1 Pediatric Board Review Course Pediatric Hematology/Oncology Kusum Viswanathan, MDChair, Department of PediatricsDirector, Divn of Pediatric Hematology/OncologyBrookdale Univ Hospital and Medical Center
2 Question6 week old term infant referred for anemia. Hb 7.5, Retic 2 %.Mother O+, Baby A -, Direct Coombs +Cord blood Hb 14.2 g/dL.Jaundice of 15mg/dL at 48 hours of life, recd photo Rx and discharged at 5 days.No complaints, pale, Bili 3.5, Direct 0.5.Blood smear shows spherocytes
3 Most likely explanation for the anemia G 6 PD deficiencyHereditary spherocytosisPhysiologic anemiaABO incompatibiltyRh hemolytic disease6
4 Newborn –anemia- important Hemoglobin at birth is 17 g/dl, MCV over 100.Falls to by 6 weeks of age- nadir of physiologic anemiaAnemia at birth could be :Hemorrhage, may not have had time to mount a retic responseAcute hemorrhage- pallor and tachypneaLook at MCV- low MCV-suggestive ofchronic feto-maternal hemorrhageAlpha Thalassemia trait.Kleihauer-Betke- Hb F resistance to acid elution
5 The treatment of choice for alloimune neonatal thrombocytopenia is random platelet transfusionIVIGSteroidsExchange transfusionWashed maternal platelets6
6 Immune thrombocytopenia Auto-immune: Pregnant women with ITP/Hx of ITPPassive transfer of antibodies (IgG) from mother.Even when mother has a normal platelet count (Splenectomy)Nadir-few days; Platelets < 50,00 have 1% risk of ICH.IVIG to mother, Fetal platelet counts, C sec, US, IVGG to babyAllo or Iso-Immune: Normal platelet count in motherSimilar to Rh disease; PL A1 antigen/ Zw-a negative mother.97% of population is PL A1 positiveSensitization early in pregnancyPlt function defect because Anti-PL-A1 interferes w/aggregation.Severe bleeding more likely; first born affectedRecovery in 2-3 weeksMother’s washed (PLA1 neg) platelets; IVIG; Ultrasound; Steroids
8 15 months old girl presented in ER with h/o URI, and scattered petechiae and ecchymoses over the body and lower extremities.Physical exam normal, no hepatosplenomegaly. WBC-14,000, Hb 12.8, Plts-5,000, Diff: Normal - Next stepperform a bone marrow aspirate to confirm the diagnosisNon-accidental injury; skeletal survey to rule out bony fracturestreatment with either IVIG or anti-DAdminister platelet transfusion6
9 Acute ITP Usually acute onset; immune mediated; post viral Peak 2-5 years of age,PE –no lymphadenopathy (LN), hepatosplenomegaly.CBC- other cell lines normal, large plts on smearTreat if plt< 10,000 or wet ITP,Treat- IVIG best response, hours; blocks Fc receptors,SEAnti-D (WInRho)- Rh+ ,hemolysis, quick responseSteroids good response, block phagocytosis, reduces antibodies, SE, inexpensive, need BMBM- Increased megakaryocytes, otherwise normalChronic- If >6 months, F>M, older, unpredictable prognosis
11 Acrodermatitis enteropathica Ataxia telangiectasia Atopic dermatitis A 2 year old boy presents for evaluation of a chronic pruritic eruption. H/o recurrent epistaxis, otitis media, and pneumoniaP/E reveals erythematous, slightly scaling patches on the trunk and in the antecubital and popliteal fossae. Petechiae too- most suggestive ofAcrodermatitis enteropathicaAtaxia telangiectasiaAtopic dermatitisLangerhans cell histiocytosisWiskott-Aldrich syndrome6
12 Large platelets Normal platelet 7-10 days Large platelets: ITPMay Hegglin (Dohle bodies in neutrophils, Plt function normal).Bernard Soulier syndrome (AR, Plat function disorder).Small platelets: Wiskott Aldrich syndrome ( X-linked, recurrent infections, eczematoid rash, platelet dysfunction)
13 Platelet function defects Normal platelet numberAbnormal aggregationGlanzmann thrombasthenia--- AR, Bleeding disorder, check h/o consanguinityafibrinogenemiaAbnormal adhesion- Bernard-Soulier, VWDIneffective ThrombopoiesisMayHegglin Anomaly (Large platelets)Disorders of Secretion: Storage pool, gray platelet syndromeHermansky Pudlak Syndrome:AR, Decreased dense granules, In Puerto RicansOculocutaneous albinism
15 AnemiaAn 18 month old girl brought in for pallor. Normal diet and PMH. She is alert, interactive, only pallor, normal vital signs, No hepatosplenomegaly, lymph nodes or bruises.CBC- Normal WBC, Plt, Hb 4.5g/dl, MCV 74,AnemiaReduced productionIncreased destructionLossWhat else do you want??
