Help
Options
Didn't know it?
click below
click below
Knew it?
click below
click below
Don't Know
Remaining cards (0)
Know
retry
shuffle
restart
0:00
Embed Code - If you would like this activity on your web page, copy the script below and paste it into your web page.
Normal Size Small Size show me how
Normal Size Small Size show me how
Pediatric Hem/Onc
Pediatric Board Review 2011
| Question | Answer |
|---|---|
| Hematopoiesis location; First 2-4 weeks of gestation | Yolk Sac |
| Hematopoiesis location; 5-8 weeks of gestation | Liver |
| Hematopoiesis location; 5 months of gestation and beyond | Bone Marrow |
| RBC lifespan in adult and newborn | 120 days; 45-60 days |
| Microcytic Hypochromic anemia; low MCV, low Fe, High TIBC, low Transferrin, low ferritin | Iron deficiency |
| Anemia - normocytic to microcytic; low FE, Low TIBC, Low to normal Tranferrin, normal to high ferritin; Low EPO | Anemia of Chronic disease |
| Anemia; Low MCV, High RDW; Chinese Toys; Mexican Candy; Neuropathy, dementia, abdominal pain, nausea, anorexia | Lead Toxicity |
| Anemia; All Hgb F at birth; Transfusion dependant; | B thalassemia major (Cooleys anemia) |
| Impaired heme synthesis; Ringed Sideroblast; Pappenheimer bodies; 15-20% develop leukemia | Sideroblastic Anemia |
| Goats Milk; Watery diarrhea, flatulence, failure to thrive | Folate Deficiency |
| Megaloblastic anemia; Strict Vegan or Lack of intrinsic factor; Neurologic: decreased proprioception, paresthesia, spastic ataxia, dementia | B12 deficiency |
| Hypersegmented neutrophils | B12 deficiency |
| Defect in Ankyrin, spectrin, band 3 or protein 4.2; MCHC increased | Spherocytosis |
| Decreased glutathione; Fava beans, sulfonamides, anti-malarials, moth balls; X-linked Autosomal Recessive; | Glucose-6-Phosphate Dehydrogenase Deficiency |
| Reduced glycolysis; Very High Hemolysis; RBC enzyme deficiency; Autosomal recessive | Pyruvate Kinase Deficiencies |
| Acquired stem cell defect; PIG-A defect; CD 59: HRF - homologous restrictive factor; CD 55: DAF - decay acceleration factor; Symptoms: hematuria, thrombocytopenia, venous thrombosis, aplastic anemia at night | Paroxysmal Nocturnal Hemoglobinuria |
| Treatment for PNH | Glucocorticoids; Monoclonal anti-C5; Stem-cell Transplant |
| Chromosome 11 - point mutation resulting in substitution of adenine for thymine resulting in valine instead of glutamic acid | Sickle Cell Mechanism |
| Upregulation of surface adhesion molecules results in adherence to endothelium | Pathophysiology of Sickle Cell diease |
| Organism that causes aplastic crisis in sickle cell disease | Parvovirus B19 |
| Therapy of choice for acute CVA from sickle cell crisis | exchange transfusion |
| Mutation in ELA2 gene; Cycles at 14 to 28 days; repeated fever, aphthous ulcers, adenopathy, skin infections, pneumonia; Nadir of peripheral neutrophils is <200 | Cyclic Neutropenia |
| Inheritance pattern of cyclic neutropenia | Autosomal Dominant |
| <10% neutrophils - chronically; Risk of MDS/AML <20%; Maturation arrest at promyelocyte stage; Autosomal recessive | Severe Congenital Neutropenia (Kostmann's Syndrome) |
| SBDS gene; Neutropenia, exocrine pancreatic failure, short stature, metaphyseal dysostoses; Recurrent skin and sinopulmonary infections; SCT is curative; Autosomal Recessive | Shwachman-Diamond Syndrome |
| Short limb dysostsis; Defect in cell-mediated immunity associated with severe VZV infections; Autosomal recessive inheritance, primarily amish | Cartilage Hair Hypoplasia |
| Self limited isoimmuizations with foreign paternal neutrophil antigens; Wears off in 6-12 weeks | Neonatal Isoimmune Neutropenia |
| Diagnosis of exclusion; asymptomatic neutropenia | Benign Neutropenia of Childhood |
| Delayed umbilical Cord seperation 4-6 weeks; Defect CD18 locus on Chromosome 21; Elevated WBC >20K; Autosomal Recessive | Leukocyte Adherence Deficiency |
| Defective superoxide burst; 2/3 X linked; 1/3 autosomal recessive; Recurrent skin, osteomyelitisi, perirectal, pneumonia from catalase producers | Chronic Granulomatous Disease |
| Genetic defects: p22-phox, p47-phox, p67-phox, gp-91phox NBT test | Chronic Granulomatous Disease |
