Oncology & Haematology - ITP/Solid Tumours/Other Flashcards

1
Q

What is thrombocytopenia?

A

a platelet count <150 x10^9/L

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2
Q

How does thrombocytopenia present?

A

bruising
petechiae
purpura
mucosal bleeding - epitaxis, bleeding gums etc
rare complication - intracranial bleeding (usually if long period of time)

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3
Q

What is ITP?

immune thrombocytopenia

A

ITP is the most common cause of thrombocytopenia
caused by destruction of circulating platelets by antiplatelet IgG autoantibodies
usually presents after a viral infection

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4
Q

How is ITP diagnosed?

A

diagnosis of exclusion
any atypical features (anaemia, neutropenia, hepatosplenomegaly, lymphadenopathy) should prompt BM investigation to rule out ALL
SLE should also be considered

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5
Q

How is acute ITP managed?

A

in 80%, disease benign & self limiting - resolves in 6-8 weeks ad can be treated at home
if major or persistent bleeding that affects daily life –> oral pred, IV anti-D or IV immunoglobulin
life threatening haemorrhage - platelet transfusions

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6
Q

How chronic ITP managed?

A
supportive treatment 
only if affects ADLs or QoL
Rituximab can be given 
Splenectomy if drug therapy fails
Regular SLE screening
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7
Q

What health advice should you give about ITP?

A

Avoid trauma as much as possible and no contact sports

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8
Q

What are the genetics of haemophilia?

A

commonest severe inherited coagulation disorder and consists of haemophilia A and B
X-linked recessive inheritance
two thirds have family history

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9
Q

What are the presenting features of haemophilia?

A
  • severe, moderate or mild
  • severe = recurrent spontaneous bleeding into joints and muscles - can lead to crippling arthritis if not treated properly
  • most present toward end of first year of life
  • NAI may be suspected if FHx
  • 40% present in neonatal period w/ intracranial haemorrhage, prolonged oozing from heel prick
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10
Q

How is haemophilia managed?

A
  • recombinant FVIII concentrate for haemophilia A or recombinant FIX concentrate for haemophilia B
  • prompt IV infusion whenever bleeding
  • home treatment encouraged to avoid delay in treatment
  • if severe prophylactic FVIII or FIX
  • Desmopressin can manage mild haemophilia A
  • MDT management and support groups available
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11
Q

How does Hodgkin’s lymphoma present?

A

peripheral lymph nodes
seen more frequently in adolescence
- painless lymphadenopathy, most frequently in the neck
- clinical history often long
- sweating, pruritus, weight loss and fever uncommon

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12
Q

How does non-Hodgkins present?

A

more common in childhood
can also occur in other organs - liver, spleen, bone marrow, CNS, bones

  • mediastinal mass with varying degrees of bone marrow infiltration (SVC obstruction can occur)
  • localised lymph node disease usually in head, neck and abdomen
  • abdominal disease presents with pain from intestinal obstruction
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13
Q

What are the SEs of chemotherapy?

A
  • Hair loss
  • Anaemia
  • Infection
  • Bruising
  • Sore mouth
  • N+V
  • Mood changes
  • Irritabilty
  • Weight gain
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14
Q

What are the long term effects of chemotherapy?

A
  • Delayed puberty
  • Reduced fertility
  • Reduced growth
  • Neurotoxicity, hepatotoxicity, renal toxicity, cardiotoxicity, pulmonary toxicity, secondary cancer + psychological effects
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15
Q

What is a neuroblastoma?:

A
  • arises from neural crest tissue in adrenal medulla and sympathetic nervous system
  • biologically unsual as can spontaneously regress
  • spectrum from benign to highly malignant
  • most common before 5 years old
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16
Q

How does a neuroblastoma present?

A
  • Pallor
  • Weight loss
  • Irregular firm abdominal mass, may cross midline
  • Hepatomegaly
  • Bone pain
  • Limp
  • Less common: paraplegia, cervical lymphadenopathy, proptosis, periorbital bruising, skin nodules
17
Q

What investigations should be carried out when investigating a neuroblastoma?

A
  • raised urinary catecholamine levels
  • biopsy
  • bone marrow sampling (metastatic disease)
  • US
  • IV pyelogram
18
Q

How is a neuroblastoma treated?

A

surgically with adjunct chemotherapy and radiotherapy

immunotherapy now used in high risk disease

19
Q

What is a Wilm’s tumour?

Wilm’s = nephroblastoma

A

originates from embryonal renal tissue
most common renal tumour of childhood
presents before 5 years, rarely seen after 10 years
usually unilateral, tends to be encapsulated and vascularised

20
Q

How does a Wilm’s tumour present?

A

abdominal mass in an otherwise well child
haematuria
less common - abdo pain, anorexia, anaemia, HTN

21
Q

What investigations are carried out for a suspected Wilm’s tumour?

A

USS/CT/MRI
urinalysis for catecholamines
BM examination
staging to assess for distant mets

22
Q

How is Wilm’s tumour managed?

A

initial chemotherapy
delayed nephrectomy
subsequent treatment once tumour has been staged

23
Q

What are the presenting features of soft tissue sarcomas?

A

rhabdomyosarcoma most commonly
presentation
- usually in head and neck - proptosis, nasal obstruction
- GU tumours- dysuria, bloody discharge etc
- metastatic disease

24
Q

What are the presenting features of osteosarcoma/Ewing sarcoma?

A
  • Male predominance
  • Osteosarcoma more common than Ewing sarcoma
  • Ewing sarcoma more common in younger children
  • Limbs most common site, persistent localised bone pain (usually otherwise well)
  • Ewing sarcoma usually substantial soft tissue mass
25
Q

What are the presenting features of retinoblastoma?

A

Retinoblastoma – malignant tumour of retinal cells
All bilateral tumours are hereditary, 20% unilateral

Clinical features
•White pupillary reflex replacing normal red one
•Squint

26
Q

What is DIC?

Disseminated intravascular coagulation

A

disorder characterised by coagulation pathway activation leading to diffuse fibrin deposition in microvasculature and consumption of coagulation factors and platelets

27
Q

What causes DIC?

A

Severe sepsis
Shock due to circulatory collapse
Extensive tissue damage from trauma or burns

28
Q

What are the clotting abnormalities seen in DIC

A
  • Thrombocytopaenia
  • Prolonged PT
  • Prolonged APTT
  • Low fibrinogen
  • Raised fibrinogen degradation products
  • D-dimers
  • Microangiopathic haemolytic anaemia
  • Reduction in naturally occurring anticoagulants, proteins C+S, antithrombin
29
Q

What is the justification for having a splenectomy?

A

severe, non-responsive chronic ITP
trauma
spontaneous rupture
neoplasia

30
Q

What are the risks and precautions associated with splenectomy?

A

risks - increased risk of infection, require lifelong abx prophylaxis
precautions - check that vaccinated against Hib, meningitis C and strep pneumoniae