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Paediatrics > Haematology + Oncology > Flashcards

Haematology + Oncology Flashcards

1
Q

Difference between adult and fetal haemaglobin

A

Fetal Hb = 2 alpha + 2 gamma
Adult Hb = 2 alpha + 2 beta
Fetal HB has higher affinity for oxygen

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2
Q

What is the pattern of Hb during infancy + childhood?

A
  • High at birth (high fetal Hb) to compensate for low O2 concentration in fetus
  • Falls after birth (fetal Hb stopped producing)
  • Rises during childhood (adult Hb slowly compensates)
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3
Q

Why does Iron deficiency cause anaemia?

A

Iron is important in producing haemoglobin

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4
Q

Using reticulocyte count as a simplistic diagnostic approach of anaemia in children

A
  1. Low reticulocytes -> problem with red cell production:
    - Infection (parvo B19)
    - IDA, FDA, Chronic renal failure, bone marrow failure, pernicious anaemia
    - Anaemia of chronic disease
    - Thalassemia
  2. High reticulocytes -> normal red cell production ->
Bilirubin raised: Haemolytic anaemia
- hered. spherocytosis (membrane disorder)
- Sickle cell or Beta-thalass. 
(haemoglobinopathies)
- G6PD
 (enzyme disorder)
- Haemolytic disease of newborn, AI
(immune)

Bilirubin normal: Blood loss, burns

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5
Q

Using MCV as a diagnostic approach of anaemia

A

Microcytic: TAILS -> thallasemia (electrophoresis) , anaemia of chronic disease, Iron deficiency (ferritin), Lead poisoning, Sideroblastic
Normocytic: AAAHH -> anaemia of chronic, acute blood loss, aplastic, haemolytic, hypothyroidism
Macrocytic: ABCDEF -> alcohol/liver disease, B12, Compensatory reticulocytosis, Drugs, Endocrine = hypothyroidism, Folate

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6
Q

Generic presentation of anaemia?

A
  • pallor, fatigue, SOB, palpitations, headaches, tachycardia, conjunctival pallor
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7
Q

Specific presentations of anaemia?

A

IDA: brittle nails/hair, spoon shaped nails, atrophic glottitis, pica, angular chelitis
Haemolytic: Jaundice
Thalassemia: Bone deformities

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8
Q

Why is IDA so common in children

A
  • poor intake - picky, breast fed
  • increased requirement
  • If veg/vegan upbringing
  • 30% of iron supply for children is from diet (compared to 5% in adults)
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9
Q

What 4 blood tests are used to diagnose IDA

A
  • MCV: microcytic, hypochromic
  • Low ferritin
  • Low serum iron
  • High TIBC
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10
Q

Tx of IDA

A
  • Oral therapy: oral iron
  • address cause - usually diet
  • blood tranfusions rarely necessary
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11
Q

4 types of haemolytic anaema

A
  • Membrane problem -> hereditary spherocytosis
  • Haemoglobin problem -> thalassemia, sickle cell
  • Enzyme problem -> G6PD
  • Immune -> AI, haemolytic disease of newborn
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12
Q

What is normal haemoglobin and what happens to Hb in alpha + beta thalassaemia? What is pattern of inheritance?

A
  • 2 alpha + 2 beta globin chains
  • alpha = defects in alpha globin chain
  • beta = defects in beta
  • autosomal recessive
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13
Q

Types of Beta thalassaemia

A
  • Minor = carrier state and asymptomatic
  • Intermedia = mild homozygous mutation. Moderate anaemia but not requiring transfusions.
  • Major = significant abnormalities in both B globin chains. Severe anaemia, failure to thrive, life long transfusions.
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14
Q

Presentation of beta thalassaemia

A
  • Major: failure to thrive, splenomegaly (haemolysis + extramedullary haematopoiesis), skull bossing + bone deformities, severe microcytic anaemia
  • Intermedia: microcytic anaemia
  • minor: may be asymptomatic
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15
Q

Tx of beta thalassaemia major

A
  • life-long transfusions to prevent anaemia and suppress extramedullary haematopoiesis
  • iron chelators for iron overload
  • splenectomy if spleenomoegaly persists
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16
Q

Why get iron overload in beta thalassaemia and what is the result of iron overload

A
  • faulty creation of RBC, recurrent transfusions and increased absorption of iron in gut in response to anaemia
  • (similar to haemochromatosis):
    fatigue, liver cirrhosis, infertility, heart failure, arthritis
17
Q

Types of alpha thalassaemia

A
  • alpha thalassaemia caused by gene deletions
  • 4 alpha gene deletion = death in utero (barts)
  • 3 genes deleted = moderate anaemia, hepatospleenomegaly
  • 2 genes deleted = asymp. carrier
  • 1 = normal clinical state
18
Q