| Oculocutaneuous albinism, neutropenia, prolonged bleeding time, NK cell dysfunction, frequent infections; Large graunules in neutrophils and eosinophils; Autosomal Recessive | Chediak-Higashi Syndrome |
| Elevated IgE >1,000; Recurrent infections; Eczema and allergies with boils | Hyperimmunoglobulin E syndrome (Job Syndrome) |
| MAHA, neurologic manifestations, renal dysfunction, thrombocytopenia, fever | TTP Thrombotic Thrombocytopenia Purpura |
| Acquired autoantibody in TTP | ADAMSTS13 |
| Treatment for ITP | IVIG, Anti-D, Corticosteroids Splenectomy |
| Maternal IgG crosses the placenta and attaches to fetal platlets PLA1 | Neonatal Alloimmune Thrombocytopenia |
| Megakaryocytic bone marrow- thrombocytopenia; Absent radii with normal thumbs; Milk protein allergy; Autosomal recessive | Thrombocytopenia absent radii syndrome (TAR) |
| Small platelets; Eczema and immunodeficiency; SCT curative; X-linked defect in WAS protein | Wiskott-Aldrich Syndrome |
| Minor autosomal dominant defect on Chromosome 22; Thrombocytopenia with uniformly large platelets; Neutrophils have large blue Dohle-like bodies | May-Hegglin Anomaly |
| Deficiency of glycoprotein Ib; Giant platelets; Abnormal platelet agglutination; Autosomal Recessive | Bernard-Soulier Syndrome |
| Abnormally formed GPIIb/IIIa; mucocutaneous bleeding; Autosomal recessive | Glanzmann's Thrombocytopenia |
| Treatment for Glanzmanns thrombocytopenia | Platelet transfusion even if normal platelet count |
| Decreased granules in platelets; Oculocutaneous albinism; Puerto Rican; Autosomal recessive | Hermansky-Pudlak Syndrome |
| Immune response to stem cells - idiopathic in majority; drugs, toxins, radiation, CMV, EBV, hepatitis, HIV, parvovirus Anemia | Aplastic Anemia |
| Treatments for Aplastic Anemia | Matched sibling SCT 80% cure cyclosporin A + anti-thymocyte globulin |
| defective DNA repair; Short stature, abnormal thumbs and radii, microcephaly, cafe-au-lait spots, renal abnormalitiies | Fanconi Anemia |
| Mutation sites in Fanconi Anemia | FANCA and FANCC |
| Mortality from fanconi anemia | head and neck cancers, squamous cell carcinoma, and hepatic malignany Risk of AML is 15% |
| Hgb 6-8; Age 1-3 yo; insidious onset; normal MCV and Adenosine deaminase; self-limited | Transient Erythroblastopenia of Childhood (TEC) |
| Anemia and reticulocytopenia; Decreased RBC precursors; <1 yo but may be diagnosed later in life; Thumb abnormalities - bifid thumb, short stature, webbed neck, congenital heart disease, mental retardation | Diamond-Blackfan anemia |
| Immune dysregulation; Punched out lesion on plain radiography; Usually >5 yo; Treat with curettage, intralesional steroids, or focal radiation | Langerhans cell histiocytosis |
| Bone disease plus skin, DI, exopthalmos, floating teeth, gingiva, chronic otitis externa | Mutifocal Histiocytosis |
| Fever, HSM, lung, marrow, skin, and FTT; infantile presentation | Systemic Histiocytosis |
| Fever, HSM, CNS symptoms, cytopenias, coagulopathy, hyperlipidemia, hyperferritinemia >1000K; Acquired due to infection; Familial due to perforin gene mutation | Hemophagocytic Lymphohistiocytosis (HLH) |
| Prolonged PTT, family history, normal PT, excluded vWD X-linked recessive | Factor VIII deficiency |
| Treatment of factor VIII def | Recombinant factor VIII; DDAVP; then consider FFP and cryo (discouraged) |
| Inheritance pattern of factor IX def | X-linked; Same therapy except using factor IX replacement |
| Most common bleeding disorder; Autosomal dominant; Prolonged epistaxis, menorrhagia, mucosal bleeding, bruising, or post-surgical bleeding; prolonged PTT | von Willebrand disease |
| Hageman Factor; autosomal recessive; Normal PT; Markedly prolonged PTT; No clinical bleeding | Factor XII deficiency |
| Clot then start bleedin 12 - 24 hours later; Euglobulin lysis test - expose clot to 1M urea | Factor XIII deficiency |
| Prolonged PT, PTT; Thrombocytopenia; MAHA; Low fibrinogen; D-Dimer increased; Schistocytes | DIC Disseminated intravascular coagulation |
