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Child Health > Haematology And Oncology > Flashcards

Haematology And Oncology Flashcards

1
Q

Causes of anaemia in infancy

A 
Physiologic anaemia of infancy
Anaemia of prematurity
Blood loss
Haemolysis 
Twin-twin transfusion
Haemolytic disease of the newborn 
Hereditary spherocytosis 
G6PD deficiency
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2
Q

Physiologic anaemia of infancy

A

Normal dip in Hb at 6-9weeks
Due to high oxygen delivery, decreased production Hb
EPO production decreases in kidneys
Less bone marrow stimulation

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3
Q

Why are premature neonates more likely to become anaemic?

A

Less time in utero receiving iron from mother
RBC creation can’t keep up with rapid growth in first few weeks
Reduced EPO levels
Blood tests remove a significant portion of the circulating volume

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4
Q

Causes of anaemia in older children

A 
Iron deficiency anaemia, diet
Blood loss: menstruation in older girls 
Sickle cell anaemia
Thalassaemia
Leukaemia
Hereditary spherocytosis
Hereditary elliptocytosis
Sideroblastic anaemia

Helminth infection:
Roundworm, hookworm, whipworms
Albendazole or mebendazole

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5
Q

Causes of microcytic anaemia

TAILS

A 
Thalassaemia
Anaemia of chronic disease
Iron deficiency anaemia
Lead poisoning
Sideroblastic anaemia
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6
Q

Causes of normocytic anaemia
3As
2Hs

A 
Acute blood loss
Anaemia of chronic disease
Aplastic anaemia
Haemolytic anaemia
Hypothyroidism
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7
Q

Causes of macrocytic anaemia

Megaloblastic

A

Megaloblastic anaemia: impaired DNA synthesis prevents cell from dividing normally

B12 deficiency
Folate deficency

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8
Q

Normoblastic macrocytic anaemia

A 
Alcohol
Reticulocytosis (haemolytic anaemia or blood loss)
Hypothyroidism
Liver disease
Azathioprine
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9
Q

Symptoms of anaemia

A 
Tiredness 
SoB
Headaches
Dizziness
Palpitations
Worsening of other conditions
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10
Q

Symptoms specific to iron deficiency anaemia

A

Pica: cravings for dirt, ice

Hair loss: iron deficiency anaemia

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11
Q

Genetic signs of anaemia

A

Pale skin
Conjunctival pallor
Tachycardia
Raised RR

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12
Q

Signs of iron deficiency anaemia

A

Koilonychia
Angular cheilitis
Atrophic glossitis
Brittle hair and nails

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13
Q

Sign of haemolytic anaemia

A

Jaundice

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14
Q

Sign of thalassaemia anaemia

A

Bone deformities

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15
Q

Complications of chemotherapy

A 
Failure to treat leukaemia 
Stunted growth and development
Immunodeficiency and infections 
Neurotoxicity
Infertility
Secondary malignancy
Cardio toxicity
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16
Q

Investigations for anaemia

A 
FBC
Blood film 
Reticulocyte count
Ferritin 
B12 and folate
Bilirubin: raised in haemolysis
Direct Coombs test: positive in autoimmune haemolytic anaemia
Haemoglobin electrophoresis: thalassaemia, sickle cell anaemia
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17
Q

Iron deficiency anaemia causes

A

Dietary insufficiency
Loss of iron, heavy menstruation
Inadequate iron absorption, e.g. Crohn’s disease
PPI as iron needs acid in stomach to stay soluble 2+
Coeliac/ Crohn’s reduce absorption in duodenum and jejunum

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18
Q

How to calculate transferrin saturation

A

Transferrin saturation = serum iron/ total iron binding capacity

Should be around 30%

Increased value of results with:
Iron supplements
Acute live damage

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19
Q

Management of iron deficiency anaemia

A

Treat underlying cause
Dietician input
Ferrous sulphate or ferrous fumarate
SE: constipation and black stools

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20
Q

Leukaemia

A

Cancer of stem cells in bone marrow

Unregulated production of certain types of blood cells

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21
Q

Types of leukaemia

A

Acute lymphoblastic leukaemia: most common in children
Acute myeloid leukaemia
Chronic myeloid leukaemia: rare

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22
Q

Epidemiology leukaemia

A

All peaks at 2-5 years

Males > females

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23
Q

Pathophysiology leukaemia

A

Genetic mutations, infections
Disruption in regulation and proliferation of lymphoid precursor cells in bone marrow
Excessive production of immature blast cells
Drop in numbers of functional RBC, WBC, platelets

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24
Q

Leukaemia risk factors

A 
Radiation exposure
Down’s syndrome
Kleinfelters syndrome
Noonan syndrome
Fanconis anaemia
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25
Q

