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good paeds > Haemotology/Oncology > Flashcards

Haemotology/Oncology Flashcards

1
Q

Post transplant lymphoproliferative disease related to what medication

A

T-cell suppression (especially with tacrolimus) is associated with a higher risk
of PTLD.

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2
Q

How much iron in 250ml blood unit

A

250mg

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3
Q

Vincristine and vinblastine mechanism of action

A

Inhibit mitosis by binding tubulin (minimal myelosuppression) METAPHASE

(Asparginase stops rna into tubulin protein)

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4
Q

Doxorubicin and doaunorubicin mechanism of action

A

Stops DNA replication by inhibiting topoisomerase and is a DNA intercalator (inserts into DNA) G2 phase
(Antibiotic) and also free radical damage like bleomycin

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5
Q

Cyclophosphamide and platinum mechanism of action

A

alkalynisation of DNA
Forms DNA cross links leading to apoptosis
Also ifosfamide

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6
Q

Cause of short stature with cranio-spinal radiotherapy

A

Failure of spinal growth

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7
Q

Highest incidence of secondary malign

A

Hodgkin lymphoma (10% at 20y)

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8
Q

Diamond-Blackfananaemia

A

Ribosomal protein disease
Dysmorphic, short, cleft, thumbs weird
Under 1, isolated MACROCYTIC anaemia from marrow failure (therefore no retics )
Bone marrow with decreased erythroid precursors but normal marrow
cellularity
(TEC also low retics but normocytic)

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9
Q

Bone marrow dysfunction with pancreas insufficiency and growth failure ? Cause and uss finding

A

Fatty infiltrate pancreas

Shwachman syndrome

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10
Q

Tumour lysis syndrome mx

A 
Hyperhydrate
Allopurinol
xanthine oxidase inhibitor
Risk of xanthine nephropathy
Rasburicase (0.2 mg/kg)
Urate oxidase
Contraindicated in G6PD deficiency
Risk of rash, hemolysis, anaphylaxis, methemoglobinemia
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11
Q

Drugs associated with mucositis

A

Bleomycin, Daunomycin, Methotrexate, Mitozantrone, Thioguanine,
Thiotepa, Vinblastine, Idarubicin, Mercaptopurine, Melphalan, 5‐
Fluorouracil, Hydroxyurea, Doxorubicin.

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12
Q

No or minimal BM suppression with which drugs

A 
Cisplatin
Asparginase
Dacarbazine
Procarbazine
mercaptopurine
Mitomycin C
Lomustine
Melphalan
New monoclonals
thioguanine
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13
Q

Haemorrhaging cysitis is SE of which chemo agents

A

Alkylating agents: Cyclophosphamide / Ifosfamide

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14
Q

At risk groups for secondary malignancy

A

Radiation exposure: AML, sarcomas
Etoposide exposure: AML, sarcomas
Hodgkins: mantle field irradiation
Genetic predispositons: retinoblastoma, fanconi anaemia, TP53 et

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15
Q

Warm autoimmune haemolytic mx

A

Supportive with folic acid. Avoid transfusing if possible
Warm antibody mediated responds to steroids

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16
Q

Dyskeratosis congenita (zinsser-Cole-England syndrome)

A

One of the disorders associated with short telomeres (Flow
FISH test). Others include Hoyeral Hreidisson and n Revesz
Syndrome)
• Genes TERT, TERC
• Ectodermal dysplasia
• Diagnostic “triad’: dystrophic nails, oral leucoplakia, reticular
pigmentation
• May present with pulmonary or liver fibrosis
• SCT for marrow failure. High morbidity and mortality
• Long term screening for oral, naso-pharyngea, vulval cancers

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17
Q

HLH

A

Hyper inflammation

High TG and high ferritin classic

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18
Q

Mild haemophilia A and VWD management

A

DDAVP in most instances - Works by releasing endogenous FVIII
Transenamic acid

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19
Q

Term male infant notice to have prolonged oozing from heel prick
site. No family history of bleeding.
Normal platelet count, PT, APTT and fibrinogen
Family reassured and baby discharged.
Mother re-presents in two weeks because umbilical stump oozing.
diagnosis?

