CPHON Exam 117 Questions with Verified Answers
Auer rods - CORRECT ANSWER AML distinguishing feature
Leukemia - CORRECT ANSWER Malignant disorder of
... [Show More] the blood and blood forming organs including the bone marrow, lymph nodes and spleen
Cytogenetics for Leukemia - CORRECT ANSWER Ploidy; abnormalities found in more than 90% of patients with ALL; with number of chromosomes and structures (translocation)
Hyperdiploidy in ALL - CORRECT ANSWER More than 46; favorable if not more than 50
Genetic associations with leukocytes - CORRECT ANSWER Trisomy 21, schwachman diamond syndrome, neurofibromatosis, fanconi anemia, Bloom syndrome, ataxia telanflectasia
Favorable AML - CORRECT ANSWER Age >2, WBC <50K, female, absence of extramedullary disease, BMI between 11&94th percentile, Down syndrome
Favorable ALL - CORRECT ANSWER Age 2-10 at diagnosis, WBC <50k, neg MRD at day 29
MRD - CORRECT ANSWER Ratio of leukemia cells to normal bone marrow cells; use DNA-PCR probes; elevated = poor prognosis and increased risk of relapse
Goal is < 5% blasts
Consolidation in leukemia chemo - CORRECT ANSWER Peg, MTX, VCR, doxo, corticosteroid, cytarabine, oral MTX, 6MP
Maintenance Chemo in leukemia - CORRECT ANSWER 2-3 years 6MP, MTX, VCR, corticosteroid, CNS prophylaxis
Classifications for Leukemia - CORRECT ANSWER French American British and WHO
Immunophenotyping - CORRECT ANSWER Monoclonal antibodies that develop in response to changes in the expression of cell surface antigens: cd10 positive in 80% of patients with ALL
ALL - CORRECT ANSWER Most common risk for TLS
AML - CORRECT ANSWER Most common risk for DIC
Adolescents - CORRECT ANSWER Most common age group to be dx with lymphoma
Non-Hodgkin's Lymphoma - CORRECT ANSWER Originates in cells and organs of immune system, more systemic, spreads rapidly and unpredictably and often aggressively, lymphoid lineage
Non-Hodgkin's Lymphoma risk - CORRECT ANSWER Increased risk with wiskotts aldrich syndrome, ataxia telanfiectisa, post BMT, SCIDS, AIDS, other immunosuppressive disorders
EBV - CORRECT ANSWER Role unclear but thought to precede or occur with b-cell transformation with Burkitts
4 types of Non hodgkins - CORRECT ANSWER Precursor (lymphoblastic), Burkitts, diffuse large B cell, anaplastic large cell
Induction chemo for AML - CORRECT ANSWER 3-7 days of cytarabine, doxo, 6tg, prednisone, vp16 ; abx and antifungal prophylaxis
Diploid - CORRECT ANSWER 46 chromosomes
Staging for non hodgkins - CORRECT ANSWER Ann Arbor or Murphy (st Jude)
Good prognostic for non hodgkins - CORRECT ANSWER Primary tumor head/neck/LN/GI stage 1 or 2
Poor prognostic for non hodgkins - CORRECT ANSWER Incomplete remission, increased tumor burden, LDH> 1000, dealt in treatment
Hodgkin's lymphoma - CORRECT ANSWER Involves lymphatic system, including spleen and all lymph nodes; characterized by reed sternburg cells
Reed-Sternberg cells - CORRECT ANSWER Abundant cytoplasm and large nucleus; most of tumor composed of inflammatory cells and fibrosis due to cytokines release
Night sweats - CORRECT ANSWER Cardinal sign of lymphoma
Hodgkin's lymphoma B cell sxs - CORRECT ANSWER Fatigue, fever for more than 3 days, weight loss 10% in 6mo, night sweats
Favorable prognostic of hodgkins - CORRECT ANSWER Localized involvement, abscense of B sxs and bulky disease
Unfavorable hodgkins - CORRECT ANSWER Advanced stage, B sxs, bulky disease, extranodal disease, male, elevated ESR
Astrocytomas - CORRECT ANSWER Most common brain tumor ~50%
