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Yr4: Child Health > Haematology/oncology > Flashcards

Haematology/oncology Flashcards

1
Q

Remind yourself of the structure of fetal Hb

Remind yourself of the structure of adult Hb

A
  • Fetal Hb= 2 alpha, 2 gamma
  • Adult Hb= 2 alpha, 2 beta
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2
Q

What is the main/key difference between fetal and adult Hb?

A

Fetal Hb has higher affinity for oxygen (important as fetal Hb needs to be able to ‘steal’ oxygen from mum’s Hb)

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3
Q

Discuss the transition from fetal to adult Hb

A
  • From 32 weeks of gestation, HbF production decreases and HbA is produced in greater quantities
  • At birth, around half of of Hb produced is HbF and half is HbA
  • All HbF is replaced by HbA at around 6-12 months of age (except in haemoglobinopathies e.g. some thalassaemias)
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4
Q

Discuss the transition from fetal to adult Hb

A
  • From 32 weeks of gestation, HbF production decreases and HbA is produced in greater quantities
  • At birth, around half of of Hb produced is HbF and half is HbA
  • All HbF is replaced by HbA at around 6-12 months of age (except in haemoglobinopathies e.g. some thalassaemias)
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5
Q

Most cases of anaemia in infancy are caused by physiological anaemia; what is physiological anaemia?

A
  • At birth, have higher Hb, higher haematocrit and larger RBCs than older children & adults
  • Once infant is breathing, there is an increase in oxygenation and hence increase in tissue oxygen level (compared to in utero)
  • Results in negative feedback on EPO levels and erythropoiesis
  • RBCs of neonate also have shorter life span (90 days)
  • Hence, both of the above cause Hb concentration to decrease over first few months of life (around 6-9 weeks)
  • It will then remain stable for a few weeks before beginning to rise due to EPO stimulation in the 4th-6th month

*If it occurs in premature babies we call it anaemia of prematurity. Same mechanism as above only their RBCs have even shorter lifespan and they have less EPO production

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6
Q

Other than physiological anaemia of infancy, state some potential causes of anaemia in infants

A
  • Anaemia of prematurity
  • Blood loss
  • Haemolysis
    • Haemolytic disease of newborn (ABO or rhesus incompatibility)
    • Hereditary spherocytosis
    • G6PD deficiency
  • Twin-twin transfusion (blood unequally distributed between twins that share placenta)
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7
Q

Why are premature neonates at higher risk of becoming anaemic?

A
  • Less time in utero receiving Fe from mother
  • Reduced EPO levels
  • RBC production cannot keep up with rapid growth over first few weeks
  • Blood tests remove a significant proportion of their circulating volume
  • *The more premature, the more likely they are to require one or more transfusions of anaemia. Becomes even more unlikley if unwell at birth e.g. neonatal sepsis*
  • *Same process occurs as in physiological anaemia of infancy but is just enhanced in premature babies due to the above*
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8
Q

Discuss the pathophysiology of haemolytic disease of the newborn

A
  • Lots of different types of rhesus antigens that may or may not be present on RBCs; most important is rhesus D antigen
  • Woman may be rhesus D negative but her baby may be rhesus D positive
  • Likely that at some point during pregnancy fetal blood will find a way into her blood stream
  • Mother will become sensitised to rhesus D antigen (her immune system will recognise it as foreign and produce antibodies)
  • Usually, sensitisation doesn’t cause problem in first pregnancy (unless happens early on e.g. in an antepartum haemorrhage)
  • During subsequent pregnancies, mother’s anti-D antibodies can cross placenta and if fetus is rhesus D positive then antibodies will attach themselves to fetal RBCs and cause the immune system of the fetus to attack it’s own RBCs
  • Leading to haemolysis causing anaemia and high bilirubin (jaundice)
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9
Q

What test can be done to check for haemolytic disease of the newborn?