A

Factor 13 deficiency

Defects in fibrinogen

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20
Q

Medulloblastoma predisposed conditions

A

Gorlin syndrome (basal-cell carcinoma nevus syndrome)
– 3% children with MB
– PTCH pathway mutation
• Turcot’s Syndrome
– Mismatch repair cancer syndrome (Lynch, HNPCC)
– Brain tumour and colorectal polyposis/cancer
– Usually HGG’s or MB in childhood/adolescence
• Li-Fraumeni Syndrome
– TP53
– Bimodal peak <10 years and 20-40 years
– Astrocytomas&raquo_space; MB

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21
Q

Medulloblastoma groups

A

Genetically defined subgroups
1. WNT-activated – monosomy 6, classic histology, excellent
outcome (EFS >90%)
2. SHH-activated – PTCH pathway, lateral tumours, nodular
desmoplastic, infants have excellent outcome, TP53 mutated
tumour have very poor prognosis
3. Non-WNT/non-SHH
Group 3 – MYC amplification, large cell anaplastic
histology (LCA), poor outcome
Group 4 – MYCN amplification, classic or LCA,
intermediate outcome

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22
Q

ATRT (atypical teratoid rhabdoid tumour
Poor prognosis
Supratentorial

A 
• Highly malignant
• Usually occurs before 2 years age
• ~15% children <3 years with malignant CNS 
tumour
• Short clinical history
• Anywhere in brain and spine
• Disseminated disease in 20%
• Biallelic inactivation of SMARCB1 in majority
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23
Q

Ependymoma mx

A

Surgery and focal radiation

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24
Q

High grade glioma

A

Small round blue cell tumours

Recent discovery of driver
mutations in histone H3.3 and
chromatin remodeling genes in
HGG

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25
Q

Craniopharyngioma MRI findings

A

Suprasellar
Calcification
Cysts

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26
Q

TS cancer type

A

Subependymal giant cell astrocytomas (SEGAs ) in 5-20%

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27
Q

Juvenile Pilocystic astrocytoma mx

A

Observe

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28
Q

Common late effect after brain tumour

A

GH deficiency

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29
Q

AML related to which syndrome

A

Downs (<3y, better survival and ALL worse survival)

Familial RUNX1 mutations
Aplastic anaemia
Myelodysplastic syndrome
Myeloproliferative disorders

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30
Q

Extramedullary haematopoesis (skin) think..

A

AML

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31
Q

AML morphology

A

PB/ BM: Blasts >20%
• Larger blasts
• Nucleus: More irregular, prominent nucleoli
• Cytoplasm: Auer rods, granules
Myeloperoxidase in flow cytomegalovirus

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32
Q

CD numbers for B and T cell ALL

A 
B = 19
T = 3

T call ALL overall higher risk and presentation WCC not related to risk

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33
Q

Which cancer has Reed-Sternberg cell
EBV associated

A

Hodgkin lymphoma

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34
Q

Radiotherapy in ewings or osteosarcoma

A

Ewings

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35
Q

Mets of wilms, ewings, osteosarcoma to

A

Lungs

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36
Q

What tumours can metastasise to marrow

A

Alveolar Rhabdomyosarcoma can spread to bone marrow

Ewings

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37
Q

Child with spinal cord compression or intractable diarrhea or opsoclonus/myoclonus = jumping eyes think of…

A

Neuroblastoma

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38
Q

Poor prognostic factors in neuroblastoma

A

Combineclinicalfeatures(Metastases,unresectable biguglyprimary,age>18mths arebad).
Andlabtests(histology,MYCNamplification 8-200+ even if local disease,11qmissingarebad).

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39
Q

Can neuroblastoma regress by itself

A

Yes

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40
Q

Diagnosis in baby: Blueberry nodules on baby rapidly followed by expanding liver and respiratory embarrassment

A

Neuroblastoma

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41
Q

Hepatoblastoma predispositions and mx

A

Genetic predisposition (~15%)
– Beckwith‐Wiedemann syndrome
• IGF2 (11p15.5) overexpression
• Relative risk 2,280
– Familial adenomatous polyposis
• APC mutations
• 1/250 lifetime risk of HB
• ~1/20 with HB have familial polyps
in colon
– Li‐Fraumeni
– Trisomy 18
– NF‐1
– Ataxia‐telangiectasia
– Fanconi anemia
– Tuberous sclerosis
LBW/prem