History of leukaemia

A 
Anaemia: lethargy, pale
Thrombocytopenia: easy bruising, bleeding, petechiae
Leukopenia: unexplained fevers, infections 
Bone pain: hyper plastic marrow 
Weight loss
Malaise
CNS involvement: headaches, seizures 
Night sweats 
Abdominal pain
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26
Q

Leukaemia examination

A

Pale
Unexplained bruising, bleeding
Lymphadenopathy
Hepatosplenomegaly

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27
Q

DD bruising

A 
Leukaemia
Immune thrombocytopenia
Trauma 
Non-accidental injury 
Ehler-Danlos
VitC deficiency
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28
Q

DD lymphadenopathy

A 
Infective: 
Infectious mononucleosis 
HIV, seroconversion illness
Eczema with secondary infection
Rubella
Toxoplasmosis
CMV
TB
Roseola infantum

Neoplastic:
Leukaemia
Lymphoma

Others:
Autoimmune conditions: SLE, rheumatoid arthritis
Sarcoidosis
Drugs: phenytoin, allopurinol, isoniazid
Graft vs host disease

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29
Q

DD pallor

A 
Pernicious anaemia
Axillary/ brachial embolus 
Acute lymphoblastic leukaemia
Neonatal hypoglycaemia
Myeloma
Neuroblastoma
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30
Q

Acute lymphoblastic leukaemia investigations

A 
FBC: pancytopenia 
Blood film: blast cells
CXR to exclude mediastinal mass
Bone marrow aspirate/ trephine
Lumbar puncture
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31
Q

Risk scoring acute lymphoblastic leukaemia

A

Cytogenetic testing
Age: 1-10 at presentation have a good prognosis
WCC>50 poorer prognosis
Females > male prognosis
CNS involvement: blasts within CSF, poorer prognosis
Leukaemia characteristics

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32
Q

Diagnosis of leukaemia

A

Urgent FBC within 48 hours

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33
Q

Management of acute lymphoblastic leukaemia

Immediate

A

Resuscitate and stabilise unwell child
If high WCC: hyperhydration to prevent hyper viscosity
If mediastinal mass: steroids to reduce airway compromises
Infection/ sepsis: broad spectrum antibodies

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34
Q

Definitive and long-term management of acute lymphoblastic leukaemia

A

UKALL 2011 protocol
IV chemotherapy, orally, intra-thecally (into CSF)
Supportive care with blood products (red cells, platelets)
Prophylactic anti-fungal therapy throughout treatment
No role for radiotherapy in management
Maintenance treatment is 2 years for girls and 3 years for boys

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35
Q

Prognosis and complications of acute lymphoblastic leukaemia

A 
90% survival 
Infertility
Avascular necrosis
Peripheral neuropathy 
Anxiety

Regular follow-up and assessment for 5 years after completion of treatment

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36
Q

Acute myeloid leukaemia pathophysiology

A

Cancer of blood and bone marrow
Myeloid stem cell-> RBC, WCC, platelets
Self-renewal and developmental arrest of progenitor cells at a particular point in their differentiation
Creating immature cells (myeloblasts) that infiltrate bone marrow, RES
Less room in blood and bone marrow for health cells
Infection, anaemia or easy bleeding can occur
Leukaemia cells can spread to CNS, skin and gums. Occasionally leukaemia cells form a solid tumour called a chloroma or granulocytic sarcoma

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37
Q

FAB classification of AML

A 
MO: AML with minimal evidence of myeloid differentiation
M1: AML without maturation
M2: AML with maturation
M3: acute promyelocytic leukaemia
M4: acute myelomonocytic leukaemia
M5: acute monocytic/ monoblastic leukaemia
M6: acute erythroleukaemia
M7: acute megakaryoblastic leukaemia
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38
Q

Risk factors for acute myeloid leukaemia

A 
Down’s syndrome
Li-Fraumeni syndrome
Aplastic anaemia
Myelodysplasia
Affected sibling
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39
Q

Clinical features of acute myeloid leukaemia

A

Classification signs of anaemia, thrombocytopenia, hepatosplenomegaly, or lymphadenopathy

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40
Q

Bone marrow failure in AML

Symptoms/ signs

A

Anaemia: pallor, lethargy, shortness of breath, dizziness, palpitations, reduced exercise tolerance

Neutropenia: fever, recurrent infections, unusual infections

Thrombocytopenia: brushing, petechia, epistaxis

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41
Q

Blast infiltration of other tissues in acute myeloid leukaemia
Symptoms/ signs

A

Bone marrow: limb pains

Reticuloendothelial: hepatosplenomegaly, lymphadenopathy, expiratory wheeze (secondary to a mediastinal mass due to lymphadenopathy or thymic infiltration/ expansion)

Testes: testicular enlargement

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42
Q

Systemic effects of cytokines released by leukaemic cells and of increased plasma viscosity (leucoastasis) due to extremely high WCC
Signs/symptoms

