic disease of the newborn?

A

Signs and Symptoms
severe anemia
compensatory hyperplasia & enlargement of blood forming organs (spleen and liver)
Treatment
prevention of sensitization with Rh immune globulin
intrauterine transfusion of affected fetuses

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21
Q

What are the 3 main presentations of G6PD?

A

Neonatal jaundice
Chronic non-spherocytic haemolytic anaemia
Intermittent episodes of intravascular haemolysis

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22
Q

Features of G6PD

A

Sporadic haemolysis
Typically induced by drugs, fava beans, fever, acidosis
Intravascular haemolysis - haemoglobinuria, rigors, fever, back pain
Treated by stopping precipitant, transfusion, renal support

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23
Q

Features of hereditary spherocytosis

A

Commonest hereditary haemolytic anaemia in Europeans - 1/5000; probably rarer in Africa
Typically autosomal dominant, but no family history in 25% cases
Heterogeneous - deficiencies of spectrin (41.5%), ankyrin (1.5%), band 3 (17%), band 4.2 (21.5%)
Clinical effects vary from mild to transfusion dependence; tends to be similar within families

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24
Q

What is sickle cell disease?

A

The most common serious genetic disorder in England affecting over 1 in 2000 live births. (Autosomal recessive)
A term covering a number of different but similar conditions that affect haemoglobin.
Types most commonly seen in UK:
Sickle Cell Anaemia (HbSS)
Sickle Haemoglobin C disease (HbSC)
Sickle Beta Thalassaemia (HbS/β thal)

