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Heme/Onc Flashcards

1
Q

Onion peel - destruction of bone and concentric layers of subperiosteal new bone. Malignancy?

A

Ewings Sarcoma

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2
Q

Where is Ewings sarcoma typically located?

A

Diaphysis (common in shaft of femur, tibia or humerus)

Note: also pelvis

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3
Q

Evidence of new bone formation under the corners and well marked radiating spicules of new new bone within the tumor

A

Osteosarcoma (classically “sunburst” appearance)

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4
Q

“Soap bubble” appearance with min or no sclerosis

A

Osteoclastoma

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5
Q

When does sickle cell disease typically first present and with what?

A

Around 6 months of age with dactylitis

Note: they overall typically have short stature

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6
Q

Male child with a unilateral testicular swelling, what is the most likely malignancy?

A

Leukaemia - rare presentation of acute leukaemia in boys. 10 - 30% of boys have testicular involvement at diagnosis. Other areas outside of marrow are CNS and then even more rarely the eye and kidneys

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7
Q

What type of anaemia is seen in chronic lead poisoning? What are other classic features?

A

Microcytic anaemia

Other features: developmental delay, constipation, emesis

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8
Q

Treatment of chronic lead poisoning?

A

Removing source + chelation

Mild: oral D-penicillamine

Severe: IV EDTA

Very severe: IM injections of dimercaprol (to increase effect of EDTA) + IV EDTA

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9
Q

Xray features of Langerhans cell histiocytosis

A

Osteolytic lesions often in skull, long bones, spine and pelvis

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10
Q

Beta-thalassaemia features on exam?

A

Frontal bossing, hepatosplenomegaly. Does not present until 1 yr old or older

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11
Q

What coag value is abnormal in Haemophilia A

A

Elevated aPTT (plts, PT and fibrinogen are normal)

Note: factor VIII def; haemophilia B is factor IX

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12
Q

What is the inheritance of G6PD deficiency?

A

X linked recessive (affects homozygous females and hertozygous males)

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13
Q

4 clinical syndromes in G6PD

A
  1. Drug sensitivity haemolytic anaemia
  2. Favism (ingestion of beans or inhalation of pollen)
  3. Neonatal jaundice
  4. Congenital non spherocytic haemolytic anaemia
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14
Q

How does Leydig cell tumour usually present?

A

Early puberty in males

Occasionally testicular mass or nodule

Note: 75% of cases are benign

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15
Q

Criteria for admission in a patient with ITP? Outpatient management plan?

A

Admission and active treatment if mucosal bleeding (lips, tongue, gum, urine or bowel) - if mucosal bleeding there may be an increased risk of intracranial bleeding

Outpatient: daily review and FBC

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16
Q

Urinary catecholamines (VMA or HVA) are elevated in the majority of cases of neuroblastoma T/F

A

T

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17
Q

All children with ALL are treated with intrathecal MTX T/F

A

T - this is to reduce the risk of CNS disese

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18
Q

Classification of haemophilia?

A

By % factor deficiency

Mild: > 5% of 35% of normal

Mod: 1% to 5% of normal

Severe: 0%

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19
Q

What is the most life saving intervention in the mgmt of acute chest in sickle cell disease?

A

Oxygen

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20
Q

Where does medulloblastoma usually occur?

A

Cerebellum - usually vermis and sometimes hemispheres

Note: Usually seen in males 5-7 yrs

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21
Q

Chronic leg ulceration is common in what anaemia?

A

Sickle cell anaemia

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22
Q

In the prognosis of neuroblastoma what age differenciates?

A

>18 months old has worse prognosis

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23
Q

N myc amplification is assoc with a poor prognosis in neuroblastoma T/F

A

T - particularly poor prognosis and rapid progression

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24
Q

Most common cause of aplastic crisis in a pt with HbSS

A

Pavovirus B19 infection

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25
Q

In sickle cell disease which patients are more susceptible to more severe retinal disease?

A

HbSC (compared to HbSS)

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26
Q

Bleeding time is increased in haemophilia and VonWB. T/F

A

F - normal in haemophilia A and increased in B and in VonWB

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27
Q

Fever is common at presentation in osteosarcoma T/F

A

F - fever is common at presentation in Ewings sarcoma

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28
Q

What is the most common cause of pancreatitic insufficiency after CF?

A

Schwachman-Diamond Syndrome

Note: this is the 3rd most common cause of congenital bone barrow failure after Fanconi and Diamond-Blackfan anaemia. But this two do not cause panceatitic insufficiency

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29
Q

What lab value is low in tumor lysis syndrome?