Acute myeloid leukaemia

A

Cytokine release: fever, malaise, fatigue, nausea

Leucostasis: headache, vomiting, cranial nerve palsies, seizures, stroke, shortness of breath, heart failure

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43
Q

DD of acute myeloid leukaemia

A

Acute lymphocytic leukaemia: pancytopenia
Iron deficiency anaemia: pallor, lethargy, SoB
Immune thrombocytopenic purpura
Immunodeficiency: recurrent infections

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44
Q

Acute myeloid leukaemia

Laboratory tests

A

FBC: pancytopenia

Blood film: blasts present, elevated overall WCC, atypical cells in blood film, presence of leukoerythroblastic features

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45
Q

Acute myeloid leukaemia

Imaging or invasive tests

A

CXR: information on whether any of the lymph glands in chest are enlarged
Bone marrow aspirate and trephine- bone marrow examination allows definitive diagnosis of acute leukaemia
Lumbar puncture: checks for leukaemia cells in CSF, may require intra-thecal chemotherapy as part of their treatment
Biopsy: AML diagnosed by biopsy of a chloroma

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46
Q

Bone marrow examination in acute myeloid leukaemia

A

Aspirate: morphological, immunological, genetic information. Information used alongside clinical factors and initial response to chemotherapy
Light microscopy: allows classification as acute lymphoblastic leukaemia or acute myeloid leukaemia
Immunophenotyping using flow cytometry, identifies patterns of cell surface antigens associated with particular subtypes of acute myeloid leukaemia

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47
Q

Management of acute myeloid leukaemia

Initial management

A

Indication; induces remission

Post-remission consolidation/ intensification; reduces risk of relapse

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48
Q

Management of acute myeloid leukaemia

Induction

A

Two cycles of chemotherapy given four weeks apart
Examining bone marrow after each cycle will allow monitoring of response to induction
Aim of induction: no evidence of leukaemia in bone marrow after induction is completed (remission)

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49
Q

Definitive and long term management of acute myeloid leukaemia

A

Post-remission therapy

Bone marrow transplant

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50
Q

Post-remission therapy AML

A

Further chemotherapy to destroy any remaining leukaemia cells and to prevent recurrence
Varying number of cycles of intensive chemotherapy and/or allogenic haematopoeitic stem cell transplantation

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51
Q

Bone marrow transplant AML

A

Reserved for children with an suboptimal response to standard chemotherapy Or if leukaemia relapses
20% of AML will require a transplant

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52
Q

Complications of treatment of acute leukaemia

A 
Neutropenic sepsis: multi-organ failure
Bone marrow suppression (myelosuppression), pancytopenia 
Nausea and vomiting
Mucositis
Hair loss
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53
Q

Specific side effects of chemotherapy agents

A

Doxorubicin- cardiotoxicity
Vincristine- peripheral neuropathy
Cyclophosphamide- reduced fertility
Cytarabine- hepatotoxicity

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54
Q

Lymphoma

A

Malignancy of the lymphatic system

Divided into Hodkin’s lymphoma and non-Hodgkins lymphoma

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55
Q

Epidemiology of lymphoma

A

Accounts for 10% of childhood cancers
More common in boys than girls
More common in older children
More than half of lymphoma cases are non-Hodgkin lymphoma

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56
Q

Pathophysiology of lymphoma

A

Multifactorial development; infection, genetic factors, environmental exposures
Lifestyle factors in adults: obesity, smoking, alcohol intake

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57
Q

History of lymphoma

A

EPV implicated in development of lymphoma. Immunosuppressed patients and those who have been treated for other cancers in the past are also at increased risk of lymphoma
B symptoms: weight loss, night sweats, fevers
Lethargy and anorexia
Visible or palpable mass

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58
Q

Examination of lymphoma

A

Non-tender lymphadenopathy
Non visible/ palpable if mediastinal or intra-abdominal lymph nodes are involved
Mediastinal lymphadenopathy: cough, wheeze or other difficulty in breathing, SVC obstruction or airway compromise can occur

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59
Q

DD of lymphoma

A

Reactive lymphadenopathy, History of recent infection. If lymph nodes themselves have become infected (lymphadenitis) they are likely to be tender and potentially fluctuant if an abscess has formed

Leukaemia: lymphadenopathy with signs/symptoms of anaemia and/or thrombocytopenia

Lymphadenopathy: can also be a sign of metastatic malignancy from another site

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60
Q

Lymphoma laboratory tests

A

Blood tests: FBC
U&E for tumour lysis syndrome can occur before treatment begins in lymphomas with rapid cell turnover
LDH: levels are usually elevated

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61
Q

Lymphoma imaging

A

USS of the area can help identify other nodes, and assists with biopsy

CXR: required if there are symptoms of mediastinal node involvement

Full body CT to determine extent of disease

Biopsy: lymph node biopsy for definitive diagnosis

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62
Q

Risk scoring lymphoma

A

Stage 1: single group of lymph nodes or a single organ
Stage 2: disease is present in 2 or more groups of lymph nodes or organs on the same side of the diaphragm
Stage 3: disease is present in lymph nodes or organs on both sides of the diaphragm
Stage 4: diffuse involvement of lymph nodes and organs such as the liver and bones