Astrocytomas - CORRECT ANSWER Small blue cell arising from cerebellum or 4th ventricle, aggressive Mets in CNS and Bm, classic, desmoplastic large cell anaplastic histology, sonic hedgehog pathway
Posterior fossa sxs (front of brain) - CORRECT ANSWER Headache, vomiting, ataxia, nystagmus, mutism, speech disturbances, dysphasia, decreased motor function, emotional lability, cranial nerve palisies, diplopia; 24-100 hours post surgery
> 100cGy - CORRECT ANSWER Amount of brain radiation associated with significant late effects
Neuroblastoma - CORRECT ANSWER Malignancy of the parasympathetic nervous system, develops from neural crest cells, found in form of mass in neck, chest, abdomen, often in adrenal gland, silent tumor; most common extra cranial solid tumor in children
ICP sxs - CORRECT ANSWER Morning headaches, lethargy, N/V
Supratentorial sxs (back of brain) - CORRECT ANSWER Heiparesis, seizures, visual changes, intellectual problems (back of brain)
Neuroblastoma patho - CORRECT ANSWER Small round blue cell neoplasm
Horner syndrome in NB - CORRECT ANSWER Unilateral ptosis, myosis, anhydrosis,
Neuroblastoma clinical sxs - CORRECT ANSWER Urine catecholamines, VIMA (vanillymadelic acid) and HVA (homovallnicllic acid) elevated
Blueberry muffin sign in NB - CORRECT ANSWER SubQ nodules appear hardened bluish nodules that can be seen or palpated; often seen with infants with favorable tumors
Mibg - CORRECT ANSWER Scan sensitive to NB, useful in determining metastic disease requires use of potassium iodine to protect thyroid radiation exposure
Favorable NB - CORRECT ANSWER Less than 1yo, hyperdiploid, stage 1, 2, or 4s, primary tumor chest neck/pelvis, high TRKA expression
Poor characteristics of NB - CORRECT ANSWER Increased copies of n-myc, increased ferritin, primary tumor in abdomen, rapid tumor progression
Osteosarcoma - CORRECT ANSWER Associated with p53 tumor suppressor gene
Osteosarcoma - CORRECT ANSWER Production of osteoid substance
Genetic associations with osteo - CORRECT ANSWER Hereditary retinoblastoma, li-faumeni syndrome, Roth mind Thompson
Osteo sxs - CORRECT ANSWER Dull aching pain, increasing at night, averages 3 months before diagnosis, possible soft tissue swelling
Distal femur, proximal tibia and humerus - CORRECT ANSWER Most common sites of osteo (long bone)
Lungs - CORRECT ANSWER Most common relapse site of osteo
Surgical reaction in osteo - CORRECT ANSWER Goal is to completely remove tumor with healthy cell margin of at least 1/2 cm around cancer site
Rotationplasty - CORRECT ANSWER Excision of femur and preservation of lower leg then lower leg turned 180 degrees
Osteo surgery options - CORRECT ANSWER Limb salvage, athrodesis, amputation, rotation plasty
Ewing's - CORRECT ANSWER Undifferentiated, malignant tumor of the bone and soft tissue, radiation sensitive
Ewing's sxs - CORRECT ANSWER Bone pain intermittent 3-9 months, palpable mass, fractures common, primary sites extremity 53% and central 47%
Ewing's labs - CORRECT ANSWER Increased ESR, increased WBC; often mistaken for osteomyelitis
Genetic condition with sunburst - CORRECT ANSWER Rotham thompason with osteo
Wilms - CORRECT ANSWER Tumor of kidney; large rapidly growing vascular abdominal tumors, gelatinous capsule
Incidence and etiology of Wilms - CORRECT ANSWER Beckwith wiseman, denys drash,; 2-3 years older, aniridia
Aniridia - CORRECT ANSWER Absence of iris; in wilms tumor
Presentation of wilms - CORRECT ANSWER Abdominal mass often found by parent bathing patients or picking up child, fever, hematuria, malaise, pain, elevated BP
Rhabdomyosarcoma - CORRECT ANSWER Malignant tumor of the mesenchymal cell origin; often formed skeletal muscle; sMall round blue cell tumor
