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Pediatric Hem/Onc

Pediatric Board Review 2011

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