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63
Q

Lymphoma B symptoms

Associated with worse prognosis

A

Weight loss
Night sweats
Fevers

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64
Q

Immediate management of lymphoma

A

Presence of a mediastinal mass with potential airway compromise is an emergency
Treatment with high dose steroids and airway support if required
SVCO may require stenting of veins to keep them patent, usually resolve with treatment of the underlying malignancy
Suggestion of tumour lysis syndrome, then hyperhydration is important. Allopurinol or rasburicase are also used

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65
Q

Definitive and long-term management of lymphoma

A

Treatment is with chemotherapy ans possibly radiotherapy, depending on the stage of disease

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66
Q

Prognosis of lymphoma

A

Hodgkins > non-Hodgkins

Majority will recover coompletley

67
Q

Complications of lymphoma

A

Tumour lysis syndrome: rapid lysis of tumour cells cases release of large amounts of phosphorous, potassium and calcium
Leading to potential kidney damage
Neutropenia, alopecia, sub-fertility
Life-long follow up for complications

68
Q

Nephroblastoma

A

Wilm’s tumour
Most common renal tumour affecting children
Usually unilateral but can be bilateral

69
Q

Nephroblastoma epidemiology

A

Affects 80 children per year in UK
Generally presents in the pre-school age group
3.5years being median age at diagnosis
Slight female predominance

70
Q

Pathophysiology of nephroblastoma

A

Tumours arise from nephrogenic rests;embryonal remnants seen in around 1% of infants at birth

71
Q

Risk factors for nephroblastoma

A

Children with certain genetic and overgrowth syndromes
WAGR (Wilms tumour, aniridia, genitourinary malformations, retardation)
Denys-drash and beckwith-wiedemann

72
Q

Nephroblastoma history

A

Abdominal mass found incidentally
Noted by parents during bathing or dressing in an otherwise well child
Abdominal swelling, abdominal pain, fever, or haematuria

73
Q

nephroblastoma examination

A

Abdominal distension
Unilateral or bilateral palpable masses
Hypertension may be noted
Rarely, in cases of advanced disease, signs of compression of other intra-abdominal structures

74
Q

DD of nephroblastoma

A

Polycystic kidney disease and hydronephrosis
Most common malignant DD in a pre-school child is a neuroblastoma: presents with HTN, abdominal pain or fever
Neuroblastoma: periorbital ecchymosis, abdominal mass which crosses midline, signs of bone marrow infiltration

75
Q

Nephroblastoma laboratory tests

A

FBC
U&E
Urine dip for haematuria
Evaluate child’s general heath at presentation

76
Q

Nephroblastoma imaging or invasive tests

A

Initial characterisation of a renal/ abdominal mass: USS, CT/MRI for staging
Biopsy

77
Q

Risk scoring nephroblastoma

A

1: tumour is only confined to the kidney and can be completely removed with surgery
2: tumour has begun to spread beyond the kidney, but can still be completely removed with surgery
3: tumour cannot be completely surgically resected because it has spread to neighbouring lymph nodes or ruptured before/during surgery
4: distant metastases, most commonly to lungs
5: bilateral tumours

78
Q

Initial management nephroblastoma

A

Supportive care

Treat co-existing infection and ensure nutrition and hydration are optimised

79
Q

Definitive and long-term management nephroblastoma

A

Stage 1 and 2 tumours may be treated solely with surgery
No additional benefit to giving chemotherapy which has additional risks later in life
Aim of surgery: preserve renal function while removing the malignant tissue
Chemotherapy: used to reduce the volume of malignant tissue before surgery, or to treat any areas of malignant disease not removed by surgery (nephrectomy)
Surgery consists of nephrectomy: in bilateral disease, attempt to preserve as much functioning renal tissue as possible

80
Q

Complications and prognosis nephroblastoma

A

Steps must be taken to protect the remaining kidney
Maintain blood pressure
Avoidance of contact sports, which could lead to abdominal trauma

Monitor for cardiotoxicity and/or radiotherapy

81
Q

Neuroblastoma

A

Paediatric cancer
Derived from neural crest cells, arising in adrenal glands or abdominal sympathetic chain
Most common cancer in children <1 year old, much less common in >5s

82
Q

Epidemiology of neuroblastoma

A

Affects around 90 children per year in the UK
Most common solid tumour in children under one year of age
36% of neuroblastomas arise from the medulla of the adrenal glands, with another 18% arising from the sympathetic chain
10% are bilateral, 10% arise from an unknown source and are first diagnosed as secondary metastases

