8 - Pediatric Hematology/Oncology

Question 1

Progressive decline in Hgb during the 1st week of life that persists for 6-8 weeks

Answer 1

Physiologic anemia of infancy

Question 2

Minimal Hgb levels in physiologic anemia of prematurity

Answer 2

7-9g/dL

Question 3

Microcytic anemia causes

Answer 3

Thalassemia
Anemia of chronic disease
Iron deficiency
Lead poisoning
Sideroblastic anemia

Question 4

Normocytic anemia causes

Answer 4

Anemia of chronic disease
Uremia
Hypothyroidism
Aplastic anemia

Question 5

Macrocytic anemia causes

Answer 5

Folate deficiency
Vitamin B12 deficiency
Drug and alcohol induced anemia

Question 6

Response to iron therapy in IDA

Answer 6

12-24hrs: subjective improvements
26-28hrs: initial bone marrow response
28-72hrs: reticulocytosis
4-30 days: increasing Hgb level
1-3 mos: repletion of iron stores

Question 7

First lab value to decline in IDA

Answer 7

Serum ferritin

Question 8

Definitive diagnosis of Thalassemia

Answer 8

Hb electrophoresis

Question 9

Hereditary spherocytosis confirmatory test

Answer 9

Osmotic fragility test

Question 10

Suggested by an increased MCHC

Answer 10

Hereditary spherocytosis

Question 11

Curative for hereditary spherocytosis

Answer 11

Splenectomy

Question 12

Mutation in Sickle cell disease

Answer 12

Missense mutation

Question 13

Definitive diagnosis of Sickle cell disease

Answer 13

Hb electrophoresis

Question 14

PBS findings of Sickle cell disease

Answer 14

Sickle cells
Howell-Jolly bodies

Question 15

Most common hereditary bleeding disorder

Answer 15

von Willebrand disease

Question 16

Most common and most serious congenital coagulation factor deficiencies

Answer 16

Hemophilia A

Question 17

Most common hereditary hypercoagulable disorder

Answer 17

Factor V Leiden

Question 18

Hallmark of hemophilia

Answer 18

Prolonged bleeding

Question 19

Where is the earliest joint hemorrhages in children located?

Answer 19

Ankles

Question 20

Most common childhood malignancy

Answer 20

Acute lymphocytic leukemia

Question 21

Single most important prognostic factor in ALL

Answer 21

Response to treatment

Question 22

Poor prognostic factors in ALL

Answer 22

• <2 years or >10 years old
• Male
• WBC >100,000 u/L on presentation
• Presence of CNS leukemia
• Presenceofmediastinalmass

Question 23

At the end of induction therapy, peripheral blast count or Minimal Residual Disease must be

Answer 23

<0.01%

Question 24

Sites of relapse in ALL

Answer 24

Bone marrow
CNS
Testes

Question 25

Most common malignant extracranial/abdominal tumor in childhood

Answer 25

Neuroblastoma

Question 26

2nd most common malignant abdominal tumor in childhood

Answer 26

Wilms tumor

Question 27

Most common solid tumor in childhood

Answer 27

Brain tumor

Question 28

Most common soft tissue tumor

Answer 28

Rhabdomyosarcoma

Question 29

Malignancy with highest mortality

Answer 29

Brain (PNET)

Question 30

WAGR Syndrome

Answer 30

Wilms tumor Aniridia GU malformation Mental retardation

Question 31

Beckwith-Wedemann syndrome

Answer 31

• Visceromegaly • Macroglossia • Omphalocele • Hyperinsulinemic hypoglycemia • Wilms tumor, hepatoblastoma

Question 32

Denys-Drash syndrome

Answer 32

• Nephropathy • Renal failure • Male pseudohermaphrodism • Wilms tumor

Question 33

Malignancy that may present with raccoon eyes, subcutaneous tumor nodules, “dancing eyes, dancing feet”

Answer 33

Neuroblastoma

Question 34

Treatment for neuroblastoma Stage 4S

Answer 34

Observation (nearly 100% survival)

Question 35

Bone tumor usually found on metaphysis of long bones with sunburst pattern on x-ray

Answer 35

Osteosarcoma

Question 36

Bone tumor usually found on diaphysis of long bones with onion-skinning or moth-eaten appearance on x-ray

Answer 36

Ewing sarcoma

Question 37

Most common congenital cause of aplastic anemia

Answer 37

Fanconi anemia

Question 38

Neonatal polycythemia

Answer 38

Hematocrit >65% in term infants