Haemotology/Oncology Flashcards by Emily W

Post transplant lymphoproliferative disease related to what medication

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

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How much iron in 250ml blood unit

250mg

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Vincristine and vinblastine mechanism of action

Inhibit mitosis by binding tubulin (minimal myelosuppression) METAPHASE

(Asparginase stops rna into tubulin protein)

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Doxorubicin and doaunorubicin mechanism of action

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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Cyclophosphamide and platinum mechanism of action

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

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Cause of short stature with cranio-spinal radiotherapy

Failure of spinal growth

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Highest incidence of secondary malign

Hodgkin lymphoma (10% at 20y)

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Diamond-Blackfananaemia

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

Fatty infiltrate pancreas

Shwachman syndrome

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Tumour lysis syndrome mx

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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Drugs associated with mucositis

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

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No or minimal BM suppression with which drugs

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

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Haemorrhaging cysitis is SE of which chemo agents

Alkylating agents: Cyclophosphamide / Ifosfamide

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At risk groups for secondary malignancy

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

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Warm autoimmune haemolytic mx

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

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Dyskeratosis congenita (zinsser-Cole-England syndrome)

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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HLH

Hyper inflammation

High TG and high ferritin classic

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Mild haemophilia A and VWD management

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

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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?

Factor 13 deficiency

Defects in fibrinogen

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Medulloblastoma predisposed conditions

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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Medulloblastoma groups

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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ATRT (atypical teratoid rhabdoid tumour
Poor prognosis
Supratentorial

• 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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Ependymoma mx

Surgery and focal radiation

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High grade glioma

Small round blue cell tumours

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

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Craniopharyngioma MRI findings

Suprasellar Calcification Cysts

TS cancer type

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

Juvenile Pilocystic astrocytoma mx

Observe

Common late effect after brain tumour

GH deficiency

AML related to which syndrome

Downs (<3y, better survival and ALL worse survival) Familial RUNX1 mutations Aplastic anaemia Myelodysplastic syndrome Myeloproliferative disorders

Extramedullary haematopoesis (skin) think..

AML

AML morphology

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

CD numbers for B and T cell ALL

``` B = 19 T = 3 ``` T call ALL overall higher risk and presentation WCC not related to risk

Which cancer has Reed-Sternberg cell EBV associated

Hodgkin lymphoma

Radiotherapy in ewings or osteosarcoma

Ewings

Mets of wilms, ewings, osteosarcoma to

Lungs

What tumours can metastasise to marrow

Alveolar Rhabdomyosarcoma can spread to bone marrow | Ewings

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

Neuroblastoma

Poor prognostic factors in neuroblastoma

Combine clinical features (Metastases, unresectable big ugly primary, age > 18 mths are bad). And lab tests (histology, MYCN amplification 8-200+ even if local disease, 11q missing are bad).

Can neuroblastoma regress by itself

Yes

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

Neuroblastoma

Hepatoblastoma predispositions and mx

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

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

10% | 8 years

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

PTEN syndrome

What is a nephrogenic rests

Embryonal reminent and may develop into nephroblastoma,

AFP is increased in...

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

Highest risk factor for invasive fungal disease

Prolonged neutropenia

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

Invasive pulm aspergillosis | Voriconazole

High risk neuroblastoma can be treated with

anti-GD2 (ch14.18)

Small round blue cells in which cancers

LERRNN | Lymphoma, ewings, rhabdomyosarcoma, retinoblastoma, nephroblastoma, neuroblastoma

Hallmark of lanherhans cell histocytosis

Tennis racket granule (birbeck granule)

Wide radiotherapy in female most common secondary cancer is

Breast

Emancipation in infant cause?

Diencephalic syndrome (hypothalamus and thalamus lesion)

PHACE syndrome stands for

``` Posterior fossa malformation Haemangiomas Arterial anomalies CoA (and others) Eye abnormality ```

Blue dots in RBC indicate

Lead poisoning (basophils stippling)

T-ALL risk of what kind of relapse

testicular

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)

Schwachman-diamond syndrome | DDx fanconi anaemia and pearson syndrome

Vwd type 1-3

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

Delayed nausea in what chemo agent

Cisplatin

``` 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. ```

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

Bad extravasion with what chemo drug

Vincristine

NRAS proto-oncogene associated with

AML

Improved symptoms in spherocytosis from what intervention

Splenectomy

What drug can trigger haemolytic in G6PD?

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

Haptoglobin very low from

Intravascular haemolysis | Or wilsons

Causes or prolonged PT

Vit k def/liver disease | Deficient 2,7, 10, 5, fibrinogen

Pain sickle cell drug

Hydroxyurea

Methylene blue used to treat what

Hereditary methaemoglobinemia

Dyskeratosis congenita

 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

Most common inherited risk factor for thrombosis

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.

AML Tx

multiagent chemotherapy (usually cytarabine based)

Favourable features for AML

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.)

Unfavourable AML features

monosomy 7 and 5, 5q-, 11q23

Imatinib treatment for?

CML with BCR-ABL

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

Neuroblastoma

Alkylsulphonate: busulphan important SE

Pulmonary oedema leading to fibrosis

Chemo agent with SE of chemical conjunctivitis

Cytarabine

Radiation skin reaction from what chemo

Daunorubicin and doxorubicin

Increased risk secondary cancer (AML)

Etoposide

Asparaginase: Side effects:

 Venous thrombosis.  Anaphylaxis.  Pancreatitis.

B cell lymphoma primary site

B for belly | T for thorax

Renal tubular toxicity which drug

Ifosfamide

Life of a platelet

10 days

Hb high affinity for o2 (epo and Hb level)

Both high

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

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)

Target cells and pencil cells mean

Target mean thalassaemia | Pencil means iron deficiency

Atypical teratoid rhabdoid tumour mutation.

INI1

Tumour from forth ventricle

Ependymoma (50% have calcification)

Post op complication post fossa op?

Posterior fossa syndrome cerebellum mutism where mute and ataxia

Most common childhood brain tumour

Astrocytoma

Spherocytes on blood film indicate (2 things)

Autoimmune haemolytic anaemia and hereditary spherocytosis

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

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

What drug avoid intrathecal?

Vincristine

Gene deletion in short, mesomelia, madelung deformity?

Shox | LERI-WEILL DYSCHONDROSTEOSIS; LWD

Sonic hedge role and importance

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)

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

Langerhands cell histocytosis

HLH mutation

SAP

10% on high dose cytarabine chemotherapy get this?

Cerebelluar toxicity and ataxia

Congenital central hypoventilation syndrome and neuroblastoma gene

PHOX2B

ALK mutation in?

Anaplastic lymphoma kinase Target for drugs Neuroblastoma Lymphoma

Alveolar rhabdomyosarcoma genetics?

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

Second most common childhood cancer?

Brain tumour

Haemotology/Oncology Flashcards

(102 cards)