Retinoblastoma - CORRECT ANSWER Characterized by cells with scant cytoplasm and hyper chronic nuclei of various sizes, arranged in tight bouquet like cluster called flexner winterstiner rosettes
leukocoria - CORRECT ANSWER Cats eye reflex; lack of normal reflex either unilateral or bilateral most common presenting signs for retinoblastoma
Sxs of retinoblastoma - CORRECT ANSWER Strabismus, eaotropia, decreased visual acuity, inflammatory signs
Fundoscopic exam - CORRECT ANSWER Eye exam for retinoblastoma
Atg - CORRECT ANSWER Immunotherapy; bind and destroy patients t lymphocytes in attempt to decrease incidence of graft rejections
Campath - CORRECT ANSWER Cd45; deplete patients circulating lymphocytes to decrease graft rejections or gvhd; immunotherapy
Alemtuzumab - CORRECT ANSWER Immunotherapy; monoclonal antibody, binds to cd52 protein in surface of mature lymphocytes; drug induced antibody dependent lysis causing removal of lymphocytes from the blood and bone marrow. Side effects hypoTN, rigors, fever, bronchospasms, chills, rash
Autoimmune hemolytic anemia - CORRECT ANSWER Malfunction of immune system where antibodies are produced against antigens on the surface of red blood cells, resulting in premature erythrocytes destruction
Autoimmune hemolytic anemia - CORRECT ANSWER Positive direct Coombs test, decreased HGB, increased retic, sphereocytes, increased bili, increased ldh
Autoimmune hemolytic anemia tx - CORRECT ANSWER Corticosteroids, ritux, splenectomy; blood transfusion, folic acid; if refractory, immunosuppressive therapy (cyclosporine( ivig, 6MP, cutoxan,VCR; warm and cold antibody
Antimetabolties chemo - CORRECT ANSWER Cytarabine, 6MP, MTX, thiotuanine, fludarabine, gemcitabine
Antitumor or antibiotic chemo - CORRECT ANSWER Bleo, dactinomycin, Dano, doxo, mitoxantrone
Chemo vesicants - CORRECT ANSWER VCR, paclixael, danu doxo
Chemo irritants - CORRECT ANSWER Ifos and bleo
B.cell lymphocytes - CORRECT ANSWER Derived from marrow, produce marrow, humoral immunity
T cell lymphocytes - CORRECT ANSWER Mature in thymus, attack invaders, cellular immunity
SVCS - CORRECT ANSWER Cough, dyspnea, orthopnea, wheezing, stridor, anxiety, confusion, edema, plethora, cyanosis of face:neck/upper arm/chest
SIADH - CORRECT ANSWER too much ADH; decrease Na, decreased UOP, increased spec grav, increased BP, weight gain; restrict fluids and monitor electrolytes; tx underlying conditions
DI - CORRECT ANSWER Too little ADH; increased Na, increased UOP, decreased spec grav, increased thirst, decreased BP increased HR; replace ddavp
Schwachman-Diamond syndrome - CORRECT ANSWER A rare congenital disorder that affects many parts of the body particularly the bone marrow, pancreases and skeletal system
Neurofibromatosis - CORRECT ANSWER A genetic disorder that causes tumors to form on nerve tissue including the brain, spinal cord and nerves
Bloom syndrome - CORRECT ANSWER A rare genetic disorder that primarily affects the skin (rash with exposure to sun) and increases chance of developing cancer
Ataxia Telangiectasia - CORRECT ANSWER A rare genetic disorder that affects the nervous system, immune system and other body systems
Wiskott-Aldrich syndrome - CORRECT ANSWER Characterized by a formal immune system function (immune deficiency) and micro thrombocytopenia
Pagets disease - CORRECT ANSWER Disorder that involves abnormal bone destruction and regrowth
Li-Fraumeni syndrome - CORRECT ANSWER Rare disorder that greatly increases the risk of developing several types of cancers (hereditary cancer predisposition)