16 Reticulocyte count Normal/Low- reduced production Iron deficiency anemia- MCV will be lowALL (leukemia)- other findings, LN, HSMDiamond Blackfan anemia-TEC: Over 1 year of age, Pallor, transient rbc production failure, recovers, MCV and Hb F high during recovery, rbc transfusion, rbc ADA normal .
18 A 2 month old girl with a history of ventricular septal defect and horseshoe kidney presents for a health maintenance visit.Her parents are concerned that she is not feeding well, appearing to become “tired” soon after beginning to breastfeed. She is tachycardic and appears pale. Her hemoglobin level is 3.8 g/dL. Peripheral smear reveals macrocytic red blood cells. The bone marrow aspirate shows normal cellularity of the marrow with markedly decreased erythroid precursors. Which of the following findings is most likely to be identified during additional physical examination of this patient?Multiple superficial hemangiomasCutis aplasiaBifid thumbsSpeckled white rings in the periphery of the irisPosterior parietal hair whorl
19 Iron deficiency Lead poisoning Sickle cell disease Thalassemia trait Microcytic anemia is a characteristic laboratory abnormality of all listed diseases exceptIron deficiencyLead poisoningSickle cell diseaseThalassemia trait6
21 QuestionA blood smear taken from a toddler shows microcytic hypochromic anemia. Iron supplementation therapy is started. When will the reticulocyte response be at maximum?a. 1-2 daysb. 5-7 daysc daysd. 3-4 weekse. about 6 weeks
22 Iron deficiency questions Low MCV, low MCHC, low retic, RDW can be normal, Low Iron, Incr TIBC, Transferrin low, Ferritin lowCauses: Inadequate dietary intakeToddlers, too much milk, less solids, Breast fed need iron supplementsBlood loss: Menstrual, GI tract, Meckels, EpistaxisD/D:Thalassemia trait- MCV much lower in prop to anemiaAnemia of chronic disease- low Fe, low TIBC, normal /high Ferritin.
