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Peds Boards > Heme/Onc > Flashcards

Heme/Onc Flashcards

0
Q
  • sarcoma in long bones (femur, humerus)
  • more common in african americans
  • teenager, usually going through a growth spurt that presents with pain
  • main site of mets are lungs
  • Tx: chemo, XRAD, surgery
A

Osteogenic sarcoma

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1
Q
  • sarcoma in the soft tissue component and long bones
  • usually not seen in african americans
  • Tx: chemo, XRAD, surgery
  • can metastasize to lungs
A

Ewing Sarcoma

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2
Q
  • tibia or femur pain worse at night, relieved by Ibuprofen

- Xray: central radiolucent (white) area surrounded by thick sclerotic bone

A

Osteoid osteoma

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3
Q

-most common type of solid tumors

A

Brain tumors

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4
Q
  • teenager with non-tender enlarged cervical LN or supraclavicular LN
  • wt loss, fevers, night sweats
  • Bx: Reed Sternberg cells
  • CBC: incr WBC, decr lymphs
A

Hodgkins Lymphoma

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5
Q
  • MC pediatric emergency
  • poor prognostic signs: 50, T cell
  • good prognostic sign: B cell
  • present with bone pain, joint pain, or swelling, fever, HSM
  • pancytopenia, nL or incr WBC
  • Dx: BM Bx
A

Acute Lymphocytic Leukemia

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6
Q
  • younger child with non-tender abd mass or head & neck region
  • lymphoblastic (T cell region)
  • non-lymphoblastic (B cell region)
  • mediastinal masses & pleural effusions associated w/ lyphoblastic type
A

Non-Hodgkin Lymphoma

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7
Q
  • solitary focal bone lesion often in skull
  • seborrheic rash, ear DC, DI, excessive urination
  • Dx: skin Bx & electron microscopy
  • Tx: surgery, steroids, chemo
A

Langerhans Cell Histiocytosis

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8
Q
  • 2nd MC solid tumor in childhood
  • presents in the adrenal glands 50%
  • 95% survival catecholamines
  • Dx: biopsy, urine VMA, HAM, CT w/ contrast, pancytopenia, incr ferritin, incr LDH
  • Bad prognosis: + n-myc amplification
A

Neuroblastoma

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9
Q

-leucocoria
-5% will have a +FHx
-Tx: surgery, chemo, radiation
-Risk for osteosarcoma later in life
-parent 2 eyes: 50% chance to kid
parent with 1 eye: 5% chance to kid

A

Retinoblastoma

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10
Q
  • adolescent girl, neg HCG, and persistent abd pain
  • benign: filled with fluid on US
  • malignant: defined with echodensities
A

Ovarian masses

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11
Q
  • MC soft tissue sarcoma in childhood
  • may present as a child with constipation
  • visible or palpable rectal mass (grape-like mass protruding from the vagina)
  • younger: head and neck
  • older truncal or extremity (may be h/o trauma)
  • Dx: Bx
  • tx: chemo, surgery, XRAD
A

Rhabdomyosarcoma

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12
Q
  • -MC pediatric abdominal malignancy
  • median age 3.5 years
  • MC presentation is an asymptomatic abd mass with occasional hypertension and gross hematuria
  • Dx: histology
  • Tx: nephrectomy, chemo, XRAD
  • associated with aniridia and hemihypertrophy
A

Wilms Tumor (Nephroblastoma)

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13
Q
  • rapid breakdown of a large number of tumor cells
  • MC at the initiation of chemotherapy for large tumors or leukemia (Burkitts)
  • high Phos, K, uric acid, low Ca
  • Tx: hydration, alkalinization, allopurinol
A

Tumor Lysis Syndrome

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14
Q
  • tumor in or around the spine
  • presents with neurologic symptoms or bowel.bladder dysfunction
  • Tx: steroids or radiation
A

Cord Compression

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15
Q
  • respiratory distress when supine
  • intubation is useless if mass is below vocal cords
  • Thymoma, Teratoma, Thyroid, T-cell lymphoma*
  • Tx: radiation in an emergency
A

Anterior mediastinal mass

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16
Q
  • extrinsic compression of the SVC by an anterior mediastinal tumor
  • MC Hodgkins or T-cell lymphoma
  • presents with red face (plethora), facial swelling, upper extremity edema, distended neck veins, and neurologic symptoms
  • Tx: underlying disorder, steroids or radiation
A

SVC syndrome

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17
Q

-causes hemorrhagic cystitis

A

Cyclophosphamide

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18
Q

-causes pulmonary fibrosis

A

Bleomycin (Blow my icing)

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19
Q

-causes cardiomyopathy

A

Anthracycline (Doxorubicin, Daunomycin)

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20
Q

-causes neurotoxicity and SIADH

A

Vincristine/Vinblastine

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21
Q

-causes pancreatitis

A

Aspariginase

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22
Q

-causes CNS problems

A

Procarbazine

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23
Q

-causes oral and GI ulcers

A

Methotrexate

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24
Q
  • physiological drop in HCT by 2nd or 3rd month of life (8-10 weeks)
  • no further evaluation is necessary
  • etiology: low erythropoietin production
A

Anemia of the Newborn

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25
Q
  • defect in one of the beta globin gene alleles
  • asymptomatic
  • mild microcytic anemia
A

