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Child Health > Haemaology/oncology > Flashcards

Haemaology/oncology Flashcards

1
Q

Childhood malignancy epidemiology

A

1/500 by 15y
1500 in the UK each year
Leukaemia: 32%, all ages, ALL = 80%, other 20% AML and ANLL
Brain/spinal tumours: 24%
Lymphoma: 10%, Hodgkin peaks in early life, then adolescence
Neuroblastoma: 7%, 1st 6y of life
Soft tissue sarcoma: 7%
Wilms tumour: 6%, 1st 6y of life
Bone tumour: 4%, peaks in early life, then adolescence
Retinoblastoma: 3%
Others: 7%

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2
Q

ALL presentation

A

Peak at 2-5yrs: clinical presentation results from infiltration of the bone marrow or other organs with leukaemic blast cells
Malaise, anorexia
Anaemia: pallor, lethargy
Neutropenia: Infection
Thrombocytopenia: bruising, petechiae, epistaxis
Bone pain
Reticulo-endothelial infiltration: hepatosplenomegaly, lymohadenopathy (superior mediastinal obstruction)
CNS infiltration: headaches, vomiting, nerve palsies
Testes infiltration: testicular enlargement

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3
Q

ALL investigations

A

FBC: low Hb, thrombocytopenia, circulating leukaemic blast cells
Bone marrow examination: confirm diagnosis, identify immunological/cytogenetic characteristics for prognostic info
CXR: identify if mediastinal mass characteristic of T cell disease

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4
Q

ALL initial management

A

Induction (4w): vincristine, dexamethasone, L-asparaginase, IT methotrexate
Consolidation & CNS protection: IT methotrexate, vincristine, steroid, theopurine
Interim maintenance: monthly vincristine, daily 6-mercaptopurine, weekly oral methotrexate, prophylactic co-trimoxazole, IT methotrexate
Delayed intensification: vincristine, dexamethasone, doxorubicin, L-asparaginase, IT methotrexate, cyclophosphamide, cytarabine
Continuing maintenance (2y in girls, 3y in boys): monthly vincristine, daily 6-mercaptopurine, weekly oral methotrexate, prophylactic co-trimoxazole, IT methotrexate

Blood transfusions: for symptomatic relief, not cure
Co-trimoxazole: prophylaxis for Pneumocystis Cariniipneumonia

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5
Q

Tumour lysis syndrome features

A

Before and during the initial induction phase of chemotherapy
Metabolic derangements cause by the systemic rapid release of intracellular contents

Hyperuricaemia
Hyperphosphataemia
Hypocalcaemia
Hyperkalaemia

Treatment:
Monitor U&Es
IV fluid therapy
Allopurinol may also be given

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6
Q

ALL relapse treatment

A

High-dose chemotherapy

Total body irradiation (TBI) + bone marrow transplantation

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7
Q

ALL high-risk prognostic factors

A

Age: <1y, >10y
Tumour load (WCC): >50x10’9/L
Genetic abnormalities: MLL rearrangement, t(4;11), hypodiploidy (<44 chromosomes)
Response to initial chemo: persistance of leukaemic blasts in bone marrow
MRD: high

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8
Q

ALL tumour cell classification

A

L1 – small uniform cells
L2 – large varied cells
L3 – large varied cells with vacuoles

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9
Q

Changes to therapy for specific types of ALL

A

T-cell ALL: cyclophosphamide, intensive treatment with L-asparaginase
Mature B-cell ALL: treat like a lymphoma, short-term intensive chemotherapy + high dose methotrexate

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10
Q

Which cells are myeloid and which are lymphoid?

A

Myeloid: platelets, erythrocyte, mast cell, basophil, neutrophil, eosinophil, monocyte, macrophages

Lymphoid: natural killer cell, T lymphocyte, B lymphocyte, plasma cell

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11
Q

Where are the sites of haematopoiesis

A

Foetal life: mainly liver

Post-natal life: mainly bone marrow

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12
Q

Neonatal blood count values

A

Iron, folic acid and vitamin B12 stores: adequate in term & preterm infants (lower)
WCC: higher in neonates (10-20x10’9/L)
Platelets: similar to adults: 150-450x10’9/L)
Hb: higher