| Decreased production in factors II, VII, IX, X, protein C and S Prolonged PT more than PTT | Vitamin K Deficiency |
| Most common childhood cancer | Acute lymphoblastic leukemia |
| Syndromes associated with ALL | Trisomy 21, ataxia telangiectasia, chromosomal breakage syndromes - Bloom and Fanconi |
| In ALL: intial WBC <50K; age >1 but less than 10; t(12,21), trisomy 4, 10, 17, t(4,11); pre-B; rapid early responder; minimal residual disease (MRD) | Good prognosis in ALL |
| In ALL: Initial WBC > 50K; Age <1 or >10; T-cell or mature b-cell; t(9,22); Slow response or persistent MRD | Poor prognosis in ALL |
| Induction therapy in ALL | vincristine, l-asparaginase, corticosteroids,(standard) +/- anthracyclines (high-risk) |
| Maintenance therapy in ALL | Methotrexate, 6-mercaptopurine, corticosteroids, vincristine IT MTX, cytarabine, steroids depending on protocol; CNS irradiation only for high risk |
| Associated syndromes with AML | Trisomy 21, fanconi, bloom, kostmann, NF-1, PNH, Etoposide( VP-16) exposure, ionizing radiation |
| FAB classification: M3 | Acute promyelocytic leukemia; t(15,17); DIC; ATRA - all trans-retinoic acid; 90% survival |
| FAB classification: M2 | Acute myeloblastic leukemia with granulocyte maturation; t(8,21) |
| FAB classification: M7 | Acute megakayoblastic leukemia Trisomy 21 |
| FAB classification: M 5 | Acute monocytic leukemia; del 11q2, t(9,11), t(11,19); CNS disease, gingival hyperplasia |
| Induction therapy for AML | corticosteroids, cytarabine, anthracycline |
| Triphasic: chronic, accelerated, blast crisis; t(9,22) BCR/ABL gene fusion; Massive leukocytosis with rare blasts | Chronic Myelogenous Leukemia |
| Therapy for CML | tyrosine kinase inhibitor |
| Splenomegaly, lymphadenopathy, cutaneous lesions, hemorrhagic symptoms; NF-1, ras gene mutation; monosomy 7 maybe present in 30% | Juvenile Myelomonocytic leukemia (JMML) |
| Therapy for Hodkins | Vincristine, bleomycin, cyclophosphamide, etoposide + XRT |
| Associated with Non-hodgkin lymphoma | Ataxia-telangiectasia, HIV, imminodeficiencies |
| mature b-cell tumor, often EBV related, often MALT-associated, fastest growing malignancy - doubling time of 18 hours; highest rate of tumor lysis | Burkitt's lymphoma t(8,14) |
| Most common non-CNS solid tumor in children; neural crest cells; elevated HVA/VMA; raccoon eyes; opsoclonus/myoclonus | Neuroblastoma |
| Loss of function of tumor suppressor genes WT-1 and WT-2 from chromosome 11- | Wilms Tumor |
| Associated with wilm's tumor | WAGR: aniridia, GU abnormalities, MR; Beckwith-Wiedemann: organomegaly, omphalocele, hemihypertrophy; Denys-Drash: pseudohermaphroditism, nephropathy; Perlman syndrome: macrocephaly, macrosomia, organomegaly, abnormal facies |
| Most common soft tissue tumor; associated with Li-fraumeni (p53) syndrome | Rhabdomyosarcoma |
| Therapy for Rhabdomyosarcoma | vincristine, actinomycin-D, cyclophosphamide |
| Associated with Osteosarcoma | Hereditary retinoblastoma; Li-Fraumeni syndrome, Rothmund-Thompson syndrome: short stature, telangiectasia, small hands/feet, hypoplastic thumbs; paget's disease, ionizing radiation, alkylating agents |
| Bone or soft tissue tumor derived from neural crest cells; lytic onion skinning, diaphysis more commonly affected | Ewing's sarcoma |
| Benign tumors of bone | Osteoblastoma; Osteochondroma; chondroblastoma; Osteoid osteoma; Enchondroma |
| Most common posterior fossa tumor; low grade; presenationwith ataxia, headache, comiting | Cerebellar astrocytoma |
| Vision changes, increased ICP, delayed puberty, neurobehavioral changes | Craniopharygioma |
| Leukocoria, strabismus, eye pain, headache, heterochromia, spontaneous hyphema, acute closed-angle glaucoma | Retinoblastoma |
| Endoderm, mesoderm, ectoderm | teratoma |
| Most common malignant germ cell tumor; gonadal, sacrococcygeal, or midline, B-hcg absent; alpha-FP reliable | Endodermal sinus tumor (yolk sac tumor) |
| Compnent of mixed germ cell tumor; B-HCG but no alpha-FP; aggressive | Choriocarcinoma |
| Most common liver malignancy in children; elevated alpha fetal protein | Hepatoblastoma |
Created by:
faithsfocus
Popular Medical sets