Mx: cisPLATIN and carboPLATIN

42
Q

Beckwith‐Wiedemann Syndrome how many percentage tcsncer and screen til what age

A

10%

8 years

43
Q

What syndrome
Macrocephaly, autism/LD, skin lesions, lipomas, AVMs
• Thyroid, breast and endometrial cancer; also colon cancer, melanoma,
renal cell carcinoma
• Thyroid cancer screening starting at 10‐12 yo

A

PTEN syndrome

44
Q

What is a nephrogenic rests

A

Embryonal reminent and may develop into nephroblastoma,

45
Q

AFP is increased in…

A

HBL, HCC, and germ cell tumours most notably yolk sac or
endodermal sinus tumour.

46
Q

Highest risk factor for invasive fungal disease

A

Prolonged neutropenia

47
Q

On CT
Small nodules, pleural based lesions with surrounding low
attenuation = halo sign (esp. if neutropenic)
With progression nodules may cavitate (and with neutrophil
recovery = air crescent sign)
? Diagnosis and treatment

A

Invasive pulm aspergillosis

Voriconazole

48
Q

High risk neuroblastoma can be treated with

A

anti-GD2 (ch14.18)

49
Q

Small round blue cells in which cancers

A

LERRNN

Lymphoma, ewings, rhabdomyosarcoma, retinoblastoma, nephroblastoma, neuroblastoma

50
Q

Hallmark of lanherhans cell histocytosis

A

Tennis racket granule (birbeck granule)

51
Q

Wide radiotherapy in female most common secondary cancer is

A

Breast

52
Q

Emancipation in infant cause?

A

Diencephalic syndrome (hypothalamus and thalamus lesion)

53
Q

PHACE syndrome stands for

A 
Posterior fossa malformation 
Haemangiomas
Arterial anomalies
CoA (and others)
Eye abnormality
54
Q

Blue dots in RBC indicate

A

Lead poisoning (basophils stippling)

55
Q

T-ALL risk of what kind of relapse

A

testicular

56
Q

Short, eczema, learning difficulties and skeletal dysplasia
Poor feeding, FTT, greasy fatty diarrhoea, risk of infections (mild neutropenia plus or minus low platelet and Hb)

Increased risk marrow aplasia

Syndrome? (Blood, exocrine pancreas failure, growth)

A

Schwachman-diamond syndrome

DDx fanconi anaemia and pearson syndrome

57
Q

Vwd type 1-3

A

1: most common 75% with reduced amounts
2: some not functioning
3: all defective

58
Q

Delayed nausea in what chemo agent

A

Cisplatin

59
Q 
Which one of the following is the most common cause of familial thrombophilia (deep vein thrombosis and 
pulmonary emboli)? 
A. Activated protein C resistance. 
B. Antithrombin III deficiency. 
C. Protein C deficiency. 
D. Protein S deficiency. 
E. Prothrombin gene mutation.
A

A

60
Q 
Which one of the following is least toxic to bone marrow? 
A. Carboplatin. 
B. Cyclophosphamide. 
C. Doxorubicin. 
D. Etoposide (VP-16). 
E. Vincristine.
A

E

61
Q

Bad extravasion with what chemo drug

A

Vincristine

62
Q

NRAS proto-oncogene associated with

A

AML

63
Q

Improved symptoms in spherocytosis from what intervention

A

Splenectomy

64
Q

What drug can trigger haemolytic in G6PD?

A

(Paracetamol NSAIDs)
Antibiotics:
Quinolones - cipro, Nitrofurantoin
Sulpha drugs (cotrimox)

Antimalarials:
Primaquine

65
Q

Haptoglobin very low from

A

Intravascular haemolysis

Or wilsons

66
Q

Causes or prolonged PT

A

Vit k def/liver disease

Deficient 2,7, 10, 5, fibrinogen

67
Q

Pain sickle cell drug

A

Hydroxyurea

68
Q

Methylene blue used to treat what

A

Hereditary methaemoglobinemia

69
Q

Dyskeratosis congenita

A

 Background:
o Multisystem disorder characterized by mucocutaneous abnormalities, bone
marrow failure and a predisposition to MDS and cancer.
o Diagnostic mucocutaneous triad: reticulate skin pigmentation of the upper body,
mucosal leukoplakia, nail dystrophy.
o Aplastic anaemia occurs in 50%.
o X linked recessive inheritance.
 Pathology: shortened telomeres.
 Treatment: androgens and stero

70
Q

Most common inherited risk factor for thrombosis

A

Factor V Leiden
Result of single nucleotide change within the factor V gene. Causes factor Va to become resistant to inactivation by activated protein C.