83
Q

Pathophysiology of neuroblastoma

A

Neuroblastoma arises from poorly differentiating embryonic cells (blasts)
In this case from neural crest
Neural crest cells are derived from developing ectoderm, and normally migrate throughout the body to form SNS and adrenal medulla
When migration is stalled, neural crest cells have the potential to acquire mutations that eventually lead to neuroblastoma
Associated with genetic mutations, MYCN and ALK oncogenes, as well as loss-of-function of the tumour suppressor PHOX2B

84
Q

Risk factors for neuroblastoma

A

No identified cause
Hirschsprung’s disease
Congenital central hypoventilation syndrome

85
Q

History of neuroblastoma

A

Abdominal distension
Fatigue
Appetite loss
Weight loss
Increased catecholamine: sweating, agitation
Metastasis: bone pain that prevents sleep, recurrent infections
Compression of SNS can lead to urinary incontinence
Occasionally, neuroblastomas can arise from the thoracic portion of sympathetic chain, causes SOB and chest pain

86
Q

Neuroblastoma examination

A

Dense abdominal swelling, across midline
Hypertensive and tachycardia due to excess catecholamine synthesis
Symptoms of metastasis: periorbital bruising for children with metastases to skull base
Neuroblastoma cells can metastasise to dermis: scattered purpura across skin to give blueberry muffin rash (non-specific)
Bone metastases: bone marrow infiltration, evidence of recurrent infections and thrombocytopenic purpura

87
Q

Neuroblastoma differentials

A

Abdominal mass:
Cysts: hepatic, polycystic kidney disease
Hyperplasia: pyloric stenosis, hepatomegaly, splenomegaly
Neoplasia: Wilm’s tumour, lymphoma, rhabdomyosarcoma, hepatoblastoma
Blueberry muffin rash: TORCH infections or acute myeloid leukaemia

88
Q

Lab tests neuroblastoma

A

Look for products of catecholamine breakdown: homovanilic acid, vanillylmandelic acid in urine
90% of patients will have raised HMA and VMA
Additionally: bone marrow and skin biopsies, evidence of metastasis to these sites

89
Q

Neuroblastoma imaging

A

USS: paediatric abdominal lumps
MRI second line
For thoracic neuroblastomas: CXR
Definitive test for neuroblastoma: MIBG scan
Radioisotope of iodine is injected, and two scans are taken 24 hours apart
Iodine stays in tumour, becoming an intensely dark region on the scan
90-95% of children with neuroblastoma will have positive features on the MIBG-scan

90
Q

Staging for neuroblastoma

Imaging

A

Neuroblastoma risk group
1: fully resectable at surgery
2A: tumour is unilateral but not fully resectable, no spread to local lymph nodes
2B: tumour is unilateral but is not resectable, with spread to ipsilateral lymph nodes
3: tumour has crossed midline, or spread to Contralateral lymph nodes
4: distant metastasis outside of local lymph nodes
4S: metastases confined to liver, skin or bone marrow in <18monyjhs

91
Q

Staging for neuroblastoma

Surgical observations

A

L1: localised tumour that doesnt involve vital structures (surgically resectable)
L2: localised tumour that does involve vital structures (non-resectable)
M: distant metastases (excludes local lymph node metastases)
MS: metastases confined to liver, skin or bone marrow in patients <18months old

92
Q

Management of neuroblastoma

A

<18months:
Will regress to a benign ganglioma
Monitoring

Older children, aggressive disease:
Surgery
L1- curative
L2;- need adjuvant chemotherapy or radiotherapy

93
Q

Factors giving improved neuroblastoma prognosis

A

Younger age at diagnosis
Assigned female at birth
Lesser tumour stage at diagnosis
MYCN mutation absent

94
Q

Complications of neuroblastoma

A

Relapse
Opsoclonus myoclonus ataxic syndrome: autoimmune disorder arising from antibody self-reactivity to proteins from dying neuroblast cells, which then lead to an immune reaction to CNS (cerebellum)

Opsoclonus: uncontrolled, irregular eye movements
Myoclonus: muscle spasm
Ataxia: lack of voluntary movement control
Confusion
Irritability

95
Q

Astrocytoma

A

Low and high grade gliomas that develop from glial cells

96
Q

Medulloblastoma

A

Usually develop in posterior fossa/ cerebellum

97
Q

Ependymoma

A

Formed from CSF producing ependymal cells

98
Q

Craniopharyngioma

A

Found at base of brain close to pituitary gland

99
Q

Germ cell tumours

A

Arise from germ cells

Found close to pituitary gland and pineal gland

100
Q

Choroid plexus tumours

A

Develop from network of ependymal cells

101
Q

Risk factors primary brain tumours

A 
Personal/family history of brain tumour, leukaemia, sarcoma and early onset breast cancer
Prior therapeutic CNS irradiation
Neurofibromatosis 1 and 2
Tuberous sclerosis 1 and 2
Von Hippel-Lindau
102
Q