A

Calcium

Note: other abnormalities are high - k, phos and uric acid

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30
Q

Male sex is a good prognostic sign for ALL T/F

A

F - female sex is a good prognostic sign

Other good prognostic signs: age > 2 yrs at presentation; normal WBC; normal chest xray; common cell type, hyperdiploid karotype

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31
Q

Petechiae is common manifestation of haemophilia T/F

A

F - petechiae are a feature of platelet disorders and haemophilia is a factor disorder

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32
Q

Factor 8 inhibitors occur in about 20% of patients with haemophilia reciveing multiple factor 8 transfusions T/F

A

T

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33
Q

What % of Wilms tumor are bilateral?

A

10%

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34
Q

What is the most common presentation of Wilms tumor?

A

Abdominal mass

Note: 30-40% may have pain ; they may also have hypertension, haematuria, anaemia and fever secondary to tumor necrosis

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35
Q

Fanconi anaemia is assoc with what malignancies and MSK features?

A

AML and solid tumors (esp head and neck SCC, hepatic and gynae)

MSK: triangular shaped head, radius and thumb dysplasia/aplasia

Note: other features - deafness, aplastic anaemia, cardiac issues, GI and kidney malformations, hypogonadism

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36
Q

What is the inheritance of G6PD deficiency?

A

X linked

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37
Q

T cell phenotype is a poor prognostic feature in ALL T/F

A

T

Other poor prognostic features: CNS disease, male sex, age < 1 or > 10, Philadelphia chromosome, WBC > 50, African American or Hispanic

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38
Q

What is Evans syndrome?

A

ITP and autoimmune haemolytic anaemia

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39
Q

When are steroids indicated in the treatment of ITP?

A

If platelets < 30 x10 or < 50 + bleeding

Note: if > 30 and no bleeding just frequent monitoring; high dose immunoglobulin may also be used

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40
Q

Pulmonary hypertension is a complication of what haem disorders?

A

Haemolytic anaemia including sickle cell anaemia

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41
Q

Decreased platelet activation to ristocetin is classically seen in what disease?

A

Von Willebrand’s disease

Note: factor VIII is carried by VBW factor and so hence it is also redcued

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42
Q

Bleeding time is increased in which haemophilia?

A

Haemophilia B (factor IX)

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43
Q

Characteristic blood film findings in Bernard-Soulier disease

A

Giant platelets and thrombocytopenia

Note: caused by deficiency of glycoprotein Ib (GpIb), the receptor for von Willebrand factor.

44
Q

How is TRK levels related to prognosis of neuroblastoma?

A

High TRK gene levels are associated with a better prognosis

45
Q

Raised LDH, neurone specific enolase and ferritin is associated with a better or worse prognosis in neuroblastoma?

A

Worse prognosis

46
Q

Abnormalities on what Chr are associated with Wilms tumor?

A

Microdelations on short arm of chr 11

(eg Beckwith Wiedmann and WAGR syndrome)

47
Q

Long term use of heparin can lead to what MSK side effect?

A

Osteoporosis

48
Q

What adjustment needs to be made to vaccination when a patient is on warfarin?

A

Give vaccines subQ instead of IM to decrease bleeding

49
Q

What nail finding is characteristic of Fe deficiency anaemia and rarely seen in other forms of anaemia?

A

Koilonycia (spoon shaped nailed)

50
Q

Splenomegaly is found in about 25% of cases of Fe deficiency anaemia T/F

A

F - mild splenomegaly seen in about 10%

51
Q

Which is more common in children Hodgkin’s or non Hodgkin’s lymphoma?

A

Hodgkins

52
Q

Features to help differenciate Wilms tumor from neuroblastoma?

A

Wilm tumor (nephroblastoma) : appear well, no irritability or signs of bone marrow infiltration

Neuroblastoma: unwell, irritable, often presents late with metastatic disease esp to bone and can have anaemia secondary to bone marrow infiltration. May have metabolic effects from catecholamines

53
Q

What is the mechanism of action of LWMH?

A

Accelerates antithrombin dependent inactivation of factor Xa

It does this by binding to antithrombin causing a conformational change.

54
Q

Typical lab findings in DIC

A

Elevated d dimer

Decreased plts and fibrinogen

Elevated or normal PT/aPTT

55
Q

Compare typical presentation and age for Hodgkin’s vs non Hodgkin’s lymphoma?

