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159 Cards in this Set
- Front
- Back
what is a first generation stem cell able to do?
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ability to differentiate into ANY type of blood cell
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what can second gen stem cells do?
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differentiate into myeloid or lymphoid precursor only
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in the first 8 weeks of gestation, the blood cells are maintained in the __ __
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yolk sac
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in the mesoblastic period, the inner region is __ __ cells
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primitive blood
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in the mesoblastic period, the outer region of the yolk sac will become the __ __
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blood vessels
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when do blood cells begin to migrate to other body tissue?
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by 6th week of gestation in the mesoblastic period
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what is the second phase of early cell development called>
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hepatic period
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when does the hepatic period of cell development occur?
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week 8 to 7 months
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From weeks 8-7months, where are blood cells formed?
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in the liver
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during the hepatic phase, the spleen will begin to generate ___ and at this time there is rampant destruction and ___ ___ will occur
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erythrocytes; erythroblastosis fetalis
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what occurs from the 2nd month ot the 2nd trimester in terms of blood production?
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activation of monocytes and lymphocytes by spleen; this will only every again occur in hemolytic crisis
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when does the thymus, lymph nodes and bone marrow become seeded with stem cells?
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by the 4th month
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___ in the thymus ceases in early childhood, but continues in ___ tissue for life
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leukopoiesis; lymphatic
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what is the final period of blood cell production?
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3rd trimester (7months on): blood produced in the bone marrow
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what is the 3rd period of blood cell production called?
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marrow or myeloid period
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in early childhood the RBCs are made in the long shafts of the __ and __
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tibia and femur; ribs; and sternum/vertebrae
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where are RBCs made in adulthood?
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bones of the axial skeleton. Pelvis
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what are the characteristics of RBCs?
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anucleate; highly flexible; biconcave discs; 80-100 femotoliters in volume
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what is the major role of RBCs?
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carry O2 to and CO2 away from cells
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erythropoietin is a ___ produced by the __-
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cytokine; kidney
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what is erythropoeitin needed for?
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necessary for erythroid proliferation and differentiation
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What occurs in the absence of erythropoeitin?
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apoptosis of erythroid committed cells
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ToF: kidney failure can cause anemia?
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true! No erythropoeitin production
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what is the name for the total volume count of platelets seen on labwork?
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MPV
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which is larger, platelets or their precursor?
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precusor: megakaryocytes
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how long do RBCs live?
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120 days
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RBCs are recycled by the __ and __
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liver and spleen
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during RBC destruction, __ and ___ are stored and ___ is released.
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iron and protein; bilirubin
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what do RBCs lack?
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nucleus, mitochondria, and cytoplasmic RNA
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what triggers the kidneys to produce erythropoeitin?
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decrease in blood O2 causes decreased O2 tension in tissues signalling to the kidneys
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ToF: people who live at higher altiutdes have more O2 available and thus less RBCs
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false opposite
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what RBC count would warrant a bleeding?
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>60
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the first microscopically recognizable RBC precursor is the ____
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pronormoblast
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what is a pronormoblast?
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RBC precursor; big cell filled with nuclei; has deep blue cytoplasm;
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how long does it take for a pronormoblast to lose nuclei and cytoplasmic RNA?
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7 days
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HgB will fill ___ the diameter of the pronormoblast.
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30%
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what is a normal reticulocyte count?
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0.5-1.5%
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what is a measurable precursor of RBC development?
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reticulocyte
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ToF: reticulocytes should be microscopically visible
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TRUE
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With normal Hgb A; reticulocytes should live in circulation for ___ __
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1 day
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the retic count can increase as high as ___ % after 1-2 weeks of iron therapy?
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20%
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what is the equation for the reticulocyte index?
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[retic count x (Hct/Normal Hct)]
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why must a retic calculation be done?
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to correct for anemia to get a true retic count
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the reticulocyte count is presented as a __
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percentage
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why is the reticulocyte production index needed?
