Hematologic and Oncologic Diseases in Children Flashcards
RBC disorder
Sickle Cell (later Erin Coyne )
Thalassemia syndromes
Thalassemia Syndrome
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
Disorders of hemostasis (clotting disorders)
Hemophilia
Von Willebrand disease
Immune thrombocytopenia
Hemophilia
Congenital deficiency in clotting factor
Results in prolonged bleeding
Von Willebrands Disease
Inherited deficiency or defective clotting protein
Characterized by mucous membrane bleeding
Immune thrombocytopenia (ITP)
Acquired disorder of excessive platelet destruction
Common pediatric hematologist disorders
Marrow failure syndromes
Marrow Failure Syndrome
Aplastic Amenia
Red Cell Aplasia
Aplastic Anemia
Profound suppression of production of formed elements of the blood
Congenital or acquired
Red cell aplasia
Lack of erythroid (RBC) cell production
Congenital (Diamond-Blackfan anemia)
Acquired (environment)
What causes childhood cancer
Genetic mutation
Cellular Mutation results in
Lack is differentiation
Loss contact inhibition
Unregulated growth
Cellular immortality
Children increased risk for cancer
Genetic predisposition
Immunodeficiency
History of cancer/treatment
Genetic predisposition
Down’s syndrome
Li-Fraumeni syndrome
Retinoblastoma gene
Fanconi Anemia
Down’s syndrome do better with what type of cancer
Leukemia. Need less treatment
Li-Fraumeni syndrome
Missing tumor suppression gene
Fanconia Anemia
Fragile DNA