Haem + onc Flashcards

1
Q

what is the cause of physiological anaemia?

A
  • around 6-9 weeks in healthy term babies, caused by negative feedback prompted by high oxygen delivery seen at birth
    • EPO by kidneys suppressed hence Hb reduced by BM
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2
Q

what are some other causes of anaemia in children?

A

infants: anaemia of prematurity, blood loss, haemolysis, twin to twin transfusion

neonates - hereditary spherocytosis, G6PD deficiency

older - iron deficiency, blood loss, sickle cell, thalassaemia, leukaemia, helminth infection

low RBC production or high RBC loss

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3
Q

what is anaemia of prematurity?

A
  • Less time in utero receiving iron from the mother
  • Red blood cell creation cannot keep up with the rapid growth in the first few weeks
  • Reduced erythropoietin levels
  • Blood testsremove a significant portion of their circulating volume
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4
Q

what is haemolytic disease of the new born?

A
  • incompatibility between rhesus antigens on surface of red blood cells of mother and foetus
  • woman rhesus D negative becomes pregnant consider the possibility of foetus becoming rhesus D positive as mothers immune system will recognise this as foreign and and produce antibodies for this
  • not a problem during first pregnancy usually, but in second mothers anti-D antibodies will cross placenta and if foetus positive antibodies will cause immune system of foetus to attack its own blood cells by binding to RBC of foetus
  • causes anaemia and high bilirubin
  • DCT done to assess
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5
Q

what are some key examination findings of anaemia?

A

generic: pale skin, conjunctival pallor, tachycardia, raised RR

koilonychia, angular chelitis, atrophic glossitis, brittle hair, jaundice, bone deformities

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6
Q

what are some initial investigations for anaemia?

A
  • 1st line - FBC, reticulocytes, film, ferritin, CRP/ ESR
  • Full blood count forhaemoglobinandMCV
  • Blood film
  • Reticulocyte count
  • Ferritin (low iron deficiency)
  • B12 and folate
  • Bilirubin (raised in haemolysis)
  • Direct Coombs test (autoimmune haemolytic anaemia)
  • Haemoglobin electrophoresis (haemoglobinopathies)
  • reticulocytes
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7
Q

how is anaemia managed?

A
  • Management depends on establishing the underlying cause and directing treatment accordingly
  • Iron deficiency can be treated with iron supplementation
  • Severe anaemia may require blood transfusions
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8
Q

what is the pathophysiology of DIC?

A
  • new-borns (particularly pre-terms) have low levels of coagulation factors and hence vulnerable to DIC
  • causes - sepsis, NEC, HIE, RDS
  • the above causes activates the coagulation cascade, promotes fibrin production and deposition and consumption of clotting factors
  • consumption of coagulation factors and platelets, inhibition of natural anticoagulants and fibrinolysis, and fibrin deposition result in the clinical picture of DIC
    • a bleeding diathesis accompanied by microvascular thrombosis that often leads to end-organ damage
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9
Q

how does DIC present?

A
  • Bleeding, from many sites in the body
  • Blood clots
  • Bruising
  • Drop in blood pressure
  • Shortness of breath
  • Confusion, memory loss or change of behavior
  • Fever

*petechiae
- bleeding (cutaneous, mucosal, umbilical, GI)

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10
Q

what complications are associated with DIC?

A

birth asphyxia, acidosis, respiratory distress syndrome, infection, necrotising enterocolitis, meconium aspiration, aspiration of amniotic fluid, brain injury, hypothermia

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11
Q

how is DIC investigated?

A
  • platelets low
  • schistocytes (fragmented RBC)
  • increased INR
  • increased APTT
  • fibrinogen low
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12
Q

how is DIC managed?

A
  • get help
  • treat cause - NEC, sepsis
  • give vitamin K 1mg IV & blood products - platelets, cryoprecipitate etc
  • severe cases consider exchange transfusion
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13
Q

what is the function of the spleen?