23 Question- Beta Thalassemia 3 year old comes for a routine check and found to be anemic. P/E Normal. CBC Hb 8.9, MCV 58, Iron levels are normal. Hemoglobin electrophoresis is done and shows Beta Thalassemia trait. All are true except:The MCV is always lowFetal hemoglobin is < 2%Hemoglobin A 2 is > 3.5%Does not respond to IronRDW is usually normal
24 Beta Thalassemia Minor Quantitative defect in globin chainsReduced production of Beta chainsHb electrophoresisHb A- 2 Alpha, 2 BetaHb F- 2 Alpha, 2 GammaHb A2- 2 Alpha, 2 DeltaExcess Alpha combines with Gamma or Delta- Increased Hb F and A2.Smear abnormalities significant even with MILD anemia.AnemiaLow MCV, normal RDW, normal reticSmear shows aniso and poikulocytosis, target cells, microcytes, misshapen cells, basophilic stipplingHb Electrophoresis: Increased Hb A2 and/or F.Normal iron studies, no response to iron
25 Beta Thalassemia Major No production of Beta chains- Chromosome 11Autosomal recessive25 % chance with each pregnancyPre-natal testing for carriersChorionic villous sampling for diagnosisTransfusion dependent-allows for normal developmentPen Prophylaxis, Anti oxidantsSplenectomy after age 5Iron overload- inherent and transfusionNeed chelators
26 Question4 year old male with no complaints. Routine CBC showed a Hb9.8, MCV 62, RDW 12.5 , Retic 0.2%, Normal Iron studies, Hemoglobin electrophoresis was normal with normal Hb A2 and Hb F. You suspect Alpha Thalassemia. All of the statements are true except:It is carried on 4 allelesThe severe form is Hydrops fetalisHemoglobin H disease occurs when 3 alleles are affectedDiagnosed by newborn screening when a “fast moving” hemoglobin is noted.Fast moving hemoglobin is made up of a hemoglobin with 4 beta chains
27 Thalassemia- Alpha Reduced Alpha chains 4 types- carried on 4 allelles. (xx/xx)One absent- Silent carrier (x-/xx)2 absent- Alpha Thal trait (xx/- - or x-/x-)3 absent- Hb H disease (x-/- -) Has 4 excess Beta chains)4 absent- Hydrops fetalis (- -/- -)NB period: Excess Gamma chains form Hb Barts- FAST moving Hb on Newborn screening
28 Case3 year old patient is brought to the ER with complaints of feeling very tired over the past 3 days.Patient is pale, jaundiced with the spleen tip palpable.CBC Hb 5, Retic 5 %, LDH Increased,What does this sound like??
29 QuestionA previously well African-American child visited Africa and was given malarial prophylaxis. He experienced pallor, fatigue, and dark urine. His hemoglobin level decreased from 14.8 to 9 g/dL.SMEAR
30 Hereditary spherocytosis Sickle cell disease Hepatitis G6PD deficiency An African-Am child visited Africa & received malaria prophylaxis. He hasHereditary spherocytosisSickle cell diseaseHepatitisG6PD deficiency6
32 Children with hereditary spherocytosis have all of the listed conditions except: positive Direct Coombssplenomegaly, gallbladder stonesabnormalities in spectrin and /or ankyrinincreased MCHCabnormal osmotic fragility test.6
34 HS- with severe anemiaA 6 year old girl who has hereditary spherocytosis presents with a 1 week history of fever. Physical examination reveals abdominal pain, vomiting, fatigue and pallor. Her hemoglobin is typically about 10 g/dL with a reticulocyte count of 9%, but now, her hemoglobin is 4 g/dL and the reticulocyte count is 1%. Her bilirubin is 1 mg/dL. Of the following, the MOST likely cause for this girl’s present illness is infection withCoxsackie virusParvovirus B19Epstein-Barr virusHepatitis A virusInfluenza A virus
35 HS- with severe anemia Coxsackie virus Parvovirus B19 Epstein-Barr virusHepatitis A virusInfluenza A virus6
36 Newborn Screening Questions You get a call from a frantic parent because she received a letter from the State regarding her baby’s test results on NBS.FS- SS disease, S-B0 Thal, Sickle cell w/ HPFH.FSA- Sickle B+ thal, Sickle cell traitFSC- SC diseaseFAS- Sickle cell traitFAC- Hb C traitFAE- Hb E traitFE - Hb EE disease, E-Thal
37 Sickle cell QuestionsHemolysis- life span days. Abnormal cell shape, abnormal adherence to endothelium, decreased oxygenation, Increased polymerization.Symptoms start by 2-4 months of age.Hb electrophoresis, S >75 %.Penicillin daily until age 5.Prevention of pneumococcal infections-asplenic.PPV (Pnu-23) age 2, 5Meningococcal vaccine early two doses and every 5 years.Folic acid daily