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25
Pathophysiology of sickle cell disease
Substitution of valine for glutamic acid on β chain (HbS) HbS polymerises when deoxygenated leading to sickle shape Occlusion of the microvascular circulation producing vascular damage, infarcts, pain Shortened survival of red cells leading to haemolysis
26
How will we diagnose sickle cell disease?
Blood Film Sickle solubility HPLC
27
What can we do to treat sickle cell disease?
Education – keeping warm, well hydrated Prophylactic Penicillin V to prevent infection due to splenic hypofunction (anti malarials when required) Ensure immunisations are up to date to prevent infection Screening such as TCD monitoring, G+M Effective pain management (NICE requirement) Prompt treatment of infections Bone Marrow Transplant Hydroxycarbamide New agents e.g. Crizanlizumab, L-Glutamine
28
What are some complications of HbSS
Enuresis Priapism Avascular necrosis Chronic ankle ulceration
29
What is thalassaemia?
Normal Adult HbA = α2β2, HbA2 = α2δ2 HbF = α2γ2 Thalassaemia = reduced rate of one or more of the globin chains Autosomal recessive α thal (4 α globin genes per cell αα/αα) β thal (2 globin genes per cell)
30
What is Beta thalassaemia
β Thalassaemia Trait ( carrier state) asymptomatic Mild anaemia, low MCV, Raised Hb A2
31
Features of B Thalassaemia
β Thalassaemia Major Progressive Severe Anaemia, low MCV, Hb F and A2 increased Jaundice Splenomegaly Failure to thrive Skeletal Deformity Delayed puberty Death early teens/adulthood
32
B Thalassaemia management
Genetic Counselling, AN diagnosis Regular blood transfusion Complications of Iron overload Liver, Heart, Pancreas, Endocrinopathy Iron chelation Bone Marrow Transplantation/Gene Therapy
33
What is haemostasis and what does it depend on?
Coagulation cascade - pl Platelets Numbers Function Coagulation factors Vascular integrity
34
Coagulation cascade
Slide 35 Paediatric haematology
35
Factors involved in Intrinsic pathway
Prekallikrein HMWK FXII FXI FIX FVIII
36
Factors involving Extrinsic pathway
FVII
37
Common pathway in coag cascade
FV FX Prothrombin Fibrinogen
38
What are the causes of thrombocytopenia
Increased Plt destruction (Immune and non immune) Dec Platelet production Disorders of platelet pooling Pseudothrombocytopenia
39
What causes increased PLT destruction (IMMUNE) in thrombocytopenia
ITP Secondary to infection (HIV, Hepatitis, CMV, EBV, Parvovirus, mumps, measles, pertussis) Drug induced – valproate, ciprofloxacin, ibuprofen, phenytoin, ranitidine, heparin, captopril Autoimmune –Evans syndrome SLE NAIT Hyperthyroidism
40
What causes increased plt destruction (Non immune) in thrombocytopenia
Microangiopathic (TTP, HUS) Kasabach-Merritt Syndrome Drugs Infection (viral associated haemophagocytic syndrome) (bacterial infection) Disseminated Intravascular Haemolysis
41
What causes decreased platelet production in thrombocytopenia
Constitutional (TAR, CAMT) Ineffective thrombopoiesis ( B12, Folate Deficiency, severe iron deficiency)) Infiltration (Leukaemia, Non haem infiltration, Osteopetrosis, storage disease) Metabolic disorders (Ketotic glycinaemia, MMA, Acidaemia) Bone Marrow Failure (Aplastic Anaemia)
42
What are some disorders of platelet pooling
Hypersplenism (Portal Hypertension, Gauchers disease)
43
What is pseudo thrombocytopenia?
Platelet activation during blood collection
44
What is the most common form immunologic thrombocytopenia
ITP
45
Features of ITP
Identified if well child, acute onset and no other concerning features, history with normal examination (other than bruising, petechiae etc) Important to review blood film periodically to ensure no evolving serious bone marrow disorder Acute 0-3 months Persistent >3-12 months Chronic >12 months Treatment rarely required unless bleeding – steroids, IVIG, TPO-RA
46
What are coagulopathies
Various errors in clotting cascade Bleeding disorders hemophilia von Willebrand disease Hypercoagulable states antithrombin, protein C, protein S, FVL, PT mutation, APS
47
What is Von willebrand disease
VWD is the commonest inherited bleeding disorder that varies in severity according to the degree of deficiency and the specific characters of the molecule that is altered.
48
WHAT ARE THE 2 MAIN ROLES OF VWF?
Mediates the adherence of platelets at sites of endothelial damage helping form platelet plug Binds and transports FVIII, protecting it from degradation
49
What is the deficiency in VWD?
Deficiency of VWF (type 1) Dysfunction of VWF (type 2A, B, M,N) Complete absence of VWF (type 3)
50
S + S of VWD?
Easy bruising Epistaxis Menorrhagia Mucosal bleeding Following surgery or trauma
50
how IS vwd INHERITED?
AD or AR for type 3 or 2N
51
How can we investigate VWD?
Clotting often shows prolonged APTT (not always!) VWD screen – FVIII, VWF:Ag, VWF activity Further investigations can be performed to identify type 1 from 2 Function:antigen ratio <0.6 RIPA Multimer Analysis Genetic analysis
52
How do we manage VWD?
Tranexamic Acid An antifibrinolytic useful for menorrhagia or mucosal bleeding Given pre procedures IV 10mg/kg and orally 15-25mg/kg tds for other bleeding or post operatively Desmopressin Used to elevate FVIII and VWF levels by releasing endothelial stores. Given IV, SC or intranasal Perform a DDAVP trial if possible to assess response Excessive fluid intake can reduce sodium so restrict fluid to 1L for 24 hours post dose Avoid use if <2 years or monitor sodium closely and avoid if know atherosclerosis Avoid in Type 2B as thrombocytopenia due to clearance of large multimers. VWF- containing concentrates Given if DDAVP inadequate or contraindicated Voncento, Wilate Given as IV bolus 12-24 hours as required by bleeding and levels. Plasma products so ensure hepatitis vaccinations given pre where possible
53
What are Haemophilia A and B and what does it cause?
Deficiency of Factor VIII/IX, ↑APTT X linked recessive- boys Prolonged bleeding Muscle bleeds Joint bleeds > arthritis and deformity Treatment Factor VIII/IX Complications of treatment
54
Presentation of severe moderate and mild haemophilia
Severe - <1IU/dl or <1% of normal Spontaneous bleeding into joints or muscles Moderate - 1-5IU/dl or 1-5% of normal Occasional spontaneous bleeding, prolonged bleeding with minor trauma or surgery Mild - 5-40IU/dl or 5-40% of normal Severe bleeding with major trauma or surgery. Spontaneous bleeding rare
55
How do we manage the bleeding in haemophilia?
Tranexamic Acid Factor concentrate( standard life and extended half life) – recombinant e.g. Advate, Benefix, Elocta Emicizumab (recombinant humanised bispecific monoclonal antibody mimicking the co-factor of activated FVIII)
56
When does leukaemia occur?
When WBC starts multiplying out of control
57
Common symptoms of leukaemia
Weight loss Fever Infection Fatigue Loss of appetite Spleen and liver enlargement Anaemia Bone joint paint Purple patches or spots Night sweats
58
Types of paediatric leukaemia
ALL - 85% AML - 13% Others - 2%
59
How do we make the diagnosis of ALL
Blood count Anaemia WCC up or down Neutropaenia Thrombocytopaenia Blast cells Bone Marrow LP
60
Features of ALL
Most common malignancy Peak age 4-7yrs Prognosis 85% cure Good Prognostic factors Age 2-10 Female WCC<50 No CNS disease Classified also on cell type B and T cell
61
Treatment for leukaemia
Supportive Prompt treatment of infection Prevention of tumour lysis Blood product support Specific Chemotherapy (ALL – Altogether, AML – Myechild) Targeted therapy Bone marrow transplant
62
Late effects of treatment
Psychological Family and social Growth particularly CNS RT Endocrine Puberty Fertility Intellectual Second malignancies

3A Paediatrics (19 decks)

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