A
  • spleen is important in reticuloendothelial system for antibody synthesis and clearance of opsonised organisms by phagocytosis
  • Antibody-mediated phagocytosis is the primary mechanism to destroy encapsulated microbes, such as pneumococcus, meningococcus, andHaemophilus
  • absence of spleen or reduced activity means the polysaccharide rich capsules of these bacteria protect them from destruction and permit them to create systemic infection
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14
Q

when would you suspect hyposplenia?

A

hyposplenia should be suspected in any patient with overwhelming infection with an encapsulated organism

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15
Q

How is impaired splenic function investigated?

A
  • blood smear - Howell-jolly bodies, Heinz bodies, target cells
  • USS with doppler to assess spleen size
  • CT/ MRI to detect spleen shape
  • radionucleotide (technetium-99m) liver and spleen scan to detect functional reticuloendothelial cells
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16
Q

how is impaired splenic function managed?

A
  • immunisation with pneumococcal, meningococcal, haemophilus vaccines
  • patients with sickle cell and impaired spleen function - antimicrobial prophylaxis
  • counselling regarding infections
  • MedicAlert bracelets
17
Q

what is leukaemia?

A
  • leukaemia is the cancer of stem cells in the bone marrow which leads to unregulated production of certain types of blood cells
    • genetic precursor in BM leads to excessive WBC production which suppresses other cell lines causing under production of others
    • pancytopenia as a result - anaemia, leukopenia, thrombocytopenia
18
Q

how might leukaemia present?

A
  • non specific
  • persistent fatigue
  • unexplained fever
  • failure to thrive
  • weight loss
  • night sweats
  • unexplained bleeding
  • abdominal pain
  • unexplained bone or joint pain
  • pallor
  • petechiae and abnormal bruising - thrombocytopenia
  • generalised lymphadenopathy
  • hepatosplenomegaly
19
Q

what are some complications of chemo?

A
  • Failure to treat the leukaemia
  • Stunted growth and development
  • Immunodeficiency and infections
  • Neurotoxicity
  • Infertility
  • Secondary malignancy
  • Cardiotoxicity
20
Q

how is leukaemia investigated?

A

*refer any child with unexplained petechiae, hepatomegaly, if leukaemia suspected FBC within 48h

  • Full blood count, which can showanaemia,leukopenia, thrombocytopeniaand high numbers of the abnormal WBCs
  • Blood film, which can showblast cells
  • Bone marrow biopsy
  • Lymph node biopsy
  • for staging - CXR, CT, LP, genetic analysis and immunophenotyping
21
Q

how is leukaemia managed?

A
  • Treatment of leukaemia will be coordinated by a paediatric oncology multi-disciplinary team
  • Leukaemia is primarily treated withchemotherapy
  • Other therapies:
    • Radiotherapy
    • Bone marrow transplant
    • Surgery
  • The overall cure rate for ALL is around 80%, but prognosis depends on individual factors
  • The outcomes are less positive for AML
22
Q

what is lymphoma?

A
  • cancer that starts in the lymphatic system divided into Hodgkin’s and non-Hodgkin’s (more)
  • accounts for over 10% of childhood cancers and more common in boys and older children
  • multi-factorial causes like infection, genetic and environmental
  • RF - Epstein-Barr virus, immunosuppressed, other cancer treated
23
Q

when might you suspect lymphoma?

A
  • weight loss, night sweats, fevers
  • visible or palpable mass
  • lethargy
  • anorexia
  • mediastinal lymphadenopathy - wheeze and cough, SOB
    • SVC obstruction and airway compromise!
  • non-tender lymphadenopathy
24
Q

how is lymphoma investigated?

A
  • FBC - differentiate from infection
  • U&E - tumour lysis syndrome
  • LDH - elevated
  • USS - identify other nodes and assists biopsy
  • CXR - mediastinal involvement
  • CT full body - extent of disease and staging
  • lymph node biopsy - definitive diagnosis
25
Q

What is tumour lysis syndrome?

A

tumours release their contents into the bloodstream, either spontaneously or in response to therapy, leading to the characteristic findings of hyperuricaemia, hyperkalaemia, hyperphosphataemia, and hypocalcaemia

26
Q

how is lymphoma managed?