38 Sickle cell questions Vaso-occlusive crisis New infant born in another country presenting with swollen hands---DactylitisAplastic crisis:low Hb, low retic, Secondary to Parvovirus B 19 infection.Splenic sequestration crisis:Sudden enlargement of the spleen in SS or older SC patientRx- TransfuseTeach spleen palpationSplenectomy
39 Associated alpha thalassemia trait Low hemoglobin The mother of a 10 month old baby with SS disease asks you about prognostic indicators. All of the following indicate likelihood of more severe disease except:High WBCAssociated alpha thalassemia traitLow hemoglobinRepeated episodes of dactylitis6
40 Sickle cell Acute Chest Syndrome True statements include all except:1. Presents with a new infiltrate on X-ray2. Due to infarction, infection, BM fat embolism3. Treat with antibiotics to cover pneumococcus, Mycoplasma, Chlamydia4. Treat with bronchodilator, Incentive spirometry, transfusion, Steroids (controversial).5. Intensive hydration is important
41 TCD- Transcranial Doppler A routine TCD on a 4 year old patient with SS disease shows a Cerebral blood flow (CBF) of 210 cm/second.What is the next step?STOP studies- STOP I and II
42 Continue indefinitely the child reaches 18 years According to the STOP protocol all children with abnormal TCD require enrollment in hypertransfusion protocol till (choose one)Repeat TCD is normalContinue indefinitelythe child reaches 18 yearsMRA/MRI are reported normal6
43 Sickle cell and Stroke Questions Affects 10 % of patientsInfarctive stroke (younger patients)Hemorrhagic stroke (older)STOP I study established the role of yearly TCD (transcranial doppler) to measure cerebral blood flow velocity as a tool for determining stroke risk.Transfusion therapy as current therapy for high risk patients (CBF> 200cm/sec)Reversal of CBF velocity is not sufficient to stop transfusion therapy. (STOP II)
44 Sickle cell and Hydroxyurea FDA approved for adultsStudies in children demonstrated efficacy and safetyIncreases hemoglobin F levelIncreases hemoglobinDecreases WBC – ancillary effectReduces number of ACS, VOCHydroxyurea is recommended by the hematologist for patients who have recurrent vaso-occlusive crises, acute chest syndrome.
45 A healthy 5 year old boy- 2 day hx of fever, P/E normal No hepatosplenomegaly, LN, no focus of infection. CBC WBC 3, Neutrophils 25 %, Hb 12, Platelet 200X109/L, ANC 750. Most appropriate step isAmoxicillin for 10 daysG- CSF for 10 days.BM aspirateRefer to a hematologistRepeat CBC in 1-2 weeks6
46 CaseA 2-year-old boy has had several 10-day-long episodes of fever, mouth ulcerations, stomatitis, and pharyngitis. These episodes have occurred at about monthly intervals. Absolute neutrophil counts have been 50/mm³on day 1 of each illness, 500/mm³ on day 10, and 1,500/mm³ on day 14.Among the following, the MOST likely cause for the findings in this patient isA. chronic benign neutropeniaB. cyclic neutropeniaC. Schwachman-Diamond syndromeD. severe congenital neutropeniaE.. transient viral bone marrow suppression
47 Abnormal BleedingEpistaxis unrelieved by 15 minutes of pressure, both nostrils, requiring an ER visit, documented drop of hemoglobin.Menstrual periods( amount, pads, duration)Bleeding after procedures (circumcision, dental extractions, T and A-delayed bleed)Ecchymoses/bruising inconsistent with the degree of trauma
48 Bleeding patient Physical Examination: Type of bleeding: Superficial or deepBruises, PetechiaeEpistaxis, Gum bleeding, Excessive menstrual bleedingSite of bleedingBleeding into the joints and soft tissuesLook for evidence of shockMedication history (Aspirin, NSAIDS)
49 Lab studies (What do they measure?) CBC and Peripheral smearPT, INR and PTTPT - Factor VII, common pathwayPTT- Factor VIII, IX, XI, XII, common pathwayMixing studies (Inhibitors and deficiency)Specific coagulation factor assays
51 Questions on Circulating anticoagulant Mixing studyIf PT or PTT is prolonged, ask for a mixing study.Mix patient plasma with equal amount of normal plasma, the test will normalize if the abnormal result is because of a deficiency in factor.If there is an anticoagulant, it will not normalize or even if it does, it will become abnormal again after incubation.