Dx of thalassaemia

A
  • FBC: microcytic anaemia

- Haemoglobin electrophoresis

19
Q

What is sickle cell anaemia

A
  • autosomal recessive genetic condition that causes sickle (crescent) shaped red blood cells
  • RBC more fragile and easily destroyed = occlusion of microcirculation and chronic haemolytic anaemia
20
Q

Presentation/complications of Sickle cell anaemia

A
  • Haemolytic anaemia: SOB, fatigue, delayed growth
  • SICKLE:
  • Stroke
  • Infections
  • Crises
  • Kidney disease (chronic)
  • Lung HTN
  • Erection persistence
21
Q

3 main Crises in sickle cell

A
    1. Vaso-occlusive (painful): sickle cell clog capillaries = distal ischaemia. Swollen, painful joints triggered by dehydration, cold, infection. CNS - stroke, seizures.
    1. Splenic sequestration crisis: RBC block blood flow within spleen. Blood pooling can cause severe anaemia and circulatory collapse.
    1. Aplastic crisis: Temporary loss of creation of new blood cells. Most commonly triggered by parvovirus B19 infection.
    1. Acute chest syndrome:
      Fever or respiratory symptoms with new infiltrates on chest XRAY. Can be infective (pneumonia or bronchiolitis) or non infective (vaso-occlusive).
22
Q

Dx of sickle cell

A
  • FBC, blood film -> anaemia + sickle cells
  • Hb electrophoresis confirms diagnosis
  • Neonatal screening by newborn screening heel prick test
23
Q

Mx of Sickle cell

A
  • penicillin prophylaxis (protect against infection)
  • Hydroxycarbamide stimulates HbF production. Does not lead to sickling (protects against crisis and ACS)
  • blood transfusion
  • bone marrow transplant can be curative
24
Q

What are the causes of haemophilia A and B

A
  • X-linked recessive inherited disorders (men only need one abnormal copy)
  • Haemophilia A = F8 deficiency
  • B = F9 deficiency
25
Q

Signs and symptoms of haemophilia

A
  • neonates: prolonged cord bleeding, intracranial hemorrhage (bulging fontanelle, lethargy, weak suck, cyanosis, apnoea, bradycardia), haematomas
  • bleeding into joints and muscles (tingling, tightness, warm)
  • gums, haematuria
26
Q

Mx of haemophilia

A
  • F8 or F9 replaced by IV infusions.
27
Q

What is the function of VWF

A
  • assists in platelet plug formation by attracting circulating platelets to site of damage
  • binds to F8 preventing its clearance
28
Q

Px of VWF disease

A
  • bleeding gums
  • nose bleeds (epistaxis)
  • HMB
  • blood clots during childbirth (rare)
29
Q

Mx of WVF disease

A
  • Desmopressin can be used to stimulates the release of VWF
  • VWF can be infused
  • Factor VIII is often infused along with plasma-derived VWF
30
Q

What is haemolytic disease of the newborn?

A
  • transplacental passage of maternal antibodies which causes immune haemolysis of fetal red blood cells
  • haemolytic anaemia and jaundice
31
Q

How does rhesus incompatibility cause haemolytic disease of the newborn

A
  • woman that is rhesus D negative has fetus that is rhesus D positive.
  • likely during pregnancy blood from fetus will cross placental barrier. Mothers immune system produces antibodies against rhesus D antigen = sensitised.
  • Subsequent pregnancy - mothers anti-D antibodies can cross placenta into fetus. Haemolytic anaemia and jaundice in neonate.
32
Q

How does ABO incompatibility cause haemolytic disease of the newborn

A
  • mother has type O blood and has a baby with type A,B or AB.
33
Q

Dx test for rhesus or ABO incompatilibilty

A
  • Combs test
34
Q

What is leukaemia

A

Cancer of stem cells in bone marrow which become red blood cells. Either myeloid or lymphoid lineage.

  • ALL most common in children (peaks 2-3 years)
  • AML next most common (peaks under 2 years)
35
Q

Px of leukaemia

A
  • excessive production of a single type of abnormal white blood cell: suppression of other cell lines = anaemia, leukopenia (WBC), thrombocytopenia (platelets) - unexplained bleeding
  • CNS involvement (ALL)
  • fever
  • failure to thrive
  • weight loss
  • Night sweats
  • Lymphadenopathy
  • bone or joint pain
  • hepatospleenomegaly
36
Q

Dx of leukaemia

A
  • FBC -> anaemia, leukopenia, thrombocytopenia
  • Blood film -> blast cells (ALL + AML), auer rods (AML)
  • bone marrow/lymph node biopsy
37
Q

Tx of leukaemia

A
  • chemotherapy primarily

- radiotherapy, bone marrow transplant

38
Q

What are the risk factors for leukaemia

A
  • radiation exposure

- Down’s syndrome predispose

Paediatrics (14 decks)

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