A

immediate
- mediastinal mass high dose steroids
- SVC obstruction
- tumour lysis syndrome hyper-hydration and allopurinol

long term
- chemo
- radiotherapy

27
Q

what may cause pancytopenia in children?

A
  • acute leukaemia’s, bone marrow failure syndromes, lupus, infections, medicine side effects, radiation, chemotherapy, autoimmune disorders, FHx of blood disorders can cause
    • OR IDIOPATHIC
28
Q

how does pancytopenia present?

A
  • weakness
  • fatigue
  • easy bruising
  • pale skin
  • rapid heart rate
  • SOB
  • bleeding problems like bleeding gums
  • infections
  • heavy menstruation
  • bruises
  • tachycardia
  • pallor
29
Q

how is pancytopenia managed?

A
  • treat underlying cause
  • Drugs that suppress the immune system - autoimmune
  • Drugs that stimulate bone marrow - EPO, granulocyte colony-stimulating factor (GCSF)
  • Bone marrow transplant
  • Blood transfusions
  • Allogenic stem cell transplant
  • Watchful monitoring - mild
30
Q

what is sickle cell disease?

A
  • inherited haemoglobinopathy caused by mutation in the gene encoding for the beta globin gene
    • autosomal recessive affecting beta-globin gene on chromosome 11
  • HbS allele results from single nucleic acid substitution from GAG to GTG in beta globin gene
  • this affects how haemoglobin polymerises and forms crystals when deoxygenated - sickling of red cells leading to vaso-occlusive crises in sufferers
31
Q

how does sickle cell predispose someone to infection?

A

splenic dysfunction, reduced opsonisation, adaptive immune dysfunction, neutrophil dysfunction, increased osteomyelitis risk

32
Q

what are some sickle cell crisis types?

A
  • acute exacerbation
  • haemolytic
  • vaso-occlusive
  • sequestration
  • aplastic
  • acute chest
33
Q

how might vase-occlusive crises present?

A
  • acute pain from vaso-occlusion crises in back, legs, knees, arms, chest and abdomen
    • hypoxia
    • infection
    • strenuous exercise
    • dehydration
    • acidosis
  • Dactylitis - painful inflammation of a digit (finger or toe) which may be first presentation in a child
34
Q

what complications might arise in someone with sickle cell?

A
  • chronic spleen damage by auto-infarction
  • pulmonary htn, leg ulcers, priapism, CKD, leg artery ischaemic stroke
  • short stature, aplastic crisis
  • life expectancy of 58 years
35
Q

what is the most common cause of death associated with sickle cell?

A

infection, sepsis, meningitis, pneumonia

36
Q

how might sickle cell be investigated?

A
  • hb elecrophoresis
  • high performance liquid chromatography
  • blood film
  • FBC, reticulocytes
  • iron studies
  • imaging - XR
  • antenatal screening
  • new born blood spot test
37
Q

when would you consider admission for someone with sickle cell?

A
  • Persistent temperature
  • Pallor, lethargy, malaise, abdominal distension.
    Severe pain requiring opiate analgesia
  • Symptoms and signs suggesting:
  • Acute chest syndrome
  • Acute cerebrovascular accident
  • Sequestration syndrome
  • Aplastic crisis
  • Fulminant priapism
38
Q

how would you manage an acute pain crisis in sickle cell?

A
  • analgesia - assess pain regularly!! paracetamol, NSAIDs and stronger opioids for severe
  • fluid hydration
  • oxygen
  • antibiotics - consider if any infection, follow local protocol
  • red cell transfusion urgent
39
Q

how would you manage sickle cell?

A
  • prophylaxis: hydroxycarbamide, regular transfusions
  • education: recognise crises, avoid alcohol and strenuous exercise

general
- avoid triggers like dehydration
- up-to-date vaccinations
- antibiotic prophylaxis
- hydroxycarbamide
- crizanlizumab
- prevents RBC sticking to vessel wall and reduces crises frequency
- BM transplant
- blood transfusions