52 A healthy 2-day-old boy born at term undergoes circumcision. Bleeding noted at the site 10 hours after the procedure and increased steadily over the past 4 hours. Findings on exam are unremarkable except for bleeding along 2 to 3 mm of the surgical site; no petechiae or purpura.Disseminated intravascular coagulationFactor VIII deficiency hemophiliaImmune thrombocytopenic purpuraNeonatal alloimmune thrombocytopeniaVon Willebrand disease6
53 Hemophilia Questions Factor VIII deficiency (Hemophilia A)-85% X-linked recessive, Carriers asymptomaticSevere<1%, Moderate 1-5, Mild 6-30 %Treat Recombinant Factor VIII 1unit/kg raises factor level by 2 %. Half life 12 hrs. Joint bleeds need100%, muscle bleeds 50 %.DDAVP for mild cases.30 % develop inhibitors after infusions with concentrate (Approx 50 infusions)Factor IX deficiency (Hemophilia B)X-linked recessive, less common
54 A patient with Hemophilia A has asked you about the possibility of his children being affected by the disease.The partner is normal.There is a 50 % chance that his sons will have the disease.There is a 50 % chance that his daughters will be carriersThere is a 100 % chance that his sons will have the diseaseThere is a 100 % chance that his daughters will be carriers
55 Case13 year old girl just started her periods and has been bleeding for the past 16 days. She has used 14 pads a day and is tired. Her vital signs are stable, Hb 9.5, PT, PTT normal.The mother had heavy periods and her 6 year old brother has nose bleeds for the past 2 years.Likely to have:
56 Questions on Von Willebrand’s Disease 1-2 % of population Type I - 80 % of cases; Quantitative defect, Autosomal dominant (AD)Type %, Qualitative defect2A, 2b (thrombocytopenia), 2M, 2N (AR)Type 3 - Severe (similar to hemophilia A)Autosomal recessive (AR)DDAVP- Releases VWF from endothelial cells and stabilizes Factor VIIISE: Water retention, Tachyphylaxis, hyponatremia.For mild Hemophilia, Type I VWD, 2Contra-indicated in Type 2BRx-Plasma derived VWF containing concentrates
57 ThrombophiliaA 14 year old male presents with chest pain and difficulty breathing. He notes that his right calf has been swollen for the last 3 days and he has difficulty placing his foot on the ground. P/E Pain on dorsiflexion, Air entry reduced. CXR and EKG are normal. VQ scan shows a filling defect and a diagnosis of DVT and pulmonary embolism is made.What are the important questions on history?History of DVT in family membersH/o recurrent late miscarriages in mother and her sisters.H/o trauma and precipitating factors
58 The most common cause of familial predisposition to thrombosis is Hemophilia antibodiesProtein C deficiencyProtein S deficiencyFactor V Leiden mutationAntithrombin III deficiency
59 Tests performed on donated units of blood all units are tested only for hepatitis B and Call units are tested only for human immuno-deficiency virus (HIV)all units are tested for HIV, hepatitis B, and hepatitis Call units are tested for HIV, hepatitis B, hepatitis C, sickle cell trait, cytomegalovirus, and Epstein-Barr virusonly units obtained from donors who have one or more risk factors are screened for HIV, all units are tested only for hepatitis B and C6
60 Questions on Transfusion CMV negative- give leukocyte reduced.Irradiated products- To prevent GVHDWashed cells-Phenotype matchedTo prevent allo-immunizationSickle negative
62 Multifocal osteomyelitis A 6-year-old girl has aching in her arms, legs, and back for > 2 weeks.Results of laboratory tests include hemoglobin, 9.4 g/dL; white blood cell count, 5,600/mm³ with no abnormal cells noted on smear; and platelet count, 106,000/mm³.Radiographs of long bones reveal osteolytic lesions and radiolucent metaphyseal growth arrest lines.ALLAplastic anemiaGaucher diseaselead poisoningMultifocal osteomyelitis6
