Neoplasia in Pediatrics
Wiskott aldrich syndrome- X -inked recessive eczema thrombocytopenia immunodeficiuncy syndrome
Hodgkin disease-malignant lymph node disorder of unknown etiology
Nonhodgkin lymphoma-heterogenous group of lymphoid tumors
Causes: Viral infxn and immunodeficiency
What is tumor lysis syndrome? overload of lysed tumor material in the blooidstream leading to hyperuricemia compromising kidney function Tx: hydration is important, allopurinol and sodium bicarbonate
What is Bloom syndrome? aka Bloom–Torre–Machacek syndrome, a rare autosomal recessive disorder characterized by short stature and predisposition to the development of cancer. Cells from a person with Bloom syndrome exhibit a striking genomic instability that includes excessive homologous recombination.
What is the most common leukemia in children? ALL (acute lymphoblastic leukemia)
In leukemia, what is induction? 4 to 6 weeks initial treatment phase for reduction of leukemic burden
Usual Rx: corticosteroid, vincristine, asparaginase with or without anthracycline
What constitutes remission? decreased of blast cells in bone marrow by 5%.
Commonly used maintenance drugs in ALL? methothrecate, 6-mercaptourine, corticosteroid, vincristine
What is Fanconi Anemia?
genetic defect in a cluster of proteins involved in DNA repair, most common in Ashkenazi Jews, frequently leds to the development of acute myelogenous leukemia
Treatment for AML?
ARA-C-it kill cells by interfering DNA synthesis, used in the treatment of AML and nonHodgkin Lymphoma, aka as cytrabine or ARA-C Arabinofuranosyl cytidine
Daunorubicin-
Wiskott aldrich syndrome- X -inked recessive eczema thrombocytopenia immunodeficiuncy syndrome
Hodgkin disease-malignant lymph node disorder of unknown etiology
Nonhodgkin lymphoma-heterogenous group of lymphoid tumors
Causes: Viral infxn and immunodeficiency
What is tumor lysis syndrome? overload of lysed tumor material in the blooidstream leading to hyperuricemia compromising kidney function Tx: hydration is important, allopurinol and sodium bicarbonate
What is Bloom syndrome? aka Bloom–Torre–Machacek syndrome, a rare autosomal recessive disorder characterized by short stature and predisposition to the development of cancer. Cells from a person with Bloom syndrome exhibit a striking genomic instability that includes excessive homologous recombination.
What is the most common leukemia in children? ALL (acute lymphoblastic leukemia)
In leukemia, what is induction? 4 to 6 weeks initial treatment phase for reduction of leukemic burden
Usual Rx: corticosteroid, vincristine, asparaginase with or without anthracycline
What constitutes remission? decreased of blast cells in bone marrow by 5%.
Commonly used maintenance drugs in ALL? methothrecate, 6-mercaptourine, corticosteroid, vincristine
What is Fanconi Anemia?
genetic defect in a cluster of proteins involved in DNA repair, most common in Ashkenazi Jews, frequently leds to the development of acute myelogenous leukemia
Treatment for AML?
ARA-C-it kill cells by interfering DNA synthesis, used in the treatment of AML and nonHodgkin Lymphoma, aka as cytrabine or ARA-C Arabinofuranosyl cytidine
Daunorubicin-