A

Direct Coombs test (DCT) -used to check for immune haemolytic anaemia

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10
Q

State 2 common causes of anaemia in older children

State some other rarer causes of anaemia in older children

A

Common

  • Fe deficiency anaemia (most common)
  • Blood loss (most frequently in menstruating girls)

Rarer causes

  • Sickle cell anaemia
  • Thalassaemia
  • Hereditary spherocytosis
  • Hereditary eliptocytosis
  • Sideroblastic anaemia
  • Leukaemia
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11
Q

Anaemias can be categories based on RBC size; state 5 causes of microcytic anaemia

*Hint: TAILS

A
  • Thalassaemia
  • Anaemia of chronic disease
  • Iron deficieny anaemia
  • Lead poisoning
  • Sideroblastica anaemia (body has Fe available but cannot incorporate it into haemoglobin)
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12
Q

Anaemias can be categories based on RBC size; state 5 causes of normocytic anaemia

*HINT: 3A’s and 2H’s

A
  • Acute blood loss
  • Anaemia of chronic disease
  • Aplastic anaemia
  • Haemolytcia aneamia
  • Hypothyroidism
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13
Q

Anaemias can be categories based on RBC size; state 8 causes of macrocytic anaemia- differentiating into megaloblastic and normoblastic macrocytic anaemias

A

Megaloblastic

  • B12 deficiency
  • Folate deficiency

Normoblastic

  • Alcohol
  • Reticulocytosis (usually from haemolytic anaemia)
  • Hypothyroidism
  • Liver disease
  • Drugs e.g. azathioprine
  • Pregnancy & neonatal period
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14
Q

State some generic symptoms of anaemia

State 2 symptoms specific to Fe deficiency anaemia

A

Anaemia symptoms

  • Tiredness
  • SOB
  • Headaches
  • Dizziness
  • Palpitations
  • Worsening of other conditions

Fe Deficiency Anaemia

  • Pica
  • Hair loss
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15
Q

State some generic signs of anaemia

State some signs that indicate specific causes of anaemia

A

Generic signs

  • Pale skiin
  • Pale conjunctiva
  • Tachycardia
  • Raised RR

Specific signs anaemia

  • Fe deficiency: koilonychia (spoon nails), brittle hair & nails, angular chelitis, atrophic glossitis (smooth tongue due to papillae atrophy)
  • Haemolytic anaemia: jaundice
  • Bone deformities: thalassaemia
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16
Q

What investigations would you do if you suspect a pt has anaemia?

A
  • FBC: haemoglobin, MCV
  • Ferritin: Fe deficiency
  • TIBC
  • Serum Fe
  • B12 & folate
  • Bilirubin: raised in haemolysis
  • Reticulocyte count: check bone marrow response- if high usually indicates anaemia due to haemolysis or blood loss
  • Blood film: diagnose G6PD (Heinz bodies, blister cells)
  • Direct Coombs test: autoimmun haemolytic anaemia
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17
Q

State 3 reasons for Fe deficiency (not asking for exact causes/disease- just broad reasons but may give examples of diseases); highlight the most common in children

A
  • Dietary insufficiency
  • Loss of Fe (e.g. heavy menstruation)
  • Inadequate absorption (e.g. Crohn’s disease)
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18
Q

Explain why medications that reduce stomach acid (e.g. PPI’s) can lead to Fe deficiency

A

Fe mainly absorbed in duodenum or jejenum

Acid is required from stomach to keep Fe in solube ferrous (Fe2+) form

If there is less acidic/environment less acidic then it will change to insoluble ferric (Fe3+) form

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19
Q

It is important to understand tests for Fe deficiency; explain what is meant by:

  • TIBC
  • Transferrin
  • Transferrin saturation
  • Ferritin
A

*Transferrrin is the carrier protein for Fe

  • TIBC: space available on transferrin molecules for the Fe to bind
  • Transferrin saturation: proportion of transferrin molecules that are bound to Fe (serum Fe/TIBC)
  • Ferritin: form Fe takes when deposited and sorted (raised in inflammation)
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20
Q

Why is serum Fe on it’s own not a useful test?