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in anemia, the patient's RBCs are depleted, creating an erroneously elevated retic count
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An RPI > 3 indicates
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increased production of retics suggesting either hemolysis or blood loss
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an RPI < 2 means
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reduced or ineffective production of retics in the bone marrow for the degree of anemia
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what do target cells have?
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increased surface to volume ratio
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where are target cells seen most often?
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severe IDA; thalassemia; liver disease
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what are spherocytes?
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hyper dense with decreased surface to volume ration
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where are spherocytes seen?
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hereditarty spherocytosis; ABO incompatability; autoimmune hemolytic anemia
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what is another name for acanthocytes?
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spur cells
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what are acanthocytes?
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cells with 5-10 spicules of varying length and apear smaller than normal cells because of spheroid shape
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where are acanthocytes seen?
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liver disease; malabsorbtive disease; hypothyroidism
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what are echinocytes?
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Burr cells: 10-30 spicules of equal size distrubted evenly over the cell
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when are burr cells seen?
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artifact; dehydration; after RBC transfusion
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what are schistocytes?
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helmet, triangular shapes or small fragments caused by contact with abnormal vascular surfaces
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when are schistocytes seen?
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severe hemolytic anema (G6PD); HUS; DIC; connective tissue disorders; prosthetic valves (ie Blender syndrome)
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what are eliptocytes?
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elliptical; normochromic cells which normally comprise less than 1% of RBCs
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when are elliptocytes seen?
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SS disease/Thalassemia; severe IDA; severe bacterial disease; malaria
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what does it mean if normoblasts or nucleated RBCs are seen in a peripheral blood smear?
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the spleen is not working
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when does splenic function go down leading to normoblasts in the peripheral smear?
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in myeloproliferative blood disorders causing cellular overload
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when are normobloasts common?
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in the newborn period
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what are the causes of normoblasts seen in the peripheral blood smear?
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hypoxia; hemolytic anemia; anemia under treatment; megaloblastic anemia; ineffective erythropoeisis; collagen vascular disease; malignant neoplasms; chemotherapy; caucher's and other storage diseases
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ToF: nucleated RBCs may be prematurely released into the peripheral blood without reticulocytosis
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TRUE
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With NRBCs, dysplastic RBC changes may occur, as shown by the appearance of ___ and ___ cells in the peripheral blood
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macroovalocytes and teardrop
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what does a smear show with NRBCs?
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spherocytes and reticulocytes
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what is the treatment for NRBCs?
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splenectomy if Hgb is greater than 10 and retics are less than 10; folic acid daily due to increased demand for erythrocytes
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ToF: the osmolotic fragility test is specific for HS?
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false; it is normal in 10-20 percent of patients with disease
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what are the characteristics of teardrop cells?
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usually microcytic and hypochromic
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when are teardrop cells seen?
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newborns; thalassemia; myloproliferative disorders
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what are stomacytes?
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rare: slit like central pallor and considered normal in small numbers
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when are stomacytes seen?
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artifact; actue alcoholism; malignancies
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when are NRBCs seen?
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hypoxia; acute bleeding; severe hemolytic anemia; post splenectomy; congenital infections
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what are blister cells?
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area under membrane that is Hgb free. Looks like a blister
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when are blister cells seen?
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G6PD (during acute hemolysis); S disease; Pulmonary emboli
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what are basophillic stippling?
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coarse or fine punctate inclusion that represent areas of visible RNA
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when are basophilic stippling seen?
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Lead poisoning; IDA; hemolytic anemia
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what are Howell Jolly bodies?
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small, well definined, round densely stained inclusions with eccentric locations
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when are howell jolly bodies seen?
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post splenectomy or hyposplenic states; newborn period; megaloblastic anemia
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___% of childhood cancers are ___
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hematologic malignancies
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what are the most common childhood cancer?
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leukemia
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what are the demographics of leukemia in children?
