Hematologic and Oncologic Diseases in Children Flashcards

1
Q

RBC disorder

A

Sickle Cell (later Erin Coyne )
Thalassemia syndromes

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2
Q

Thalassemia Syndrome

A

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

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3
Q

Disorders of hemostasis (clotting disorders)

A

Hemophilia
Von Willebrand disease
Immune thrombocytopenia

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4
Q

Hemophilia

A

Congenital deficiency in clotting factor
Results in prolonged bleeding

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5
Q

Von Willebrands Disease

A

Inherited deficiency or defective clotting protein
Characterized by mucous membrane bleeding

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6
Q

Immune thrombocytopenia (ITP)

A

Acquired disorder of excessive platelet destruction

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7
Q

Common pediatric hematologist disorders

A

Marrow failure syndromes

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8
Q

Marrow Failure Syndrome

A

Aplastic Amenia
Red Cell Aplasia

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9
Q

Aplastic Anemia

A

Profound suppression of production of formed elements of the blood

Congenital or acquired

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10
Q

Red cell aplasia

A

Lack of erythroid (RBC) cell production

Congenital (Diamond-Blackfan anemia)

Acquired (environment)

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11
Q

What causes childhood cancer

A

Genetic mutation

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12
Q

Cellular Mutation results in

A

Lack is differentiation
Loss contact inhibition
Unregulated growth
Cellular immortality

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13
Q

Children increased risk for cancer

A

Genetic predisposition
Immunodeficiency
History of cancer/treatment

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14
Q

Genetic predisposition

A

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

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15
Q

Down’s syndrome do better with what type of cancer

A

Leukemia. Need less treatment

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16
Q

Li-Fraumeni syndrome

A

Missing tumor suppression gene

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17
Q

Fanconia Anemia

A

Fragile DNA

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18
Q

Immunodeficiency

A

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

19
Q

Worldwide what is the most common inherited pediatric hematology disorder

A

Sickle cell

20
Q

Who might be at higher risk for developing pediatric cancer

A

Down’s syndrome

21
Q

What are the top 2 pediatric cancer diagnoses

A

ALL/AML
Brain

22
Q

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

A

Hematologic malignancies
Solid tumors

23
Q

How are solid tumors named

A

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

24
Q

3 germ cell layers in developing embryo

A

Ectoderm
Mesoderm
Endoderm

25
Q

Ectoderm includes

A

CNS (brain and spinal cord)

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

26
Q

Mesoderm

A

Blood
Lymphatics
Bone
Muscle
Heart
Kidney
Gonads

27
Q

Endoderm

A

Epithelium
GI tract
Thyroid

28
Q

Most childhood cancers arise from which embryonic germ cells

A

Ectoderm
Mesoderm

29
Q

2 common types of childhood cancers

A

Hematologic

Solid tumors

30
Q

Hematologic cancers

A

Leukemia (blood and bone marrow)

Lymphoma (lymph)

31
Q

Solid tumors

A

CNS
Neuroblastoma
Rhabdosarcoma
Wilms Tumor
Ewings sarcoma/osteosarcoma
Retinoblastoma

32
Q

CNS

A

Brain and spinal cord

33
Q

Neuroblastoma

A

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

34
Q

Rhabdomyosarcoma

A

Muscle

35
Q

Wilms Tumor

A

Kidney

36
Q

Ewing sarcoma / osteosarcoma

A

Bones

37
Q

Retinoblastoma

A

Eyes

38
Q

Patterns of onset (infancy and early childhood)

A

Neuroblastoma
Wilms Tumor
Retinoblastoma

39
Q

Patterns of onset (adolescence)

A

Hodgkin lymphoma
Osteosarcoma

40
Q

Reasons for improved survival

A

Clinical trials
Multimodal therapy
Intense supportive care
Multidisciplinary approach

41
Q

Clinical trials. Name of group

A

Children’s oncology group

42
Q

COG

A

NCI- (National cancer institute) supported pediatric clinical trial group

North America, Canada, Australia, New Zealand, Europe and Saudia Arabia

220 institutions
8500 individual members

43
Q

Treatment modalities

A

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

44
Q

Children and adolescents better treated in adult or pediatric institutions

A

Better outcomes in specialized pediatric oncology centers