A

Serum Fe varies significantly throughout day (higher in morning & after eating Fe containing meals)

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21
Q

Explain why a pt with Fe deficiency anaemia may have normal ferritin

A
  • Ferritin released when there is inflammation e.g. infection or cancer
  • High ferritin most likely related to inflammation rather than Fe overload
  • Pt with Fe deficiency anaemia may have normal ferritin if they have other reasons for raised ferritin e.g. infection
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22
Q

Would the following values be increased or decreased in Fe deficiency anaemia:

  • TIBC
  • Transferrin
A
  • TIBC: increased (less Fe in body so less bound to transferrin so more space for Fe to bind)
  • Transferrin: increased (body tries to compensate for low Fe by making more transferrin to increase carrying capacity)
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23
Q

Would the following values be increased or decreased in Fe overload:

  • TIBC
  • Transferrin
A
  • TIBC: decreased
  • Trasnferrin: decreased
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24
Q

Discuss the management of Fe deficiency anaemia

A
  • Treat underlying cause
    • Most common cause is dietary insufficiency hence give oral Fe supplementation (ferrous sulphate or fumarate) and dietician input (NOTE: if due to poor absorption may give IV supplementation)
  • Blood transfusion (rarely necessary as children able to tolerate a low Hb well)
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25
Q

For sickle cell anaemia, discuss:

  • What it is
  • Inheritance pattern
  • Mutation
A
  • Genetic condition that results in sickle (crescent) shaped RBCs
  • Autosomal recessive
  • Substitution of Glu to Val at the sixth amino acid position in the beta-chain hemoglobin; beta globin gene on chromosome 11.
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26
Q

Discuss the pathophysiology of sickle cell disease/why it is a problem

A
  • Polar glutamate replaced with to non-polar valine
  • This decreases the water solubility of deoxy-Hb
  • Leads to abnormal beta globin chains and abnormal haemoglobin (known as Haemoglobin S)
  • Abnormal beta globin chains are prone to polymerising when in the deoxygenated state
  • Leads to characteristic sickle shaped RBCs
  • Sickled RBCs are more fragile and prone to damage and also lead to further complications e.g.:
    • Vaso-occlusive crisis
    • Splenic sequestration crisis
    • Aplastic crisis
    • Acute chest syndrome
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27
Q

Sickle cell trait (where only inherit one mutated gene) results in milder form of sickle cell disease; true or false?

A

True

  • HbAS patients sickle at p02 2.5 - 4 kPa
  • HbSS patients at p02 5 - 6 kPa
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28
Q

Explain why sickle cell disease is more common in patients from areas traditionally affected by malaria (e.g. Africa, India, Middle East, Carribean)

A
  • Having one copy of gene (also known as sickle cell trait) reduces the severity of malaria
  • Consequently, pts with sickle cell trait more likely to survive in these areas and pass on their genes
  • “Selective advantage/natural selection”
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29
Q

When and who do we test for sickle cell disease?

A
  • Pregnant women at risk of having sickle cell gene are offered testing during pregnancy
  • Tested for during newborn screening heel prick test

*definitive diagnostic test is Haemoglobin electrophoresis

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30
Q

State some potential complications of sickle cell disease

A
  • Anaemia
  • Increased risk of infection
  • Avascular necrosis in large joints (e.g. hip)
  • Pulmonary hypertension
  • Priapism
  • CKD
  • Sickle cell crisis
    • Vaso-occlusive ‘painful’
    • Splenic sequestration
    • Aplastic
    • Acute chest syndrome
    • Haemolytic (rare)
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31
Q

Why does sickle cell disease lead to anaemia?

A

Sickle cells are fragile and more easily destroyed hence lifespan of sickled RBC is ~10-20 days

*NOTE: may also cause more severe anaemia in splenic sequestration crisis and aplastic crisis

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32
Q

State the 5 types of sickle cell crisis

A
  • Vaso-occlusive
  • Splenic sequestration
  • Aplastic
  • Acute chest syndrome
  • Haemolytic (rare)
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33
Q

For vaso-occlusive crisis, discuss:

  • What causes crisis
  • Precipitators (4)
  • Presentation
  • Management
  • Complications
A
  • Sickled RBCs have become stuck and are clogging capillaries causing distal ischaemia
  • Maybe precipitated by dehydration, infection, deoxygenation, cold weather (associated with raised hematocrit)
  • Presentation:
    • Pain (anywhere but usually in bones in arms, legs, spine, chest), fever, priapism, dactylitis, signs of precipitating factor
  • Diagnosis= clinical
  • Management:
    • Analgesia
    • Fluids
    • High flow oxygen
    • Abx if infection suspected
    • Priapism= urological emergency (aspiration of blood from penis)
  • May get infarcts in various organs including the bones (e.g. avascular necrosis of hip, hand-foot syndrome in children, lungs, spleen and brain)
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34
Q

