Hematologic and Oncologic Diseases in Children

Question 1

RBC disorder

Answer 1

Sickle Cell (later Erin Coyne )
Thalassemia syndromes

Question 2

Thalassemia Syndrome

Answer 2

Inherited defect of hgb chain (alpha or beta)
Inadequate hgb production
Excessive destruction of RBC
Frequent RBC transfusions required
>2 million carry trait in US

Question 3

Disorders of hemostasis (clotting disorders)

Answer 3

Hemophilia
Von Willebrand disease
Immune thrombocytopenia

Question 4

Hemophilia

Answer 4

Congenital deficiency in clotting factor
Results in prolonged bleeding

Question 5

Von Willebrands Disease

Answer 5

Inherited deficiency or defective clotting protein
Characterized by mucous membrane bleeding

Question 6

Immune thrombocytopenia (ITP)

Answer 6

Acquired disorder of excessive platelet destruction

Question 7

Common pediatric hematologist disorders

Answer 7

Marrow failure syndromes

Question 8

Marrow Failure Syndrome

Answer 8

Aplastic Amenia
Red Cell Aplasia

Question 9

Aplastic Anemia

Answer 9

Profound suppression of production of formed elements of the blood

Congenital or acquired

Question 10

Red cell aplasia

Answer 10

Lack of erythroid (RBC) cell production

Congenital (Diamond-Blackfan anemia)

Acquired (environment)

Question 11

What causes childhood cancer

Answer 11

Genetic mutation

Question 12

Cellular Mutation results in

Answer 12

Lack is differentiation
Loss contact inhibition
Unregulated growth
Cellular immortality

Question 13

Children increased risk for cancer

Answer 13

Genetic predisposition
Immunodeficiency
History of cancer/treatment

Question 14

Genetic predisposition

Answer 14

Down’s syndrome
Li-Fraumeni syndrome
Retinoblastoma gene
Fanconi Anemia

Question 15

Down’s syndrome do better with what type of cancer

Answer 15

Leukemia. Need less treatment

Question 16

Li-Fraumeni syndrome

Answer 16

Missing tumor suppression gene

Question 17

Fanconia Anemia

Answer 17

Fragile DNA

Question 18

Immunodeficiency

Answer 18

Congenital/acquired
Treatment related (post solid organ transplant patients)

Question 19

Worldwide what is the most common inherited pediatric hematology disorder

Answer 19

Sickle cell

Question 20

Who might be at higher risk for developing pediatric cancer

Answer 20

Down’s syndrome

Question 21

What are the top 2 pediatric cancer diagnoses

Answer 21

ALL/AML
Brain

Question 22

Childhood cancer is not a single disease but a wide range of diseases that are classified into two broad subcategories:

Answer 22

Hematologic malignancies
Solid tumors

Question 23

How are solid tumors named

Answer 23

Based on type of tissue and/or site which the cancer develops

Question 24

3 germ cell layers in developing embryo

Answer 24

Ectoderm
Mesoderm
Endoderm

Question 25

Ectoderm includes

Answer 25

CNS (brain and spinal cord) SNS (adrenal- small gland on top is each kidney)-hormones that control HR, BP and other functions

Question 26

Mesoderm

Answer 26

Blood Lymphatics Bone Muscle Heart Kidney Gonads

Question 27

Endoderm

Answer 27

Epithelium GI tract Thyroid

Question 28

Most childhood cancers arise from which embryonic germ cells

Answer 28

Ectoderm Mesoderm

Question 29

2 common types of childhood cancers

Answer 29

Hematologic Solid tumors

Question 30

Hematologic cancers

Answer 30

Leukemia (blood and bone marrow) Lymphoma (lymph)

Question 31

Solid tumors

Answer 31

CNS Neuroblastoma Rhabdosarcoma Wilms Tumor Ewings sarcoma/osteosarcoma Retinoblastoma

Question 32

CNS

Answer 32

Brain and spinal cord

Question 33

Neuroblastoma

Answer 33

SNS - found in small adrenal glands on top of kidneys It can develop in the belly, chest, neck, pelvis, and bones. Usually children younger than 5

Question 34

Rhabdomyosarcoma

Answer 34

Muscle

Question 35

Wilms Tumor

Answer 35

Kidney

Question 36

Ewing sarcoma / osteosarcoma

Answer 36

Bones

Question 37

Retinoblastoma

Answer 37

Eyes

Question 38

Patterns of onset (infancy and early childhood)

Answer 38

Neuroblastoma Wilms Tumor Retinoblastoma

Question 39

Patterns of onset (adolescence)

Answer 39

Hodgkin lymphoma Osteosarcoma

Question 40

Reasons for improved survival

Answer 40

Clinical trials Multimodal therapy Intense supportive care Multidisciplinary approach

Question 41

Clinical trials. Name of group

Answer 41

Children’s oncology group

Question 42

COG

Answer 42

NCI- (National cancer institute) supported pediatric clinical trial group North America, Canada, Australia, New Zealand, Europe and Saudia Arabia 220 institutions 8500 individual members

Question 43

Treatment modalities

Answer 43

Chemotherapy Biotherapy Immunotherapy Radiation Surgery HSCT (Haematopoietic stem cell transplant)

Question 44

Children and adolescents better treated in adult or pediatric institutions

Answer 44

Better outcomes in specialized pediatric oncology centers