63 ALL (Acute Lymphoblastic leukemia) 75% of all LeukemiasCan present with generalized bone pain, fatigueBruising, nose bleedsUnusual fevers, infectionLymphadenopathy, hepatosplenomegaly
64 ALL- Should know (Acute Lymphoblastic leukemia) Abnormal to see blasts in the peripheral smearDiagnosis: >25 % blasts in the BM.Normal marrow has < 5 % blastsSingle most common childhood cancer (29% of all childhood cancers); cases per yearPeak age 2-5 yearsMore likely in Trisomy 21, Ataxia-Telangiectasia, Bloom syndrome, Kostmann’s, Monosomy 7, DBA, Fanconi anemia, 20% risk in twin if age <5yrs
66 ALL- Prognosis Prognosis: WBC, Age, Cytogenetics good if hyperdiploidy (>50),trisomy 4,10,17, t(12,21)Bad if hypodiploid, Ph. chrom t (9,22),t(4,11), t(8,14)Immunophenotype: Pre-B good, B and T-worseEarly response, Minimal residual disease (MRD)Infants: 50 % survivalEarly relapse is a poor sign
67 Down Syndrome and Leukemia 10-20 fold increaseDS: 400 fold Increase in M7 AMLSuperior response to Rx of AMLTransient Myeloproliferative disorder in newborn which resolves within 3 months.
68 Acute Myeloid Leukemia (AML) 20 % of all leukemiasIncreased incidence in < 1 year of ageHigher incidence:Downs, Fanconi, Bloom, DBA, Kostmann, Neurofibromatosis I, Schwachman-DiamondChloromas– solid collection in bone/soft tissuesTypes: M0-M7, commonest M2DIC in M3- (Promyelocytic- APML)M7- Downs syndrome
69 Question Hodgkins16 year old male presents with a painless swelling in the supraclavicular region. Biopsy shows Hodgkins disease Stage 2. All are true except:1. Presence of “B” symptoms is worse for prognosis2. B symptoms are fever, increasing size of mass3. Staging depends on whether it is on one or both sides of the diaphragm4. Sperm banking should be done5. Second malignancy can occur in patients who receive combination chemo and RT
70 Question4 year old boy presents with a 6 day history of bilateral, rapidy progressive facial swelling. There is no history of associated trauma or upper respiratory symptoms. On P/E, there is significant swelling below both eyes and of both cheeks. The angle of the jaw is maintained. On examination of the oral cavity, both the soft and hard palate are swollen as are the gingiva. The teeth are loose and inverted due to the degree of gingival swelling. A CT of the sinuses reveals extensive bilateral opacification of both maxillary sinuses. LDH was 1,850 U/L. Which of the following is the most likely to cause of this patient’s physical findings? Human herpesvirus 6CytomegalovirusCryptosporidium hominisParvovirus B19Epstein-Barr virus
71 Burkitt’s Lymphoma Endemic Burkitt’s African type, head and neck, jaw 95 % chance of EBVSporadic Burkitt’sAbdomen15-20 % chance of EBVt (8;14)Immunotherapy: Anti-CD 20 monoclonal antibody; (Rituximab)
72 Question5 yr old boy with progressive vomiting, headache, unsteady gait and diplopia for 4 weeks. MRI shows a contrast enhancing tumor in the 4th ventricle with obstructive hydrocephalus.
73 Brain Tumors- Important to know 20% of all malignancies in childrenAge 3-7 yearsMost often infratentorialSx: Persistent vomiting, headache, gait imbalance, diplopia, ataxia, vision loss, school deterioration, growth decelerationAssociations with Inherited Genetic disorders:Neurofibromatosis, Tuberous sclerosis, Von-Hippel-Lindau disease, Li-Fraumeni (glioma), Turcot syndrome
74 Medulloblastoma - most common CNS tumor Trt: Resection, Craniospinal RT, ChemoPrognosis: Age, large size, degree of resection, dissemination, histology.
75 Wilms TumorAn 18-month-old girl is being evaluated because her mother thinks her abdomen seems “full.” Physical examination reveals an abdominal mass. Ultrasonography identifies a solid renal mass. At surgery, a stage I Wilms tumor is found.