For splenic sequestration crisis, discuss:

  • What causes crisis
  • Presentation
  • Complications
  • Management
A
  • Sickled RBCs blocking blood flow within the spleen causing an acutely enlarged painful spleen
  • Presentation: abdo pain, shock
  • Complications: severe anaemia, hypovolaemic shock, splenic infarction which results in increased susceptibility to infection
  • Management:
    • Emergency management: blood transfusions, fluid resuscitation
    • Splenectomy prevents sequestration crisis
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35
Q

For aplastic crisis, discuss:

  • What causes crisis
  • Most common trigger
  • Management
  • Complications
A
  • Temporary loss of production of new blood cells
  • Most common trigger: parvovirus B19 infection
  • Management: varies but may include blood transfusions, platelet transfusions, immunosupressants, hormones to stimulate bone marrow, stem cell transplant etc…
36
Q

For acute chest syndrome, discuss:

    • Causes
  • Presentation
  • What is required for diagnosis
  • Management
A
  • Causes can be due to infection (e.g. pneumonia or brochiolitis) or non-infective (vaso-occlusion or emobli)
  • Presentation: dyspnoea, chest pain, cough
  • Diagnosis requires:
    • Fever or respiratory symptoms with
    • New infiltrates seen on CXR
  • Medical emergency with high mortality:
    • Antibiotics or antivirals for infection
    • Blood transfusions for anaemia
    • Incentive spirometry
    • Artificial ventilation with NIV or intubation
37
Q

What is a haemolytic crisis? Is it common?

A

Fall in Hb due to haemolysis- rare.

38
Q

Discuss the general management of sickle cell disease

A
  • Avoid dehydration and other causes of triggers
  • Ensure vaccines up to date
  • Antibiotic prophylaxis (usually penicillin V)
  • Hydroxycarbamide to stimulate production of fetal Hb
  • Blood transfusion for severe anaemia
  • Bone marrow transplant (can be curative)
39
Q

Outline management of sickle cell crisis (not asking for management of specific crisis, but overall/general management of sickle cell crisis)

A
  • Oxygen
  • Analgesia
  • Fluids
  • Consider abx if infection
  • Blood transfusion
40
Q

Discuss the prognosis of sickle cell disease, include:

  • Life expectancy
  • Most common cause of death in children
  • Most common cause of death in adults
A
  • 58yrs
  • Commonest cause death children= infection or stroke
  • Commonest cause death adults= acute chest syndrome
41
Q

What is leukaemia?

Explain why it can lead to pancytopenia

A
  • Leukaemia is a progressive, malignant disease of the blood-forming organs, characterised by distorted proliferation and development of leukocytes and their precursors in the blood and bone marrow. It can be classified as acute or chronic, according to the degree of cell differentiation (not the duration of disease), and as myelogenous or lymphocytic, according to the predominant type of cell involved (myeloid or lymphoid).
  • Disruptions in the regulation and proliferation of lymphoid precursor cells in the bone marrow leads to excessive production of immature “blast” cells and a subsequent drop in numbers of functional red blood cells, white blood cells and platelets.
42
Q

State 3 types of leukaemia that affect children, order from most common to least common

A
  • Acute lymphoblastic leukaemia (ALL)
  • Acute myeloid leukaemia (AML)
  • Chronic myeloid leukaemia (CML)- rare

*Other rare leukaemia’s also exist

43
Q

State the peak ages of presentation for ALL and AML

A
  • Acute lymphoblastic leukaemia (ALL): 2-3yrs
  • Acute myeloid leukaemia (AML: <2yrs
44
Q

State some risk factors for developing leukaemia

A
  • Radiation exposure (e.g. AXR in pregnancy)
  • Conditions with increased risk:
    • Down’s syndrome
    • Noonan’s syndrome
    • Kleinfelter syndrome
    • Fanconi’s anaemia
45
Q

Describe presentation of leukaemia

A

Typically non-specific:

  • Persistent fatigue
  • Unexplained fever
  • Failure to thrive
  • Weight loss
  • Nights sweats
  • Pallor (anaemia)
  • Petechiae & abnormal bruising (thrombocytopenia)
  • Unexplained bleeding (thrombocytopenia)
  • Abdo pain
  • Generalised lymphadenopathy
  • Unexplained or persistent bone or joint pain (due to infiltrates)
  • Hepatosplenomegaly
46
Q

NICE recommend immediate referral to specialist for leukaemia if they have what 2 features?