History of brain tumour

A 
Headache: mass effect or hydrocephalus
N/V
Behavioural change: due to tumours found in frontal lobe
Polyuria/poldipsia: diabetes insipidus
Seizures 
Altered GCS
103
Q

Primary brain tumour examination

A

General: behaviour, conscious level, alertness
Visual: diplopia, reduced visual acuity/fields, eye movement, fundoscopy
Motor signs: abnormal gait or coordination, swallowing difficulties, weakness
Delayed growth
Delayed, arrest or precocious puberty
Increased head circumference if under 2 years old (growth chart)

104
Q

DD primary brain tumour

A 
Migraine or tension headaches
Meningitis/ encephalitis 
Intracranial haemorrhage/ stroke
Otitis media: unsteady gait
Neurofibromatosis
105
Q

Brain tumour investigations

A

MRI

2nd: contrast enhanced CT

106
Q

Management CNS malignancy

A 
Analgesia, antiemetic, anticonvulsants, fluid/dietary support, treatment to lower ICP (steroids)
Surgical resection
CSF shunts for hydrocephalus
Radiotherapy
Chemotherapy: BBB?
Proton therapy
Stem cell transplantation
107
Q

Complications of CNS malignancy

A 
Epilepsy and seizures
Sleep disturbance
Effects on puberty/ fertility
Hearing loss
Impaired growth 
Cognitive impairment
Secondary malignancy
108
Q

Idiopathic thrombocytopenic purpura

A 
Low platelet count
Causing a purpuric rash 
TY2 hypersensitivity reaction 
Antibodies target platelets 
Non-blanching rash
109
Q

Presentation of ITP

A 
<10 years old 
Recent viral illness
Symptoms onset 24-48hours
Bleeding: gums, epistaxis, menorrhagia 
Bruising
Petechiae or purpuric rash, caused by bleeding under the skin
110
Q

ITP management

A

Urgent FBC for platelets
Exclude heparin induced thrombocytopenia and leukaemia
Resolve <3 months

Active bleeding or severe thrombocytopenia:
Prednisolone
IV immunoglobulins
Blood transfusions
Platelet transfusions: temporarily work until antibodies destroy them too

111
Q

Advice for patients with thrombocytopenia

A 
Avoid contact sports 
Avoid IM injections and lumbar puncture
Avoid NSAIDS, aspirin, blood thinners
Advice on managing nosebleeds
Seek help after any injury that may cause internal bleeding
112
Q

ITP complications

A

Chronic ITP
Anaemia
Intracranial and subarachnoid haemorrhage
GI bleeding

113
Q

Sickle cell anaemia

A

Genetic condition that causes sickle shaped RBC

Makes them fragile and more easily destroyed, leading to haemolytic anaemia

114
Q

Sickle cell anaemia pathophysiology

A

Haemoglobin is the protein in RBC that transports oxygen
HbF—>HbA at 6 weeks of age
HbS
Autosomal recessive
Abnormal gene for beta-haemoglobin on chromosome 11

115
Q

Sickle cell and malaria

A

Sickle cell trait reduces risk of malaria

Selective advantage

116
Q

Sickle cell diagnosis

A

Newborn screening heel prick test at 5 days of age

117
Q

Sickle cell complications

A 
Anaemia
Increased risk of infection
Stroke
Avascular necrosis in large joints
Pulmonary hypertension
Painful and persistent penile erections
Chronic kidney disease
Sickle cell crises
Acute chest styndrome
118
Q

Sickle cell disease management

A

Avoid dehydration and triggers
Ensure vaccines are up to date
Antibiotic prophylaxis: Penicillin V (phenoxymethylpenicillin)
Hydroxycarbamide: stimulates production of HbF
Blood transfusion for severe anaemia
Bone marrow transplant can be curative

119
Q

Sickle cell crisis

A 
Low threshold for hospital admission
Analgesia
Rehydrate
Oxygen 
Treat infection
Keep warm 
Penile aspiration to treat priapism 
Blood transfusion
120
Q

Vaso-occlusive crisis

Sickle cell disease

A

Sickle shaped blood cells clogging capillaries and causing distal ischaemia
Associated with dehydration and raised haematocrit
Symptoms: pain, fever, symptoms of triggering infection
Priapism in men: trapping blood in penis, urological emergency and treated with aspiration of blood from the penis

121
Q

Splenic sequestration crisis

A

RBC blocking blood flow within spleen
Causes acutely enlarged and painful spleen
Pooling of blood in spleen: severe anaemia, circulatory collapse (hypovolaemic shock)

Emergency
Management is supportive, with blood transfusions and fluid resuscitation to treat anaemia and shock
Recurrent crisis: splenectomy is management, as recurrent crises can lead to splenic infarction and susceptibility to infections

122
Q

Aplastic crisis

A

Temporary loss of creation of new blood cells
Most commonly triggered by infection with parvovirus B19
Leads to anaemia
Management is supportive: blood transfusions, usually resolves spontaneously within a week