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13
Q

Anaemia Hb levels in children

A

Neonate: Hb<14
1-12m: Hb<10
1-12y: Hb<11

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14
Q

Reasons for the physiological changes in haemoglobin from neonates to adults

A

Fetal Hb: 2 alpha + 2 gamma units
Adult Hb: 2 alpha + 2 beta units
HbF gradually replaced by HbA in 1st year of life
At term, Hb is high to compensate for low O2 concentrations in the foetus
Hb falls over the 1st weeks due to RBC production

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15
Q

Iron deficiency anaemia clinical features

A

Fatigue, slow feeding, conjunctival/tongue/palmar crease pallor

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16
Q

Iron deficiency anaemia investigations

A

FBC: Microcytic (low MCV) hypochromic (low MHC) anaemia

Low serum ferritin

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17
Q

Iron deficiency anaemia management

A

Dietary advice
Oral supplementation
Malabsorption investigated if no improvement
Blood transfusion rarely needed

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18
Q

Dietary sources of iron

A 
Red meat, liver, kidney, oily fish
Pulses, beans, peas
Fortified breakfast cereals with added vitC
Wholemeal products
Dark green vegetables
Dried fruit
Nuts &amp; seeds

AVOID: cow’s milk, tea (tannin inhibits iron uptake), high fibre foods (phytates inhibit iron absorption)

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19
Q

Effects of folate/B12 deficiency

A

Macrocytic megaloblastic anaemia

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20
Q

Types of haemolytic anaemia

A

Intravascular haemolysis: increased red cell destruction in the circulation
Extravascular haemolysis: liver/spleen red cell destruction

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21
Q

Main causes of haemolysis

A

Neonates: immune haemolytic anaemias
Children: intrinsic abnormalities of red blood cells…
Red cell membrane disorders: e.g. hereditary spherocytosis
Red cell enzyme disorders: e.g. glucose-6-phosphate dehydrogenase deficiency
Haemoglobinopathies: e.g. β-thalassaemia major, sickle cell disease

22
Q

Haemolysis consequences

A 
Anaemia
Hepatomegaly
Splenomegaly
Increased blood levels of unconjugated bilirubin
Excess urinary urobilinogen
23
Q

Haemolysis diagnostic clues

A

Raised reticulocyte count (on the blood film this is called ‘polychromasia’ as the reticulocytes have a characteristic lilac colour)

Unconjugated bilirubinaemia and increased urinary urobilinogen

Abnormal appearance of the red cells on a blood film e.g. spherocytes, sickle shaped or very hypochromic

Positive direct antiglobulin test only if an immune cause, as this test identifies antibody-coated RBCs.

Increased RBC precursors in the bone marrow

24
Q

Hereditary spherocytosis pathophysiology

A

Mutations in genes for proteins of the red cell membrane: mainly spectrin, ankyrin or band 3

  • -> the red cell loses part of its membrane when it passes through the spleen
  • -> reduction in its surface-to-volume ratio cause the cells to become spheroidal
  • -> less deformable than normal
  • -> destruction in the microvasculature of the spleen
25
Q

Hereditary spherocytosis clinical features

A

Family Hx, may be asymptomatic
Jaundice: intermittent, may be haemolytic jaundice
Anaemia: Hb level may transiently fall during infections
Splenomegaly
Aplastic crisis: uncommon, transient (2-4weeks), caused by parovirus B19 infection
Gallstones: increased bilirubin excretion

26
Q

Hereditary spherocytosis investigations

A

Blood film: spherocytes
Osmotic fragility test
Dye binding test

Direct antibody test: exclude autoimmune haemolytic anaemia in the absence of a family history of hereditary spherocytosis

27
Q

Hereditary spherocytosis treatment

A

Oral folic acid: mild disease

Splenectomy: indicated for poor growth or troublesome symptoms, usually deferred until >7y/o because of post-op sepsis risk, patients vaccinated against H.influenzae, meningitis C and S.pneumonia prior to the op, lifelong daily oral penicillin prophylaxis

Aplastic crisis from parovirus B19 infection: 1-2 blood transfusions over 3-4 weeks when no RBC are produced

28
Q

Glucose-6-phosphate dehydrogenase deficiency pathophysiology

A

G6PD = rate-limiting enzyme in the pentose phosphate pathway. Essential for preventing oxidative
damage to red cells. RBCs lacking G6PD are susceptible to oxidant-induced haemolysis
Haemolysis is predominantly intravascular

29
Q

Glucose-6-phosphate dehydrogenase deficiency presentation

A 
Male: X-linked
High prevalence (10–20%): central Africa, Mediterranean, the Middle East and the Far East

Neonatal jaundice: first 3 days of life

Acute haemolysis precipitated by:
Infection: most common
Certain drugs
Fava beans: broad beans
Naphthalene in mothballs

Fever, malaise, dark urine (urobilinogen + haemoglobim)

30
Q

Glucose-6-phosphate dehydrogenase deficiency diagnosis

A

Measure G6PD activity in red blood cells

During a haemolytic crisis G6PD levels may be misleadingly elevated due to the higher enzyme concentration in reticulocytes (produced in increased numbers in response to the destruction of mature red cells) –> a repeat assay is required in the steady state to confirm the diagnosis

31
Q

What are haemoglobinopathies?