If leukaemia is suspected based on the non-specific presentation, how soon do NICE recommend a FBC?

A
  • Unexplained petechiae or hepatomegaly
  • FBC within 48hrs
47
Q

What investigations may you do if you suspect leukaemia (think about diagnosis & staging)

A

For diagnosis…

  • FBC: anaemia, leukopenia, thrombocytopenia, high numbers abnormal WBC
  • Blood film: blast cells
  • Bone marrow biopsy
  • Lymph node biopsy

For staging….

  • CXR
  • CT scan
  • Lumbar puncture (check for CNS involvement)
  • Genetic analysis and immunophenotyping of abnormal cells
48
Q

Discuss the management of leukaemia

A

Treatment coordinated by paediatric oncologist and MDT:

  • Primarily treated with chemotherapy
  • Other therapies:
    • Radiotherapy
    • Bone marrow transplant
    • Surgery
  • Blood product transfusions
49
Q

State some potential complications of chemotherapy

A
  • Failure to treat leukaemia
  • Stunted growth & development
  • Immunodeficiency and infections
  • Neurotoxicity
  • Infertility
  • Secondary malignancy
  • Cardiotoxicity
50
Q

Discuss the prognosis of leukaemia

A
  • Overall cure rate is 80%
  • Prognosis depends on individual factors
  • Outcomes less positive for AML
51
Q

What is lymphoma?

What are different types of lymphoma?

A
  • Malignancy of lymphatic system
  • Types:
    • Hodgkin’s
    • Non-hodgkin’s
52
Q

Infection with what virus has been identified as risk factor for lymphoma?

A

Epstein Barr virus

53
Q

Describe clinical presentation of lymphoma

A
  • B symptoms
    • Weight loss (>10% in last 6 months)
    • Night sweats
    • Fevers
  • Lethargy
  • Anorexia
  • Non-tender lymphadenopathy
  • Mediastinal lymphadenopathy may present with cough, wheeze, difficulty breathing, SVC obstruction
54
Q

What investigations would you do for suspected lymphoma?

A
  • FBC
  • U&Es: important tumour lysis syndrome
  • Ultrasound: if suspect mediastinal node involvement
  • CXR: if suspect mediastinal node involvement
  • Full body CT: staging
  • Lymph node biopsy: for definitive diagnosis
55
Q

What scoring system is used to stage lymphoma?

A

Ann Arbor

  • Stage 1: single group lymph nodes or single organ
  • Stage 2: 2 or more groups of lymph nodes or organs on same side of diaphragm
  • Stage 3: disease in lymph nodes or organs on both sides of diaphragm
  • Stage 4: diffuse involvement of lymph nodes and other organs e.g. liver & bones

*Presence of B symptoms associated with worse prognosis and a B is added to any stage if they are present e.g. 1B, 2B etc..

56
Q

What is the long term management of lymphoma?

A
  • Chemotherapy with possible radiotherapy
  • All have life-long follow up to asses for late effects of cancer treatment
57
Q

Discuss the prognosis of lymphoma

A
  • Majority of children recover completely (Hodgkin’s > non-Hodgkin’s)
58
Q

Discuss the pathophysiology of G6PD deficiency

What is the inheritance pattern?

A
  • G6PD is enzyme in first step of pentose phosphate pathway and converts glucose-6-phosphate into 6-phosphogluconate (reaction also converts NADP into NADPH)
  • NADPH required to convert GSSG (oxidised glutathione) back to into GSH (reduced form)
  • GSH protects RBCs against oxidative damage
  • Hence in G6PD deficiency RBCs are at increased risk of damage due to oxidative stress

Inheritance pattern= x-linked recessive

59
Q

Discuss presentation of G6PD, including:

  • Ethnicities more common in
  • Triggers
  • Signs & symptoms
A
  • Mediterranean, middle eastern, african
  • Crises triggered by infections, medications (e.g. antimalarials, ciprofloxacin), broad beans
  • Signs & symptoms:
    • Often presents with neonatal jaundice
    • Other features: anaemia, intermittent jaundcie (in response to triggers), gallstones, splenomegaly
60
Q

What investigations would you do to diagnose G6PD deficiency?