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ALL has highest rate in 2-5 years old; males are higher than females; Hispanics > Whites > Blacks
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what are the common leukemias of childhood?
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ALL; ANLL; CML
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what are the common CNS cancers of childhood?
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Glioma; medulloblastoma
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What are the solid tumors of childhood?
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neuroblastoma; wilms tumor; rhabdomyosarcoma; osteosarcoma
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which childhood cancer is caused by heredity
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retinoblastoma
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what aer the environmental causes of childhood cancers?
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organic solvents; pesticides; HIV; parental smoking
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what are the drugs thought to cause cancer?
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diethylstibestrol; chemo; benzone
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ToF: the japanese survivors of the atomic bomb have a low risk of cancer
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false: high risk of leukemia
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what are the genetic abnormalities that are associated with an increased risk for childhood cancer?
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downs; NF1; li-fraumeni syndrome; basal cell nevus cancer syndrom
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children with down syndrome have a ___ times incidence of leukemia greater than the population at large with _-% of children with Down having leukemia by at 5
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15; 1
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of the newborns with transient myeloproliferatice disease, 25% will develop ___ _____
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acute megakaryoblastic leukemia
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what children have an increased risk of ALL?
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Downs; ataxia telangiectasia; Bloom syndrome; schwachman syndrome; fanconi anemia; klinefelter; and NF1
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ToF: in ALL, children will present with massive HSM and abnormalities in LFTs
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false: no changes in LFTs
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what are important sites of infiltrations of ALL?
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CNS and testes
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what are the clinical presentations of ALL related to expansion of the bone marrow?
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bone pain; limp; lytic lesions on xray or abnormalities on bone scan; pathologic fractures are uncommon
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why might atypicals be present in the peripheral smear of ALL?
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viruses, and therefore a biopsy is needed
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coagulopathies result from a rare subtyep of ANLL: ___ __ ___
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acute promyeloctyic leukemia
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neutropenia of ALL can result in __, whereas bleeding results from ___
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sepsis; thrombocytopenia
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why can airway obstruction occur in ALL?
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large mediatstinal lymphadenopathy (seen in T-cell ALL) adolesecent boys
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___ damages vital organs in ANLL
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hyperleukoctyosis
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what is thought to be classically elevelated in ALL?
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uric acid do to breakdown of leukemic cells
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A rare B-cell subtype of ALL can present in __ __
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renal failure
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ToF; Hypercalcemia appears with ALL
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true, but also can have hypocalcemia
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what are the symptoms of CNS involvement with leukemia?
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increased ICP; headache; vomiting; papilledema
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what are the signs of testicular infiltration of leukemia?
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painless, firm enlargement of one or both testes
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what are the common presenting symptoms of leukemia?
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fever; fatigue; cutaenous or mucosal bleeding; bone pain; pallor; anorexia; weight loss
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what are the favorable factors of ALL?
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dx between 1-9; WBC < 50,000; no chromosomal abnormalities; pre-B cell;
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What do infants (<6months) who are dx with ALL often have?
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MLL gene rearrangements, CD10 negative/FLT3 positive
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__ __ ALL accounts for 80-85%
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precursor B-cell
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which groups often have T-cell ALL?
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older males with higher WBC presenting
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what are the other malignancies than leukemia that have bone marrow involvement?
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neuroblastoma; retinoblastoma; rhabdomyosarcoma; non-hodgekins
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what are the non-malignant conditions that are differentials for ALL?
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JRA; mono; ITP; pertussis; parapertussis; aplastic anemia; acute infectious lymphocytosis
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what are the characteristics of very high risk ALL?
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t(9,22), hypodiploidy, MLL translocation with slow response; induction failure
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ToF: AML is common in childhood
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FALSE
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what is the mortality rate of AML?
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50% with standard risk
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what is the treatment like for AML?