Rothmund-Thomson Syndrome - CORRECT ANSWER A rare condition affecting many parts of the body, particularly the skin and increasing risk or developing cancer (osteo)
Beckwith-Wiedemann syndrome - CORRECT ANSWER An overgrowth syndrome
Denys-Drash syndrome - CORRECT ANSWER A condition affecting the kidneys and genitalia
Simpson-Golabi-Behmel Syndrome - CORRECT ANSWER An overgrowth syndrome, affects many parts of the body (face, chest, abdomen, heart, kidneys, liver, spleen, intellectual abilities)
Ewing's, rhabdomyosarcoma, non hodgkins, neuroblatoma - CORRECT ANSWER 4 cancers associated with small round blue cell
4 cancers associated with small round blue cell - CORRECT ANSWER Ewing's, rhabdomyosarcoma, non hodgkins, neuroblatoma
pharmokinetics - CORRECT ANSWER the study of drugs within the body, absorption , distribution, metabolism, excretion
Pharmodynamics - CORRECT ANSWER concentration of available drug
multimodal therapy - CORRECT ANSWER combo of one or more types of therapy
adjuvant chemo - CORRECT ANSWER chemo to treat residual disease
combo chemo - CORRECT ANSWER use of more than one drug
neoadjuvant chemo - CORRECT ANSWER used preop to decrease tumor bulk
sanctuary site - CORRECT ANSWER area where malignant cells are sequestered
cell life cycle - CORRECT ANSWER g0, g1, S, g2, M
alkylating agents - CORRECT ANSWER cyclophosphamide, ifosfamide, melphalan, mechlorethamine, thiotepa, mitocyin, procarbazine, busulfan, dacarbazine, temzolamide, carboplatin/cisplatin, topotecan/ironotecan
akylating agents - CORRECT ANSWER chemo's that are cell cycle non-specific, cross link DNA strand, unlink coiled DNA, inhibit DNA synthesis, major toxicities: hematopoetic, GI tract, reproductive
antimetabolites agents - CORRECT ANSWER most agents are cell cycle specific (S phase), substitute erroneous metabolites or structural analogues during DNA synthesis, major toxicities: hematopoetic, GI
antimetabolites - CORRECT ANSWER MTX (Folic Acid antagonists), 5-FU, cytosine arbinoside (cytarabine), gemcitabine (pyridimine antagonists) 6-MP, 6-TG, Fludarabine (purine antagonists)
antitumor antibiotics - CORRECT ANSWER anthracylcines: dano, doxo, idarubicin, mitaxantrone, dactinomycin, bleo, mitomycin
plank alkaloids - CORRECT ANSWER vinblastine/vincristine, etoposide, pacitaxel, docetexal
nitrosureas - CORRECT ANSWER most are cell cycle non-specific, cross blood brain barrier, interfere with DNA replication and repair
nitrosureas - CORRECT ANSWER Carmustine, Lomustine
plant alkaloids - CORRECT ANSWER cell cycle specific predominantly in M phase, cause arrest during mitosis, DNA strand breakage/death; major toxicities: neurologic, GI, reproductive, second malignancies
antiangiogenic agents - CORRECT ANSWER prevent growth of new microvessels from capillary endothelial cells to the tumor; new classification of cancer drugs
antiangiogenic agents - CORRECT ANSWER thalidomide, anti-vascular endothelial growth factor
hormonal agents - CORRECT ANSWER bind with DNA/modifies transcription process; lyse lymphocytes, indrect effect on other malignancies, used in supportive care
hormonal agents - CORRECT ANSWER prednisone, dexamethasone
bio response modifiers - CORRECT ANSWER direct antitumor activity--cytotoxic, anti-proliferation mechanism, affect differentiation/maturation of cells, prevent metasis; can cause cap leak, flu like/fever/chills/bone pain
bio response modifiers - CORRECT ANSWER alpha interferon, interleukin 2, g-csf, gm-csf, erythropoietin, monocolonal antibodies [Show Less]