A

Hodgkins’s: slow growing painless lymphadenopathy, typically teenage or young adult

Non Hodgkin’s: rapidly enlarging LN mass, often unwell and high risk for tumor lysis, peak 7-10 years

56
Q

What is the most common site of non Hodgkin’s lymphoma?

A

Abdomen

57
Q

What is the most important prognostic marker in AML?

A

Bone marrow karyotype

t(8,21) or chr 16 inversion = low risk relapse

Changes in chr 3,5, or 7 = high risk of relapse

58
Q

A patient who presents with a PE and is unable to become therapeutic on heparin infusion is suggestive of what disorder?

A

Antithrombin III deficiency

Antithombin is a natural anticoagulant –> deficiency lead to emboli

Furthermore heparin relies on an adequate level of antithrombin to be effective

59
Q

What monoclonal antibodies can be used in the treatment of resistent ITP? What screening needs to be done prior to initiation?

A

Rituximab

Hep B screening

60
Q

4 classic symptoms of a phaechromocytoma

A

Severe HTN

Headaches

Palpitations

Diaphoresis

61
Q

What are the clinical features of cyclic neutropenia?

A

Fever, malaise, lymphadenopathy and mouth sores. Symptoms occur roughly every 21 ones and last 3-10 days.

Note: neutrophil nadir is < 200

62
Q

2 side effects of L-asparaginase

A

Flu like symptoms

Neuro: sleepiness, agitation, hallucinations, seizure

Note: less commonly pancreatitis

63
Q

What is the most common site of met of Wilms tumor?

A

Lung

64
Q

When is the peak incidence of ALL

A

2 to 5 yrs

65
Q

Orbital proptosis and ecchymosis may be seen in which malignancy?

A

Neuroblastoma - due to periorbital mets

66
Q

What paraneoplastic features can be seen with neuroblastoma?

A

Diarrhoea (due to vasoactive intestinal peptide)

Opsoclonus myoclonus syndrome

67
Q

What is the most common site of osteosarcoma and Ewings sarcoma

A

Osteosarcoma - distal femur (xray “sunburst”)

Ewings - pelvis (xray “moth eaten”)

68
Q

How does non endemic Brukitt lymphoma usually present?

A

Abdo distension and ascities

Note: can also present with intussecpetion

69
Q

How does endemic Burkitts lymphoma usually present?

A

Jaw or facial bone tumor with local and distal spread. One feature of local spread is inappropriate loosening of teeth

70
Q

NHL is more common in males or females?

A

Males (3:1)

71
Q

EBV is found in about 50% of Burkitt NHL in the US T/F

A

F - found in about 15 -20%

72
Q

Most causes of NHL are high grade diffuse neoplasms

A

T

(for paediatric cases)

73
Q

Serious long term risk individuals treated with etoposide?

A

Leukaemia

74
Q

Cutaneous findings associated with Langerhans cell histiocytosis

A

Haemorrhagic crusted papules and papulovesicles

Recalcitrant seborrheic dermatitis like erruptions in association with lymphadenopathy

Note: also can present with diabetes insipitus; mastoid bone lesions can lead to chronic recurrent ear infections

75
Q

x ray findings in osteoid osteoma?

A

Radiolucent sharp rounded lesion < 2cm diameter, surrounded by sclerosis

Note: proximal femur is most common location

76
Q

Birbeck granules are found in what pathology

A

Langerhan’s cell histiocytosis

Note: they are intracytoplasmic inclusion bodies that appear as rod or tennis racquet shaped structures

77
Q

What are the most common complications of sickle cell trait?

A

Renal papillary necrosis

Hypoosthenuria (inability to concentrate urine)

Note: it is unusual for those with sickle cell trait to have complications of sickle cell disease

78
Q

Hydroxyurea tx for sickle cell disease results in a decrease in HbF. T/F

A

F - it results in an increase in HbF, causing a decrease in HbS.

Note: side effect is decreased plts and leukocytes

79
Q

Widening of diploic spaces on MRI is seen in what anaemia?

A

Thalassemia major

80
Q

Xray femur showing sclerosis, resulting in “bone within bone appearance” said to reflect old cortex within new cortex is associated with beta thalasaemia T/F

A

F - associated with sickle cell disease

81
Q

Features of thrombotic thrombocytopenic purpura?

A

Classically presents with 5 findings:

Low plts

microangiopathic haemolytic anaemia

neurologic symptoms

renal failure

fever

Note: tx is plasampharesis and steroids. Caused by antibodies against ADAMTS13 (a protease that breaks down ultralarge vWF multimers)

82
Q

When does chronic benign neutropaenia usually present?