A
  • G6PD enzyme assay (around 3/12 after acute episode as RBCs with most severely reduced G6PD activity will have been haemolysed during acute episode hence won’t be measured in assay if do too early)
  • Blood film: Heinz bodies, bite & blister cells may be seen

May do others e.g. FBC for anaemia, LFTs for gallstones, bilirubin etc…

61
Q

Discuss the management of G6PD deficiency

A
  • Avoid triggers (medications, broad beans, minimise infections)
  • *Medications to avoid: primaquine, ciprofloxacin, nitrofurantoin, sulfonylureas, sulfasalazine & other sulphonamide drugs*
62
Q

For hereditary spherocytosis, discuss:

  • Inheritance pattern
  • Which ethnicities common in
  • Pathophysiology
A
  • Autosomal dominant
  • Norther Europeans
  • RBCs are sphere shaped so fragile & easily destroyed when passing through spleen (extravascular haemolysis)
63
Q

Discuss typical presentation of hereditary speherocytosis

A
  • Neonatal jaundice
  • Failure to thrive
  • Gallstones
  • Splenomegaly
  • Haemolytic crisis (anaemia & jaundice)
  • Aplastic crisis triggered by parvovirus infection
64
Q

Explain what happens during an aplastic crisis in pts with hereditary spherocytosis; include trigger

A
  • Often triggered by parvovirus infection
  • Usually when haemolysis occurs leading to anaemia & jaundice the bone marrow responds by creating new RBCs
  • In aplastic crisis there is no reticulocyte response
65
Q

What investigations would you do to diagnose hereditary spherocytosis?

A
  • Blood film: spherocytes
  • MCHC: raised
  • Reticulocytes: raised (or low in aplastic crisis)

*If above results are not conclusive can do EMA binding test

66
Q

Discuss the management of hereditary spherocytosis (think about acute & long term management)

A

Acute:

  • Supportive treatment
  • Transfusion if necessary

Long term:

  • Folate replacement
  • Splenectomy
  • Cholecystectomy (gallstones)
67
Q

Compare G6PD and hereditary spherocytosis in terms of:

  • Inheritance pattern
  • Ethnicities affected
  • Haemolysis
A
68
Q

Hereditary elliptocytosis very similar to hereditary spherocytosis except RBCs are elliptical. Autosomal dominant also. Management similar.

A
69
Q

Remind yourself of pathophysiology of thalassaemia

A
  • Autosomal recessive genetic defect resulting in reduced production rate of either alpha or beta globin chains (alpha thalassaemia: alpha chains reduced, beta thalassaemia: beta chains reduced)
  • RBCs more fragile and hence break down more easily; spleen removes all damaged RBCs
  • Bone marrow expands to produce more RBCs in response to chronic anaemia resulting in:
    • Prominent bony features (e.g. pronounced forehead & cheek bones)
    • Susceptibility to fractures
70
Q

State some clinical features of thalassaemia (not asking to distinguish between different types)

A
  • Microcytic anaemia (low mean corpuscular volume)
  • Fatigue
  • Pallor
  • Jaundice
  • Gallstones
  • Splenomegaly
  • Poor growth and development
  • Pronounced forehead and malar eminences
71
Q

For alpha thalassaemia, discuss:

  • Four different types
  • Presentation of each
  • Managment
    *
A
  • Monitoring FBC
  • Blood transfusions if required
    • May need Fe chelation if multiple transfusions required
  • Bone marrow transplant (can be curative)
  • Splenectomy
72
Q

For beta thalassemia, discuss:

  • 3 different types
  • Presentations of each
  • Management of each
A
73
Q

For idiopathic thrombocytopenic purpura (ITP), discuss:

  • What it tis
  • Type of hypersensitivity reaction
  • Pathophysiology
A
  • Idiopathic thrombocytopenia causing a purpuric (non-blanching) rash (can be spontaneous or triggered by e.g. viral infection)
  • Type II hypersensitivity
  • Antibodies, against glycoprotein IIb or IIIa complex, destroying platelets
74
Q

Describe typical presentation of idiopathic thrombocytopenic purpura

A
  • Bleeding (e.g. gums, epistaxis, menorrhagia)
  • Bruising
  • Petechial or purpuric rash

*remember, petechia are pin-prick spots (1mm), purpura are larger (3-10mm) spots, larger areas (>10mm) called ecchymoses. All due to bleeding under skin & are non-blanching.