A

RBC disorders which cause haemolytic anaemia because of reduced/absent production of HbA (α- and β-thalassaemias), or because of the production of an abnormal Hb (sickle cell disease)
α-Thalassaemias –> deletions (occasionally mutations) in the α-globin gene
β-Thalassaemia and sickle cell disease are caused by mutations in the β-globin gene

32
Q

Main forms of sickle cell disease

A

Sickle cell anaemia (HbSS): homozygous for HbS, small amounts of HbF, no HbA

HbSC disease (HbSC): HbS from one parent and HbC from the other parent, no HbA because they have no normal β-globin genes (HbC is formed as a result
of a different point mutation in β-globin)

Sickle β-thalassaemia: HbS from one parent and β-thalassaemia trait from the other, no normal β-globin genes and most patients can make no HbA

Sickle trait: HbS from one parent and a normal β-globin gene from the other parent, 40% of the haemoglobin is HbS, carriers of HbS, so can transmit HbS to their offspring, asymptomatic

33
Q

Sickle cell pathophysiology

A

HbS: point mutation in codon 6 of the β-globin gene = glutamine –> valine
HbS polymerises within red blood cells forming rigid tubular spiral bodies which deform the red cells into a sickle shape
Reduced lifespan, may be trapped in the microcirculation –> vaso-occlusion –> ischaemia in an organ or bone
Exacerbated by low oxygen tension, dehydration, cold

34
Q

Clinical manifestations of sickle cell disease

A

Anaemia: moderate with jaundice from chronic haemolysis
Infection: Pneumococci/H. Influenzae & Salmonella osteomyolitis due to hyposplenism secondary to chronic sickling & microinfarction
Vaso-occlusive crises: hand-foot syndrome
Acute anaemia: haemolytic crises (infection), aplastic crises (parovirus), sequestration crises (sickled cells in spleen)
Priapism
Splenomegaly: common in young children
Long-term problems: short stature, delayed puberty, stroke, cognitive problems, adenotonsillar hypertrophy, cardiac enlargement + heart failure, renal dysfunction, pigment gallstones, leg ulcers

35
Q

Sickle cell prophylaxis

A

Fully immunised: pneumococcal, Haemophilus influenzae type B, meningococcus
Daily oral penicillin throughout childhood
o.d. p.o folic acid
Avoid: cold exposure, dehydration, excessive exercise/stress, hypoxia

36
Q

Sickle cell treatment

A

Acute crises: oral/i.v. analgesia & oral/i.v. hydration
Infection: antibiotics +/- oxygen if reduced sats
Acute chest syndrome, stroke, priapism = exchange transfusion

Chronic: recurrent admissions for painful vaso-occlusive crises/acute chest syndrome = hydroxyurea (increases HbF production + helps protect against further crises)
Side-effects: white blood cell suppression

Severe (stroke/no response to hydroxyurea): bone marrow transplant –> cure rate is 90%
but there is a 5% risk of fatal transplant-related complications

37
Q

Types of β-Thalassaemia

A

HBB gene mutation on chromosome 11 - autosomal recessive

β-Thalassaemia major: most severe, HbA (α2β2) cannot be produced because of the abnormal β-globin gene

β-Thalassaemia intermedia: milder/variable severity, β-
globin mutations allow a small amount of HbA and/or a large amount of HbF to be produced

β-Thalassaemia trait: heterozygotes are usually asymptomatic, red cells are hypochromic and microcytic + normal serum ferritin

38
Q

β-thalassaemia clincal features

A 
Severe anaemia: transfusion dependent, from 3–6 months of age
FTT
Extramedullary haematopoiesis: prevented by regular blood transfusions
Pallor
Jaundice
Bossing of the skull
Maxillary overgrowth
Splenomegaly
Hepatomegaly
39
Q