76 Stage I Wilms- Chance of 4-year survival is CLOSEST to B. 45%C. 60%D. 75%E. 95%6
77 Wilms Tumor- Important Presentation: No Sx, HT, Hematuria, MalaiseHistology: favorable(FH) vs unfavorable (UH)Staging: I-local, II-excised, III-residual, IV-metastases, V -bilateralTreatment: Nephrectomy, Chemo-all, St I-II-2 drugs-18 weeks, St III-IV- 3 drugs+ RTPrognosis:FH: > 90% at 2 yearsUH: < 60% at 2 years
78 Polydactyly Aniridia Cryptorchidism Hemihypertrophy Congenital anomalies associated with Wilms’ tumor include all of the following exceptPolydactylyAniridiaHemihypertrophyCryptorchidismDenys-Drash syndrome6
79 A 9 year old previously healthy girl manifests progressive painless proptosis and decreased visual acuity of the left eye during a 2 month period. The most likely diagnosis is:a. Pseudotumor of the orbitb. Trichinosisc. Retinoblastomad. Rhabdomyosarcomae. Orbital cellulitis
80 Rhabdomyosarcoma Painless non tender mass 60% under age 6 Sites: head & neck, GU, Extremities, mets lungs.Majority sporadic, associations: B-W, Li Fraumeni, NF 1Types:Embryonal 70%, better prognosisAlveolar 30 %, trunk, worse prognosisTreatment: Surgery, Chemo, local control RT
81 MassThe mother of a 22-month-old boy reports that he has been fussy and tired. Findings on physical examination confirm the presence of a nontender rt upper quadrant mass. Bilateral periorbital ecchymoses also are noted.Of the following, the MOST likely cause for these findings isA. multicystic kidney diseaseB. neuroblastomaC. non-Hodgkin lymphomaD. HepatoblastomaE. Wilms tumor
82 All statements are true about Neuroblastoma except: Most common extra-cranial solid tumorPrognosis better with N-myc oncogene amplification and tumor diploidy (DNA index 1)Most common cancer in the first year of lifeFrequent in <4 years, 97 % cases by 10 yearsMost commonly diagnosed as Stage III or IV6
83 Case A 16 year old male comes in because he fell in the supermarket. P/E shows a small painless mass on the medial aspect of the knee.X ray shows a fracture and a lytic sunburst pattern. (periosteal elevation)-OSXray shows an onion peel appearance- ESXray- radioluscent, sharp, rounded lesion < 2cm with reactive sclerosis- Osteoid Osteoma
86 Osteogenic Sarcoma MRI, Bone scan, Biopsy, CT Chest for mets. Peak incidence- 2nd decadePredisposition: Hereditary retinoblastomas, Li-Fraumeni, Pagets, RT, Alkylating agents60 % near the knee (Metaphyses of long bones)History of fall, pain common symptom, mass, no systemic symptoms.Treatment: Sperm banking, Neo-adjuvant Chemotherapy, limb preserving surgery.
87 A 16 year old Caucasian female comes with complaints of chest pain and difficulty breathing for the past one week. She has had fever, wt loss over the last 2 months. She has reduced air entry and CXR shows a moth eaten appearance of one of the ribs and a pleural effusion.Biopsy is done and is consistent with Ewing’s Sarcoma
89 Retinoblastoma- need to know Presentation: Leukocoria (cats eye reflex), dilated pupil, esotropia, strabismusUnilateral 75 % (could be hereditary/non)60 % unilateral and non hereditary15 % unilateral and hereditary (RB1 mutation)Bilateral 25 %25 % are bilateral and hereditary, have RB1 mutationEarlier age, 11mos, Can develop in each eye separatelyHigher incidence of sarcoma, melanoma, brain tumors.
90 A child with ALL was started on Chemotherapy. She had a WBC 82,000, Hb 9gm, plt ct 45,000. She develops tumor lysis syndrome: Which depicts Tumor lysisK high, P high, LDH normal, Na highK high, P normal, LDH high, Na nlK normal, P high, LDH high, Na highK normal, P normal, LDH high, Na normalK high, P high, LDH high, Na normal.