What is the pathophysiology?

A

Usually diagnosed around 8 - 11 months and lasts about 2 yrs

Autoantibodies to granulocytes

83
Q

Children with chronic benign neutropaenia are at increased risk for bacterial and fungal infections T/F

A

F

Note: bone marrow will show a normocellular marrow with normal trilineage haematopoesis

84
Q

Ewings sarcoma is more common in what race?

A

Caaucasians

85
Q

Hypersegmented neutrophils are classic findings of what 2 anaemias?

A

Megaloblastic anaemia due to B12 or folate def

86
Q

What is the differential diagnosis for an isolated prolonged PT?

A
  1. Early liver failure
  2. Early/mild vit K def
  3. Early DIC
  4. Factor 7 deficiency
  5. Factor 7 inhibitor
87
Q

Name a pathology that causes thrombocytopaenia in which the platelets are small

A

Wiskott-Aldrich syndrome

88
Q

What is Kasabach-Merritt AKA?

A

Haemangioma thrombocytopaenia syndrome

Note: complication of a large haemangioma due to localised intravascular coagulation and hypofibrinogenaemia

89
Q

Microcytic anaemia and increased Hb A is seen in what pathology?

A

Beta thal trait

90
Q

Child presents with morning headaches, emesis, bitemporal hemianopsia and sleep issues. What malignancy?

A

Craniopharyngioma

Most common benign paediatric brain tumor. Also associated with panhypopit; can present with growth failure

91
Q

What is seen on the blood smear in ITP?

A

Large platelets (megathrombocytes)

92
Q

Drugs that can preciptate haemolysis in G6PD deficiency?

A

Anti malarials

Vit K analogs

Aspirin

Nitrofuratoin

Chloramphenicol

93
Q

Risk factors for hepatoblastoma?

A

Beckwith-Wiedemann syndrome

History of permaturity

Note: alpha feto protein is typically elevated

94
Q

What is the most common primary liver malignancy in children?

A

If < 3 yrs hepatoblastoma

If > 3 yrs hepatocellular carcinoma (hep B is an RF)

Note: both may have elevation of AFP

95
Q

Lead poisoning is associated with what levels of Zn and erythrocyte protoporphyrin respectively?

A

Both will be elevated

96
Q

Quick way to differenciate between iron def and thalasaemia on labs results?

A

Both are microcytic (eg decreased MCV)

In Fe def RBC count is low

In thalasaemia RBC count is high or normal

97
Q

Macrocytic anaemia in an 3 month old with absence or marked deficiency of RBCs in an otherwise normal cellular bone marrow

A

Diamond Blackfan anaemia

98
Q

What is the treatment of Diamond Blackfan Anaemia

A

Steroids

99
Q

Elevated levels of fetal Hb and erythrocyte adenosine deaminase are typical in what anaemia?

A

Diamond Blackfan Anaemia

100
Q

Diamond blackfan anaemia vs transient erythroblastopenia of childhood

A

DBA: <1 yr old; more significant anaemia and may have congenital abnormalities (such as absent thumbs)

TEC: usually 1-4 yrs old

101
Q

Light colored hair, small “snubbed” nose, hypertelorism and a thick upper lip. What pathology?

A

Diamond Blackfan Anaemia

Note: RBCs are typically normocytic and macrocytic without associated hypersegmented neutrophils. Plus and CBC typically normal

102
Q

What is Kostmann syndrome AKA

A

Severe congenital neutropenia

Note: most pts never have an ANC > 200; bone marrow shows deficit in myeloid cell maturation, specifically promyelocytic arrest. Compare to chronic benign neutropaenia which has normal trilinear precursors and granulocytopaenia

103
Q

Heinz bodies and bite and blister cells are seen in what pathology?

A

G6PD deficiency

Note: Heinz bodies are small round inclusions w/in the body of the RBC

104
Q

Acanthocytes are usually seen in what type of disease?

A

Liver disease

Post splenectomy

Hypothyroidism

105
Q

In additional to hereditary spherocytosis what disease are spherocytes seen in?

A

AI haemolytic anaemia

106
Q

Classic trial of paroxysmal nocturnal hemoglobinuria?

A
  1. Pancytopaenia
  2. Haemolytic anaemia
  3. Aterial and venous thromboses

Note: PIGA gene (on x chr) abnormality is one cause

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