75
Q

What investigation is done to confirm ITP?

A
  • Urgent FBC for platelet count
76
Q

Discuss the management of ITP

A

Severity & hence management depends on platelet count; usually no treatment required and pts are just monitored until platelets return to normal (around 70% remit spontaneously in first 3 months).

If actively bleeding or severe thrombocytopenia (e.g. <10):

  • Prednisolone
  • IV immunoglobulins
  • Tranexamic acid
  • Blood transfusions
  • Platelet transfusions (only temporary as antibodies will destroy transfused platelets)

Education for all:

  • Avoid contact sports
  • Avoid IM injections & procedures (e.g. LP)
  • Avoid NSAIDs, aspirin, blood thinners
  • Advice on managing nose bleeds
  • Seek help after injury (in case of internal bleeding)
77
Q

State some potential complications of ITP

A
  • Chronic ITP
  • Anaemia
  • Intracranial haemorrhage
  • Subarachnoid haemorrhage
  • GI bleeding
78
Q

State some examples of conditions associated with hyposplenism

A
79
Q

State some potential causes of DIC

A
  • Sepsis
  • Trauma (including major burns)
  • Malignancy
  • Obstetric complications (e.g. eclampsia, placental abruption) less relevant paeds)
  • Severe immune related reactions (e.g. transfusion reaction)
  • Severe organ failure (e.g. severe acute pancreatitis)
80
Q

Explain the pathophysiology of DIC

A
  • Under normal homeostatic conditions, coagulation & fibrinolysis are coupled and there is a balance between coagulation & fibrinolysis
  • In DIC, processes of coagulation and fibrinolysis are dysregulated resulting in widespread clotting and resultant bleeding as all clotting factors are being used up
  • Critical mediator of DIC is tissue factor (TF)
  • TF is not normally exposed to general circulation however can come into contact with general circulation due to vascular damage (e.g. sepsis), trauma, obstetric complications, malignancy. Abundant in tissues such as lung, brain & placenta (explaining why it can develop in pts with trauma)
  • TF binds to coagulation factors then triggers extrinsic pathway (via factor VII) which subsequently triggers intrinsic pathway (XII to XI to IX)
81
Q

What would you see on blood results for DIC?

A
  • Decreased platelets
  • Decreased fibrinogen
  • Increased PT and APTT
  • Increased bleeding time
  • Increased fibrinogen degradation products
  • Schistocytes due to microangiopathic haemolytic anaemia
82
Q

Describe clinical features of DIC

A
  • Evidence/history of precipitating factor
  • Bleeding (e.g. nose, GI, sites of venepuncture/cannulation, ear, resp)
  • New confusion
  • Petechiae/purpura
  • Widespread bruising without history of trauma
  • Livedo reticularis (mottled lace-like patterning of the skin)
  • Purpura fulminans (widespread skin necrosis)
  • Localised infarction and gangrene e.g. fingers/toes
  • Signs of circulatory collapse (e.g. hypotension, tachycardia)
83
Q

What investigations would you do for DIC and what results would you expect?

A
  • FBC: thrombocytopenia
  • Coagulation screen: prolonged PT (extrinsic & common pathway) & APTT (intrinsic & common pathway)
  • Clauss fibrinogen: decreased
  • Fibrin degradation product/D-dimer: raised
84
Q

Discuss the management of DIC

A
  • Treat underlying disorder
  • Supportive treatment to restore coagulation. Complicated but may consider:
    • Platelets
    • FFP
    • Clotting factors
    • Cryoprecipitate
    • If thrombosis prominent, therapeutic heparin
    • If not bleeding, prophylactic heparin to protect against VTE

*More detail in cancer care block?

85
Q

State some potential complications of DIC

A
  • Multi-organ failure
  • Life-threatening haemorrhage
  • Intracranial haemorrhage
  • Gangrene & loss of digits
  • Cardiac tamponade
  • Haemothorax

Yr4: Child Health (21 decks)

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