Oncology & Haematology - ITP/Solid Tumours/Other Flashcards
What is thrombocytopenia?
a platelet count <150 x10^9/L
How does thrombocytopenia present?
bruising
petechiae
purpura
mucosal bleeding - epitaxis, bleeding gums etc
rare complication - intracranial bleeding (usually if long period of time)
What is ITP?
immune thrombocytopenia
ITP is the most common cause of thrombocytopenia
caused by destruction of circulating platelets by antiplatelet IgG autoantibodies
usually presents after a viral infection
How is ITP diagnosed?
diagnosis of exclusion
any atypical features (anaemia, neutropenia, hepatosplenomegaly, lymphadenopathy) should prompt BM investigation to rule out ALL
SLE should also be considered
How is acute ITP managed?
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
How chronic ITP managed?
supportive treatment only if affects ADLs or QoL Rituximab can be given Splenectomy if drug therapy fails Regular SLE screening
What health advice should you give about ITP?
Avoid trauma as much as possible and no contact sports
What are the genetics of haemophilia?
commonest severe inherited coagulation disorder and consists of haemophilia A and B
X-linked recessive inheritance
two thirds have family history
What are the presenting features of haemophilia?
- 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
How is haemophilia managed?
- 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
How does Hodgkin’s lymphoma present?
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
How does non-Hodgkins present?
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
What are the SEs of chemotherapy?
- Hair loss
- Anaemia
- Infection
- Bruising
- Sore mouth
- N+V
- Mood changes
- Irritabilty
- Weight gain
What are the long term effects of chemotherapy?
- Delayed puberty
- Reduced fertility
- Reduced growth
- Neurotoxicity, hepatotoxicity, renal toxicity, cardiotoxicity, pulmonary toxicity, secondary cancer + psychological effects
What is a neuroblastoma?:
- 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
How does a neuroblastoma present?
- Pallor
- Weight loss
- Irregular firm abdominal mass, may cross midline
- Hepatomegaly
- Bone pain
- Limp
- Less common: paraplegia, cervical lymphadenopathy, proptosis, periorbital bruising, skin nodules
What investigations should be carried out when investigating a neuroblastoma?
- raised urinary catecholamine levels
- biopsy
- bone marrow sampling (metastatic disease)
- US
- IV pyelogram
How is a neuroblastoma treated?
surgically with adjunct chemotherapy and radiotherapy
immunotherapy now used in high risk disease
What is a Wilm’s tumour?
Wilm’s = nephroblastoma
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
How does a Wilm’s tumour present?
abdominal mass in an otherwise well child
haematuria
less common - abdo pain, anorexia, anaemia, HTN
What investigations are carried out for a suspected Wilm’s tumour?
USS/CT/MRI
urinalysis for catecholamines
BM examination
staging to assess for distant mets
How is Wilm’s tumour managed?
initial chemotherapy
delayed nephrectomy
subsequent treatment once tumour has been staged
What are the presenting features of soft tissue sarcomas?
rhabdomyosarcoma most commonly
presentation
- usually in head and neck - proptosis, nasal obstruction
- GU tumours- dysuria, bloody discharge etc
- metastatic disease
What are the presenting features of osteosarcoma/Ewing sarcoma?
- 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
What are the presenting features of retinoblastoma?
Retinoblastoma – malignant tumour of retinal cells
All bilateral tumours are hereditary, 20% unilateral
Clinical features
•White pupillary reflex replacing normal red one
•Squint
What is DIC?
Disseminated intravascular coagulation
disorder characterised by coagulation pathway activation leading to diffuse fibrin deposition in microvasculature and consumption of coagulation factors and platelets
What causes DIC?
Severe sepsis
Shock due to circulatory collapse
Extensive tissue damage from trauma or burns
What are the clotting abnormalities seen in DIC
- 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
What is the justification for having a splenectomy?
severe, non-responsive chronic ITP
trauma
spontaneous rupture
neoplasia
What are the risks and precautions associated with splenectomy?
risks - increased risk of infection, require lifelong abx prophylaxis
precautions - check that vaccinated against Hib, meningitis C and strep pneumoniae