71
Q

AML Tx

A

multiagent chemotherapy (usually cytarabine based)

72
Q

Favourable features for AML

A

Downs
t(8;21)
t(15;17)
inv(16)
M3 (Acute promyelocytic leukaemia is characterized by a gene rearrangement
involving the retinoic acid receptor (t(15;17); PML-RARA) and is very
responsive to all-trans-retinoic acid combined with anthracyclines and
cytarabine.)

73
Q

Unfavourable AML features

A

monosomy 7 and 5, 5q-, 11q23

74
Q

Imatinib treatment for?

A

CML with BCR-ABL

75
Q

Tumour markers: catecholamine metabolites (homovanillic acid – HVA,
vanillylmandelic – VMA) in urine?

A

Neuroblastoma

76
Q

Alkylsulphonate: busulphan
important SE

A

Pulmonary oedema leading to fibrosis

77
Q

Chemo agent with SE of chemical conjunctivitis

A

Cytarabine

78
Q

Radiation skin reaction from what chemo

A

Daunorubicin and doxorubicin

79
Q

Increased risk secondary cancer (AML)

A

Etoposide

80
Q

Asparaginase: Side effects:

A

 Venous thrombosis.
 Anaphylaxis.
 Pancreatitis.

81
Q

B cell lymphoma primary site

A

B for belly

T for thorax

82
Q

Renal tubular toxicity which drug

A

Ifosfamide

83
Q

Life of a platelet

A

10 days

84
Q

Hb high affinity for o2 (epo and Hb level)

A

Both high

85
Q

Two weeks after transplant present with fluid retention, hyperbilirubinemia, and painful hepatomegaly

A

Veno-occlusive disease (VOD) of the liver remains one of the most feared complications associated with high-dose chemotherapy and hematopoietic stem cell transplantation (SCT)

86
Q

Target cells and pencil cells mean

A

Target mean thalassaemia

Pencil means iron deficiency

87
Q

Atypical teratoid rhabdoid tumour mutation.

A

INI1

88
Q

Tumour from forth ventricle

A

Ependymoma (50% have calcification)

89
Q

Post op complication post fossa op?

A

Posterior fossa syndrome cerebellum mutism where mute and ataxia

90
Q

Most common childhood brain tumour

A

Astrocytoma

91
Q

Spherocytes on blood film indicate (2 things)

A

Autoimmune haemolytic anaemia and hereditary spherocytosis

92
Q

Child presents with otitis media, HSM, lyric skull lesion and diabetes insipidus (drinks lots)
Investigations and diagnosis

A

Investigations show birbeck tennis racket shape granule and Langerin CD207 and CD1a

93
Q

What drug avoid intrathecal?

A

Vincristine

94
Q

Gene deletion in short, mesomelia, madelung deformity?

A

Shox

LERI-WEILL DYSCHONDROSTEOSIS; LWD

95
Q

Sonic hedge role and importance

A

Regulates cell differentiation and proliferation and important in embryo development
Related to Gorlin syndrome (craniofacial and skeletal abnormalities and increased risk medulloblastoma and BCC - gene PTCH1)

96
Q

Child with CD1a positivity, birbeck tennis racket granules and langerin CD207 has?

A

Langerhands cell histocytosis

97
Q

HLH mutation

A

SAP

98
Q

10% on high dose cytarabine chemotherapy get this?

A

Cerebelluar toxicity and ataxia

99
Q

Congenital central hypoventilation syndrome and neuroblastoma gene

A

PHOX2B

100
Q

ALK mutation in?

A

Anaplastic lymphoma kinase
Target for drugs
Neuroblastoma
Lymphoma

101
Q

Alveolar rhabdomyosarcoma genetics?

A

T(1:13), t(2:13)

102
Q

Second most common childhood cancer?

A

Brain tumour

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