Heme/Onc

Question 0

14 yo boy with painful swelling of mid-femur. Multiple layers of periosteal bone formation.

Answer 0

Ewing Sarcoma

Question 1

16 yo with painless firm mass at proximal tibia. What radiology findings? And when is this the most common secondary malignancy?

Answer 1

Osteogenic Sarcoma. XR findings are sunburst pattern and codman triangle. Most common secondary malignancy in retinoblastoma.

Question 2

Li Fraumeni syndrome has increased incidence of what cancer

Answer 2

Osteogenic sarcoma

Question 3

12 yo boy with unilateral thigh pain at night. Relieved by NSAIDs. X-ray findings? Tx?

Answer 3

Osteoid osteoma. XR findings are central radiolucent area surrounded by sclerotic bone. Tx is supportive care

Question 4

6 yo girl with cranial nerve palsies, ataxia, head tilt

Answer 4

Brainstem glioma

Question 5

Poor prognostic signs for LCH

Answer 5

Age < 2, male gender, organ involvement

Question 6

Most frequent secondary malignancy in Hodgkin survivors

Answer 6

AML

Question 7

ALL poor prognostic signs

Answer 7

Age 10, WBC >50,000, steroid tx, hypodiploidy, MLL rearrangement t(4;11)

Question 8

What cancer to think about in a 16 yo otherwise healthy male with isolated splenomegaly. Treatment?

Answer 8

CML; txt is BMT

Question 9

Auer rods, leukoerythroblastic reaction (nRBC). Is there testicular involvement?

Answer 9

AML; No testicular involvement

Question 10

Philadelphia chromosome t(9;22). BCR-abl gene. Associated with what cancer? Tx?

Answer 10

CML; tx with imatinib (tyrosine kinase inhibitor)

Question 11

Teen with non-tender cervical or supraclavicular node. What is it and what do you see on bx

Answer 11

Hodgkin lymphoma; Reed-Sternberg cells

Question 12

6 yo boy with RLQ mass, pain, and LAD below the diaphragm. Negative px indicators?

Answer 12

Burkitt’s lymphoma; neg px: CNS involvement, BM involvement, high LDH > 1000

Question 13

Cancer associated with intussusception

Answer 13

Burkitt’s lymphoma

Question 14

T(8;14) c-myc oncogene

Answer 14

Burkitt’s lymphoma

Question 15

Serum markers for Hodgkin’s disease

Answer 15

ESR and serum copper

Question 16

Most common tumor in infants? Px factors?

Answer 16

Neuroblastoma, px good for age <18 mos, stage localized, histopathology, hyperdiploidy

Question 17

2 yo black girl with painless abdominal mass and hematuria. Prognostic factor that is most important?

Answer 17

Wilm’s tumor; histology (bad=clear cells! a aplastic cells)

Question 18

Child with constipation and rectal mass

Answer 18

Rhabdomyosarcoma

Question 19

What factors shift the oxygen dissociation curve to the right (higher affinity)?

Answer 19

2,3 DPG, acidosis, hypoxia, fever

Question 20

Buchanans rule to determine Hgb

Answer 20

11+ (age in yrs x 0.1)

Question 21

Buchanans rule to determine MCV

Answer 21

70 + (age in years)

Question 22

Hair on end appearance of skull X-ray

Answer 22

Beta thalassemia; indicates extra medullary hematopoiesis

Question 23

8 yo girl with repeated episodes of pallor, jaundice, and hemolytic anemia that is hyperchromic with high MCHC

Answer 23

Hereditary spherocytosis

Question 24

2 yo boy with recent viral illness with pallor, normochromic normocytic anemia with low retic count, normal WBC and platelets

Answer 24

Transient erythroblastopenia of childhood

Question 25

School age child with macrocytic anemia, mild to moderate thrombocytopenia, leukopenia, and upper limb abnormalities, short stature. How do you diagnose?

Answer 25

Fanconi's anemia, dx with chromosome breakage study

Question 26

Infant with normochromic, macrocytic anemia, reticulocytopenia, triphalangeal thumbs, short stature. How do you dx? How to treat?

Answer 26

Diamond blackfan anemia. Dx with elevated RBC ADA. Tx with steroids

Question 27

5 mo old with recurrent skin infections, hypo pigmentation of skin and hair. Inheritance? Dx?

Answer 27

Chediak-Higashi. Autosomal recessive. Giant neutrophil granules on smear

Question 28

Infant with recurrent severe infections, omphalitis, and neutropenia

Answer 28

Kostmanns Syndrome (Severe congenital neutropenia)

Question 29

Impaired wound healing or delayed separation of umbilical cord, with high WBC in absence of infection. Tx?

Answer 29

Leukocyte adhesion deficiency. Tx BMT

Question 30

Most common pathogen in cyclic neutropenia? Inheritance?

Answer 30

C. Perfringens. Autosomal dominant

Question 31

Pancreatic insufficiency, short stature, recurrent infections and neutropenia. Also with clinodactyly or syndactyly

Answer 31

Shwachman-Diamond Syndrome

Question 32

Baby boy with bad diaper rash, bruising, frequent ear and lung infections. Small platelets. Inheritance? Tx?

Answer 32

Wiskott-Aldrich. X-linked ( only in boys). Tx is BMT

Question 33

Infant with massive GI bleeds and family history of similar episodes. What do the PT, PTT, and bleeding time look like? Tx?

Answer 33

Glanzmann's thrombasthenia which is AR. PT, PTT are normal. Bleeding time prolonged. Tx with platelet transfusions.

Question 34

Infant with bleeding, normal coags, prolonged bleeding time, and giant platelets

Answer 34

Bernard-Soulier

Question 35

Newborn with petechiae, wet purpura, and platelet count of 1000. Mom with normal platelets. Tx?

Answer 35

Neonatal alloimmune ITP. Tx with maternal platelets

Question 36

ITP look a like with HSM

Answer 36

Think leukemia!

Question 37

Mucosal bleeding with positive family history, normal coags and prolonged bleeding time. Inheritance? Tx?

Answer 37

Von Willebrand disease. Autosomal dominant. Tx severe bleeding with factor 8. If unavailable, cryoprecipitate. Mild bleeding with DDAVP

Question 38

What coags are abnormal in hemophilias?

Answer 38

PTT is prolonged

Question 39

Most common complication in hemophilia?

Answer 39

Chronic hemarthroses leading to joint destruction

Question 40

Massive DIC at birth with family history of clotting. Tx?

Answer 40

Protein C or S deficiency causing neonatal purpura fulminans. Tx FFP, heparin while starting warfarin

Question 41

Recurrent severe VTE, heparin fails to anticoagulate.

Answer 41

Antithrombin III Deficiency. Tx warfarin lifelong

Question 42

Late onset thromboses, most common cause

Answer 42

Factor V Leiden

Question 43

Most common form of sickle cell disease?

Answer 43

FS

Question 44

Sickle cell trait

Answer 44

FAS

Question 45

When to start PCN ppx in sickle cell disease

Answer 45

2 months