123
Q

Acute chest syndrome

A

Diagnosis:
Fever or respiratory symptoms
New infiltrates seen on a CXR

Acute chest syndrome can be due to infection (pneumonia or bronchiolitis) or non-infective causes (pulmonary vaso-occlusion or fat emboli)

124
Q

Acute chest syndrome management

A

Antibiotics or antivirals for infections
Blood transfusions for anaemia
Incentive spirometry using a machine that encourages effective and deep breathing
Artificial ventilation with NIV or intubation may be required

125
Q

Thalassaemia

A

Genetic defect in the protein chains that make up haemoglobin
Normal Hb: 2 alpha and 2 beta
Both conditions are autosomal recessive
Varying degrees of anaemia
RBC more fragile and break down more easily
Spleen removes dead RBC, splenomegaly
Bone marrow expands to produce extra red blood cells to compensate for the chronic anaemia
Susceptibility to fractures and prominent features, such as a pronounced forehead and malar eminences (cheek bones)

126
Q

Thalassaemia potential signs and symptoms

A 
Microcytic anaemia (low mean corpuscular volume)
Fatigue
Pallor
Jaundice
Gallstones
Splenomegaly
Poor growth and development
Pronounced forehead and malar eminences
127
Q

Diagnosis of thalassaemia

A

FBC: microcytic anaemia
Haemoglobin and electrophoresis: used to diagnose globin abnormalities
DNA testing: genetic abnormality

Pregnant women offered a screening test for thalassaemia at booking

128
Q

Thalassaemia iron overload

A

Iron overload due to faulty creation of RBC, recurrent transfusions and increased absorption of iron in the gut in response to anaemia
Monitor serum ferritin levels in thalassaemia
Management of iron overload: limiting transfusions and performing iron chelation

129
Q

Iron overload in thalassaemia

Effects

A 
Fatigue
Liver cirrhosis
Infertility
Impotence
Heart failure
Arthritis
Diabetes
Osteoporosis and joint pain
130
Q

Management of alpha thalassaemia

A 
Monitoring the FBC
Monitoring for complications
Blood transfusions
Splenectomy may be performed
Bone marrow transplant can be curative

Caused by defects in the alpha globin chains

131
Q

Beta thalassaemia management

A

Caused by defects in beta globin chains
Gene defect can either consist of abnormal copies that retain some function or deletion genes where there is no function in beta globin protein at all

Thalassaemia minor
Thalassaemia intermedia
Thalassaemia major

132
Q

Thalassaemia minor

A

Carries of abnormally functioning beta globin gene
One normal and one abnormal gene
Mild microcytic anaemia, usually patients only require monitoring and no active treatment

133
Q

Thalassaemia intermediate

A

Two abnormal copies of the beta globin gene
Can either be two defective genes or one defective and one deletion gene

Causes a more significant microcytic anaemia
Patients require monitoring and occasional blood transfusions
Iron chelation to prevent iron overload, when they require more transfusions

134
Q

Thalassaemia major

A

Homozygous for deletion genes
No functioning beta globin genes at all
Presents with severe anaemia and failure to thrive in eagerly childhood

135
Q

Causes of thalassaemia major

A

Severe microcytic anaemia
Splenomegaly
Bone deformities

136
Q

Management of thalassaemia major

A

Regular transfusions
Iron chelation and splenectomy
Bone marrow transplant can potentially be curative

137
Q

Hereditary spherocytosis

A

Condition where RBC are sphere shaped, making them fragile and easily destroyed when passing through spleen
Most common haemolytic anaemia in Northern European
Autosomal dominant

138
Q

Presentation of hereditary spherocytosis

A

Jaundice
Anaemia
Gallstones
Splenomegaly

Haemolytic crisis
Aplastic anaemia

139
Q

Haemolytic crisis in hereditary spherocytosis

A

triggered by infections, where haemolysis, anaemia and jaundice is more significant

140
Q

Hereditary spherocytosis aplastic crises

A

Increased anaemia, haemolysis, jaundice
Without normal response from bone marrow of creating new RBCs
No reticulocyte response
Often triggered by infection with parvovirus

141
Q

Diagnosis of hereditary spherocytosis

A

FH, clinical features
Along with spherocytosis on blood film
Mean corpuscular Haemoglobin concentration is raised on FBC
Reticulocytes raised due to rapid turnover of RBCs

142
Q

Management of hereditary spherocytosis

A

Treat with folate Supplementation
Splenectomy
Cholecystectomy if gallstones are the problem
Transfusions may be required during acute crises

143
Q

Hereditary elliptocytosis

In hereditary spherocytosis

A

RBC ellipse shaped
Autosomal dominant
Presentation and management are very similar

144
Q

G6PD deficiency

A

X-linked recessive, usually affects males
More common in Mediterranean, Middle Eastern, African patients
Crises that are triggered by infections, medications or fava beans (broad beans)