β-thalassaemia management

A

Lifelong monthly RBC transfusions: maintain haemoglobin concentration above 10 g/dl

Iron chelation + subcut. desferrioxamine OR oral iron chelator (from 2-3yo): transfusions cause chronic iron overload (cardiac failure, liver cirrhosis, diabetes, infertility, FTT)

Cure = bone marrow transplant

40
Q

Complications of multiple blood transfusions

A

Iron deposition: heart (cardiomyopathy), liver (cirrhosis), pancreas (diabetes), pituitary gland (delayed growth and sexual maturation), skin (hyperpigmentation)

Antibody formation (10%): allo-antibodies to transfused red cells in the patient make finding compatible blood difficult

Infection (<10%): hepatitis A, B, C, HIV, malaria, prions (e.g. new variant CJD)

Venous access: traumatic in young children, central venous access device (e.g. Portacath) may be required; these predispose to infection

41
Q

α-thalassaemia types

A

α-thalassaemia major: deletion of all four α-globin genes–> no HbA (α2β2) produced, presents in mid-
trimester with fetal hydrops (oedema and ascites) from fetal anaemia, fatal in utero/within hours of delivery

HbH disease (3 α-globin genes deleted): mild–moderate anaemia, occasional patients are transfusion-dependent

α-thalassaemia trait (1 or 2 α-globin genes deleted): usually asymptomatic, anaemia is mild/absent, red cells may be hypochromic and microcytic, which may cause confusion with iron deficiency

42
Q

Thrombocytopenia platelet levels

A

Severe thrombocytopenia (platelets <20 × 109/L): risk of spontaneous bleeding

Moderate thrombocytopenia (platelets 20–50 × 109/L): risk of excess bleeding during operations or trauma but low risk of spontaneous bleeding

Mild thrombocytopenia (platelets 50–150 × 109/L): low risk of bleeding unless there is a major
operation or severe trauma
43
Q

Causes of purpura/easy bruising

A

Increased platelet destruction/consumption: immune (ITP, SLE, alloimmune neonataly thrombocytopenia), non-immune (haemolytic uraemic syndrome, thrombotic thrombocytopenic purpura, DIC, congenital heart disease, giant haemangiomas, hypersplenism)

Impaired platelet production: congenital (Fanconi anaemia, Wiskott-Aldrich syndrome, Bernard-Soulier syndrome), acquired (aplastic anaemia, marrow infiltration (leukaemia), drug-induced)

Platelet dysfunction: congenital (rare disorders – eg. Glanzmann thromboasthenia), acquired (uraemia, cardiopulmonary bypass)

Vascular disorders: congenital (Ehlers-Danlos, Marfan syndrome), acquired (meningococcal, vasculitis (SLE, Henoch-Schonlein purpura), scurvy)

44
Q

Immune thrombocytopenia pathophysiology

A

Destruction of circulating platelets by anti-platelet IgG autoantibodies
Reduced platelet count may be accompanied by a compensatory increase of megakaryocytes in the bone marrow

45
Q

ITP presentation

A

2-10yo
1–2 weeks after a viral infection

Petechiae, purpura and/or superficial bruising
Epistaxis + other mucosal bleeding
Profuse bleeding is uncommon
Platelet count often falls to <10 × 10’9/L
Intracranial bleeding: rare

46
Q

Prompts for bone marrow examination

A

Atypical features: anaemia, neutropenia, hepatosplenomegaly or marked lymphadenopathy

If child is going to be treated with steroids (may mask ALL diagnosis)

47
Q

Haemophilia A grading

A

Mild: factor VIII:C = >5-40%, bleed after surgery
Moderate: factor VIII:C = 1-5%, bleed after minor trauma
Severe: factor VIII:C = <1%, spontaneous joint/muscle bleeds

48
Q

Haemophilia presentation

A

40% present neonatally: intracranial haemorrhage, bleeding post-circumcision, prolonged oozing from heel stick & venepuncture sites

Most present around 1yo when crawling/walking
Bleeding in joints & muscles –> arthritis if not treated
When no family history, NAI may be suspected

49
Q

Disseminated intravascular coagulation pathophysiology

A

Coagulation pathway activation –> diffuse fibrin deposition in the microvasculature + consumption of coagulation factors and platelets
Commonest causes of activation: severe sepsis/shock
Purpura fulminans may occur

50
Q

DIC presentation

A

Bruising, purpura, haemorrhage

Child Health (15 decks)

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