Heme/Onc

Question 1

Diagnosis of von Willebrand

Answer 1

vWF antigen
vWF activity/ristocetin cofactor
- Normal > 50%
- Abnormal < 30%
- 30-50% is borderline
Factor VIII

Prolonged PTT in some with factor 8 problems

Question 2

von Willebrand Disease (Inheritence and Px)

Answer 2

autosomal dominant
mucosal bleeding

Question 3

Wiskott Aldrich

Answer 3

Immunodeficiency, Eczema, thrombocytopenia

immunoglobulin problems: Elevated IgA and IgE (WEEskott AAAAldrich) & low IgM and IgG)

Question 4

Long term chemo complication f/u

Answer 4

Doxo/Radiation - ECHO q2-5 years based on anthracycline dose and radiation dose
Bleomycin - baseline PFTs, then PRN
Prednisone - bone density, annual eye exam
Vincristine - annual exam for peripheral neuropathy

Question 5

Post radiation screening

Answer 5

PFTs - baseline then prn
ECHO q2-5 years
TSH, T4 annually

Question 6

Ataxia, nystagmus and muscle jerks

Answer 6

neuroblastoma
Urine HVA/VMA to diagnose

Question 7

Lymphoma

Answer 7

B symptoms, supraclavicular node, pancytopenia, risk of SVC syndrome from mediastinal mass, do a CXR first to r/o mediastinal mass before CT so they don’t die in the scanner

Question 8

Hemoglobin electrophoresis

Answer 8

FA = Normal
FS = sickle cell disease
FAS = sickle cell trait
FSA = sickle cell-beta thalassemia

0% HBA = sickle cell disease
50% HBA/HBS = trait

Question 9

stroke in SCD

Answer 9

Screening
Annual TCD ultrasounds (age 2 to 16) to identify high-risk patients

Acute management
Maintain high index of suspicion, evaluate with CT scan +/- MRI
In children, AIS is more common than hemorrhagic, but either may occur
Exchange transfusion for acute stroke to get HbS < 30%

Prevention
Strong evidence for chronic transfusions for primary and secondary prevention

Question 10

Mentzner index

Answer 10

Mentzer index = MCV/RBC

<13 = thalassemia (marrow produces normal number of cells (RBC) but MCV small)
(thal-LESS-semia)

> 13 = Iron Def Anemia (marrow can’t produce as many cells (low substrate) so MCV AND RBC low)

Question 11

treatment of vWD Type 3

Answer 11

treatment with a virally attenuated, VWF-containing concentrate (Humate P)

Cryoprecipitate can be used if not a baby (it has vWF and factor 8 but is not virally attenuated)

Question 12

leukemia prognosis predictors

Answer 12

Age - <1yr or >10yr = worse outcomes
Concentration of circulating blasts (WBC count >50 = worse outcomes)
Philadelphia chromosome (t9;22)
– occurs in 3-5% of kids with ALL
– translocation site involves abl proto-oncogene with production of an abnormal fusion protein (bcr abl) that has tyrosine kinase activity
– worse response to conventional chemo
– tyrosine kinase inhibitor imatinib – – actively binds to bcr abl fusion protein
JAK kinase
– greater freq of this mutation in high risk ALL
– worse treatment outcomes

Question 13

An 11 month old presents with a scaly rash all over, especially in the diaper area. He also has exopthalmos and HSM. Xrays show bony lucencies on the scalp. What is the likely diagnosis?

Answer 13

langerhans cell histiocytosis (LCH)

• bone: bony lucencies
• skin (difficult-to-treat scaly, papular, seborrheic dermatitis of the scalp, diaper, axillary, or posterior auricular regions)
• hent: exopthalmos
• endo: DI
• lymphadenopathy, HSM
• pulm infiltrates

Question 14

diagnosing LCH

Answer 14

Tissue biopsy = diagnostic → easiest to perform on skin or bone lesions

Other studies to be done in all patients: CBC, LFTs, coag, skeletal survey, chest XR, and measurement of urine osmolality

Question 15

Opsoclonus myoclonus syndrome (OMS)

Answer 15

paraneoplastic syndrome characterized by rapid multidirectional eye movements, involuntary muscle spasms, and irritability. Thought to be secondary to autoimmune reaction

Question 16

transient myeloproliferative disease

Answer 16

Trisomy 21
high leuks, blasts in the peripheral blood, associated anemia, thrombocytopenia, and hepatomegaly.

don’t need bone marrow - do flow cytometry

kids with T21 should be screened with CBC + diff + smear at birth to evaluate for TMD

No treatment if low risk as most spontaneously resolve.