145
Q

Pathophysiology of G6PD deficiency

A

Enzyme is responsible for helping protect cell from damage by reactive oxygen species
ROS are reactive molecules that contain oxygen, producing during normal cell metabolism and in higher quantities during stress on the cell
G6PD enzyme deficiency makes cells more vulnerable to ROS
Leading to haemolysis in RBC
Periods of increased stress, with higher production of ROS can lead to acute haemolytic anaemia

146
Q

G6PD deficiency presentation

A 
Neonatal jaundice
Anaemia
Intermittent jaundice
Gallstones
Splenomegaly

Heinz bodies may be seen on a blood film
Heinz bodies are blobs of denatured haemoglobin (inclusions) seen within RBCs

Diagnosis can be made by going a G6PD deficiency

147
Q

Management of G6PD deficiency

A

Patients should avoid triggers of acute haemolysis where possible
Includes avoiding fava beans and certain medications

148
Q

G6PD deficiency

Medications that trigger haemolysis and should be avoided

A 
Primaquine
Ciprofloxacin 
Nitrofurantoin
Trimethoprim
Sulphonylureas
Sulfasalazine and other sulphonamide drugs
149
Q

Henoch-schonlein purpura

A

IgA vasculitis
Presents with a purpuric rash affecting lower limbs and buttocks in children
IgA deposits in blood vessels
Inflammation occurs in the affected organs
Affects skin, kidneys, GI tract
Condition triggered by an upper airway infection or gastroenteritis
Most common <10 years

150
Q

Four classic features of HSP

A

Purpura- lumps under skin containing blood
Joint pain
Abdominal pain
Renal involvement

151
Q

HSP purpura

A 
Purpura:
Palpable under skin 
Start on legs and spread to buttocks 
Also affect trunks and arms 
Skin ulceration and necrosis can develop
Red-purple in colour
152
Q

Arthralgia or arthritis HSP

A

Arthralgia or arthritis:
Mostly affecting knees and ankles
Joints can becomes swollen and painful
Reduced range of movement

153
Q

HSP abdominal pain

A 
Abdominal pain:
No GI involvement
GI haemorrhage
Intussusception
Bowel infarction
154
Q

HSP kidneys

A

Kidneys:
IgA nephritis
HSP nephritis
Microscopic or macroscopic haematuria and proteinuria
>2+ protein on urine dipstick, child has developed nephrotic syndrome and will have a degree of oedema

155
Q

Diagnosis of HSP

A

Exclude meningococcal septicaemia, leukaemia, ITP, HUS

FBC and blood film: thrombocytopenia, sepsis, leukaemia
Renal profile for kidney involvement
Serum albumin: nephrotic syndrome
CRP for sepsis
Blood cultures
Urine dipstick for proteinuria 
Urine protein: creatinine ratio 
Blood pressure for HTN
156
Q

EULAR/PRINTO/PRES criteria for diagnosing HSP

A

Palpable purpura

+ one of

Diffuse abdominal pain
Arthritis or arthralgia
IgA deposits on histology (biopsy)
Proteinuria or haematuria

157
Q

Management of HSP

A

Supportive, simple analgesia, rest, hydration

Urine dipstick for renal involvement
Blood pressure for HTN

Abdominal pain settles in a few days
Patients without kidney involvement recover in 4-6weeks
A third have a recurrence of disease within 6 months
ESRD in a small proportion

158
Q

DIC

A 
Tissue factor (transmembrane glycoprotein)
Exposed to circulation after vascular damage: present on surface of endothelial cells, macrophages, and monocytes 
TF is released in response to exposure to cytokines (IL-1), TNF, and endo toxin 
Abundant in lungs, brain and placenta 
TF triggers extrinsic and intrinsic pathway
159
Q

Causes of DIC

A

Sepsis
Trauma
Obstetric complications, e.g. amniotic fluid embolism or haemolysis, elevated LFT, low platelets (HELLP syndrome)
Malignancy

160
Q

Diagnosis of DIC

A 
Decreased platelets
Decreased fibrinogen
Increased PT & APTT
Increased FDP
Schiscocytes due to microangiopathic haemolytic anaemia
161
Q

Diagnosis of hyposplenism

A

Radionucleotide labelled red cell scan

162
Q

Causes of hyposplenism

A

Splenic artery embolizartion

Splenectomy for trauma

163
Q

Management of hyposplenism

A

Pneumococcal, Haemophilus TYB, meningococcal TYC vaccines. 2 weeks prior to splenectomy or two weeks following splenectomy

Annual influenza vaccine

Post-splenectomy crisis, penicillin or macrolides prophylaxis for high risk individuals

Risk of malaria

164
Q

Hyposplenism blood film

A 
Target cells
Howell-Jolly bodies
Pappenheimer bodies
Siderotic granules
Acanthocytes

Child Health (20 decks)

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