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most toxicity of all childhood cancers with multiple chemotherapies
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what is the ultimate treatment for AML?
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HSCT
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what are the congenital syndroms associated with increased risk of AML?
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polycythemia vera; thrombocytopenia; idiopathic myelofibrosis and myelodysplastic syndrome
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what is myelodysplastic syndrome?
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hypercellularity with dysplastic changes in meyloid precursor cells
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___ have a higher risk of NHL?
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males
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ToF: most cases of NHL in children are high grade
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TRUE
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what are the types of NHL?
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mature B-cell; lymphoblastic; anaplastic
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lymphoproliferative disease in NHL is associated with __
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immunodeficiency
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what is Stage I NHL?
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single tumor or nodal area (excluding mediastinum, abdomen)
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if a child has NHL with regional involvement, multiple primaries on one side of the diaphragm, they are stage__
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II
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what is stage III NHL?
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tumors on both sides of the diaphragm, primary intrathoracis or extensive abndominal disease
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what is Stage IV NHL?
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bone marrow and or cns involvement
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what is the cure rate of HL?
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90-95%
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what pathogen is associated with HL?
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EBV
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what are the classifcations of classic HL?
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lymphocyte-rich; nodular sclerosis; mixed-cellularity; lymphocyte depleted
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what is the most common pediatric solid tumor of the head and neck?
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rhabdo
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how do primary nasopharyngeal tumors of rhabdo present?
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nasal congestion, mouth breathing and unilateral otitis
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what is rhabdo?
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malignant tumor of skeletal muscle origin
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what are the most common sites of rhabdo?
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head, neck, GU tract, and extremities
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what are the syndromes associated with Rhabdo?
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Li Fraumeni (p53); costello (HRAS mutation); beckwith-wiedemann; NF1; Noonan
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what age group has the best prognosis with rhabdo?
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1/9/2013
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what are the favorable sites for rhabdo?
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orbit; nonparameningeal head and neck; paratestis; vulva; vagina; uterus; bilary tract
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what size tumors of rhabdo have best survival?
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<5cm
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in rhabdo, ___ histology is favorable and the ___ histology is unfavorable
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embryonal; alveolar
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what is the most common malignancy in the first month after birth?
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neuroblastoma
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where can neuroblastoma occur?
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anywhere along the sympathetic chanin including cervical ganglia
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what syndrome can occur with neuroblastoma?
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horner's syndrome, sweat on 1/2 of face
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what is secreted in neuroblastoma?
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catecholamines (VMA, HVA)
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what does the secretion of cathecholamines in Neuroblastoma cause?
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diaphoresis and hypertension
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what is a nonspecific marker of cellular turnover in Neuroblastoma?
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LDH
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what is part of the diagnosis of neuroblastoma?
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LDH; serum phosphorus and CMP; chest xray; US for neck masses; CT with contrast
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what is the survival of Wilms?
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>90%
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what are the malformations associated with Wilms?
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beckwith-weidemann; Anirida (PAX6); Aniridia/GU anomalies/MR -del(11p13); Denys-Drash syndrome- germline WT1 and somatic CTNNB1 mutations
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what is stage 1 Wilms?
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limited to the kidney, GTR
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what stage of wilms has regional extension, GTR?
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II
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what stage of wilms has residual tumor in abdomen?
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III
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what stage is Metastatic Wilms?
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IV
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what is stage V wilms?
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bilateral
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what are the prognostic factors for wilms?
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anaplastic (focal) or (diffuse); nephroblastomatosis; loss of heterozygosity for 1p and 16q
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what are the common brain tumors of the infratentorium?
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astrocytoma; ependymoma; medulloblastoma; brain stem glioma; ATRT
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what are the common brain tumors of the supratentorium?
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astrocytoma; PNET, CPC, ependymoma; germinoma; ganglioglioma
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what are the common brain tumors of the sellar/suprasellar region?
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craniopharyngioma; astrocytoma; germinoma
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