Indications to treat: hyperleukocytosis (WBC>100), severe liver dysfunction, hydrops fetalis, cardiac effusions, and DIC.
Low dose 7 days cytarabine arabinoside; high risk toxicity

Question 17

Questions about heparin insensitivity

Answer 17

= antithrombin 3 deficiency

Question 18

Ewing Sarcoma
XR and BMA findings

Answer 18

Eww, onions, Eww moths (moth eaten lytic appearance), Eww cancer in my axial bones. Bilateral BMA and biopsy shows small round blue cell tumor

Question 19

Osteosarcoma

Answer 19

XR: Sunburst O like the sun

tumors at the ends of long bones near the physis

Question 20

Leukemia PEARLS

Answer 20

ulcerative mucositis, pancytopenia, HSM

Question 21

Vaccines in SCD

Answer 21

13-valent pneumococcal conjugate and polysaccharide vaccines against Streptococcus pneumoniae

Both conjugated quadrivalent meningococcal (A,C,W,Y) and serogroup B vaccines targeting Neisseria meningitidis

Extra booster dose against Haemophilus influenzae type B (Hib), immunization against hepatitis A and B, and annual influenza vaccines are all recommended.

In the context of travel, vaccination against Salmonella typhi and malaria prophylaxis should be offered

Question 22

tumor lysis syndrome

Answer 22

high potassium
high phosphate
high urate
low calcium

Treatment for TLS:
- hyperhydration
- rasburicase (neutralizes the high urate)
- urine alkalinization

Question 23

Leukemia Presentation

Answer 23

Cytopenias (Normal or high WBC but neutropenia)
Bulky disease (HSM, LNs, mediastinal mass, bony pain, leukemia cutis)
Sanctuary sites (CNS, Testicular - must look)
Constutional (Wt loss >10%, drenching night sweats, fever)

Question 24

Leukemia investigations

Answer 24

CBC, smear
tumor lysis labs
coags
MUST GET CXR
LP, Bone marrow aspirate

Question 25

High risk ALL

Answer 25

Young or old (<1 or >10)
WBC > 50
CNS or testicular disease
unfavourable cytogenetics (ex. philadelphia)
poor response to induction chemo

Question 26

ALL Treatment

Answer 26

chemotherapy alone almost all cases (rarely radiation) and usually over 2.5 yrs
HSCT only transplant for relapse

Question 27

AML Treatment

Answer 27

shorter treatment of chemo and may get stem cell up front
typically worse outcomes than ALL

Question 28

LN red flags

Answer 28

> 2cm, supraclavicular, non-tender, fixed, firm/rubbery/matted, HSM

Question 29

young child with diffuse lymphadenopathy

Answer 29

more likely reactive/viral unelss red flags

if teenager think hodgkin lymphoma

do not give steroids to these kids until tissue is obtained

Question 30

Supraclavicular lymph node

Answer 30

NEED biopsy (this is the best test)
should get CXR to look for mediastinal mass
dont do steroids

Question 31

Most likely cancer in teenagers

Answer 31

most likely lymphoma

Question 32

ataxia, gait change, increased ICP and torticollis

Answer 32

think posterior fossa tumors

Question 32

Diencephalic syndrome

Answer 32

happy kid that is cachectic with failure to thrive

Question 33

What age to start hydroxyurea in SCD

Answer 33

9months of age

Question 33

What age do pts with SCD need abx prophylaxis

Answer 33

2mo (spleen involutes at 3mo) - 5 years

Question 34

Vincristine Side Effects

Answer 34

Foot drop
Vocal cord dysfunction
Jaw Pain
Sensory Neuropathy

Question 35

Bleomycin Side effects

Answer 35

Pulmonary Fibrosis

Question 36

Cyclophosphamide Side Effects

Answer 36

Hemmorhagic Cystitis
SIADH
Myelosuppression
Nausea

Question 37

Cytarabine (Ara C) Side Effects

Answer 37

Conjunctivitis
Fever
Rash

Question 38

Methotrexate Side Effects

Answer 38

Mucositis
Myelosuppression
Transaminitis

Question 39

Doxorubicin Side Effects

Answer 39

Dialated Cardiomyopathy (need echo q2-5 years)
Red secretions
Mucositis

Question 40

Cisplatin Side Effects

Answer 40

Metal taste in mouth
Ototoxicity
Nephrotoxicity