91 Tumor lysis syndrome Rapid destruction of cancer cells. Release of intracellular ions, also Uric acid, can cause tubular obstruction and damage.Treatment: Allopurinol or Rasburicase early, hydration, alkalinization, diuretic therapy.
92 Spinal cord compression Presentation: back pain worse with movement, neck flexion, straight leg raising, valsalvaWeakness; partial/complete paralysis, IncontinenceLocal tumor extension or metastasisLymphomas, neuroblastoma, soft tissue sarcomasTherapy: Urgent treatment to relieve pressure and prevent permanent neurologic damage.Neurology/Neurosurgery consultHigh dose dexamethasoneEmergency radiation therapyLaminectomy
93 Questions on-Superior Vena Cava Syndrome Mass lesion obstructs flow through the SVCSymptoms- Dyspnea, edema of face, neck, upper extremitiesPeriorbital edema, conjunctival edema (itchy eyes)Dysphagia, resp distress, vocal cord paralysisTracheal compression: resp distress, wheezingDue to- NHL, Tcell ALL, Lymphoma,Secondary cause: occluded central venous catheter
94 Chemotherapy-Side effects that you need to know Anthracyclines: Cardiac toxicityVincristine: foot drop, peripheral neuropathyCisplatinum: kidney, deafnessMethotrexate, 6MP: Liver toxicityBleomycin: Pulmonary fibrosisAsparaginase: Pancreatitis, CoagulopathyEtoposide (VP-16): Secondary AMLCyclophosphamaide: Hemorrhagic cystitis(MESNA as Uroprotector) and Infertility
95 Fever, Neutropenia Single most important risk factor: ANC Organisms: Gram negative infection, Staph epi in catheter patientsMedication: Broad spectrum 3rd generation antibioticsAnti-fungal after 4 daysExamine patient thoroughly
96 16-year-old girl, completed therapy at age 8 for Hodgkins disease with Involved field RT and chemo. She now develops petechiae, purpura, lymphadenopathy and hepatosplenomegaly.Lab include: plt 12,000,Hb 8.0 gm/dL; and WBC 13,000/mm³. She has….AML as a second malignancyDisseminated varicellaDrug-induced ITPLate-onset aplastic anemia due to chemotherapyViral-induced ITP6
97 Follicle stimulating hormone Gonadotropin releasing hormone You are evaluating a 9 year old child for short stature. She was treated at 3 yrs of age for ALL, received cranial RT.Her height is < 5th percentile and she is Tanner stage I. Most likely to have an abnormal test ofGrowth hormoneEstradiolFollicle stimulating hormoneGonadotropin releasing hormoneThyroid stimulating hormone6
98 Need to know- Late effects of cancer therapy Hypothalamic pituitary axis is impaired; central hypothyroid and Adrenal insuff.RT doses higher in brain tumorGH is dose sensitive to the effects of RTAge related: < 5 years susceptiblePanhypopituitarism with higher dosesovarian failure with RT
99 A 16 year old boy is receiving chemo for rhabdomyosarcoma He recd a year of cycles of Vincristine, Actinimycin-D and Cyclophosphamide.Most likely endocrinologic late effect of this therapyGrowth hormone deficiencyHypothyroidismImpotenceInfertilityOsteoporosis6
100 Chemotherapy effects Chemotherapy with alkylating agents Females: less effects than malesnormal pubertyearly menopauseMales:irreversible gonadal toxicitysterility with azospermiaPuberty usually not affected (leydig cells)
101 The most common reason for the failure of hematopoietic stem cell transplantation is Veno-occlusive disease of the liverDisease recurrenceInfectionGraft vs. host diseaseGraft rejection6
102 GVHD ( Graft vs Host disease): All are true except It is the reaction of the donor lymphocytes against the host.Acute GVHD starts within the first 100 days and chronic is after 100 days.Affects the skin, liver and GI tractIrradiation of blood products does not helpComplete HLA matching prevents GVHD6