Thalassemia Minor or Trait

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26
Q
  • defects in both beta globin gene alleles
  • -mild phenotype
  • requires infrequent transfusions (after a viral infx)
A

Thalassemia Intermedia

27
Q
  • severe deficiency of beta globin due to a mutation in both beta globin gene alleles
  • present in the first year of life with profound microcytic anemia
  • require regular blood transfusions
  • small for age Greek child with HSM
  • skull Xray with hair on end appearance (extramedullary hematopoiesis)
  • complications: cholelithiasis, hemosiderosis
A

Thalassemia major (Cooley’s anemia)

28
Q
  • Dx: Hb electrophoresis
  • very low HB A1, incr A2 (alpha–delta)
  • incr Hb F (alpha-gamma)
A

Beta thalassemia

29
Q
  • microcytic anemia

- deletion of one or more of the alpha globin gene alleles

A

Alpha Thalassemia

30
Q

-high Hb F and Hb S

A

Sickle Cell Anemia

31
Q
  • higher risk in breast fed infants, infants given cows milk
  • present with pallor and tachycardia
  • low Hb and MCV
  • high RDW
A

Iron Deficiency Anemia

32
Q
  • may be normocytic or microcytic
  • low TIBC
  • high ferritin
  • Tx with Fe is not necessary
A

Anemia of chronic disease

33
Q
  • pale kid with vague symptoms (irritability, lethargy, sleplessness, headaches, belly pain, constipation)
  • usually coexists with iron deficiency
  • Dx: venous blood level
  • Tx; chelation (penicillamine, dimercaprol, EDTA)
  • elevated free erythrocyte protoporphyrin
  • ringed sideroblast
A

Lead Poisoning

34
Q
  • instrinsic factor deficiency
  • bacterial overgrowth
  • s/p bowel resection
  • infants whose mothers are vegetarians
  • macrocytic anemia
  • Schilling test for absorption
  • AKA cobalamin
A

Vitamin B12 Deficiency

35
Q
  • infant fed goats milk

- macrocytic anemia

A

Folate deficiency

36
Q
  • lack of intrinsic factor
  • weakness, paresthesia, sore tongue
  • Dx: erythrocyte folic acid concentration
  • Tx: IM cobalamin
A

Pernicious anemia

37
Q
  • black or mediterranean boy with dark urine, jaundiced, and anemic
  • exposure to mothballs, antimalarials, nitrofurantoin, bactrim, cipro or fava beans triggers hemolytic crisis
  • sudden onset of pallor and anemia
  • Heinz bodies: small purple granules in the red cell that form as a result of damage to the Hb molecule
  • X linked
A

G6PD deficiency

38
Q

-G6PD mainly found in African Americans

A

Type A

39
Q

-G6PD mainly found in Mediterraneans

A

Type B

40
Q
  • defects in the surface of red cells resulting in loss of erythrocyte surface area
  • MC autosomal dominant
  • increased MCHC, low retic ct
  • Dx: osmotic fragility testing
  • etiology: spectrin deficiency or mutation
  • presents with mod anemia, splenomegaly, and intermittent jaundice
  • Tx: folic acid or splenectomy
  • parvovirus causes aplastic crisis
A

Hereditary Spherocytosis

41
Q
  • -anemia, jaundice, and splenomegaly
  • cells depleted of lactate and ATP–> buildup of DPG–>right shift of the oxygen binding curve
  • increase in exercise tolerance
  • Northern Europeans
  • Autosomal recessive
A

Pyruvate Kinase Deficiency

42
Q
  • caused by a substitution at AA6 of the beta globin chain (valine for glutamic acid)
  • newborn screening
  • vasoocclusive crisis
  • splenic sequestration
  • aplastic crisis
  • need PCN PPx
  • Howell-Jolly Bodies
A

Sickle Cell Anemia

43
Q
  • chest pain, infiltrate, and hypoxia in a pt with sickle cell
  • Dx: CXR, ABG
  • Tx: transfusion
A

Acute chest syndrome

44
Q

-when sickle cell children have trouble concentrating their urine

A

hyposthenuria

45
Q

-MC cause of RUQ pain in a child with sickle cell

A

Gallstones

46
Q
  • pallor, mucosal ulcerations, and bruising
  • anemia, low WBC, low plts
  • increase in fetal Hb and macrocytosis
  • normal liver and no LAD
  • h/o chloramphenicol use
  • Tx: bone marrow Tx
A

Aplastic anemia

47
Q
  • presents after age 3
  • skin pigmentation, growth retardation, renal abnl, absent or hypoplastic thumb
  • macrocytic anemia with elevated fetal Hb
  • autosomal recessive
  • at risk of AML or myelodysplastic syndrome
A

Fanconi Anemia

48
Q
  • arrest in the mutation of red cells
  • anemia at birth, macrocytic
  • dysmorphology (thumb abnl, UG abnl, craniofacial probs)
  • need transfusions
  • steroids sometimes helpful
A

Diamond Blackfan Anemia

49
Q
  • suppression of erythroid production
  • toddlers
  • spontaneous recovery
  • normal MC
A

Transient Erythroblastopenia of Childhood

50
Q
  • WBC contain lysosomal granules
  • abnl chemotaxis
  • frequent lung and skin infx, easy bruisability, oculocutaneous albinism
  • S. aureus, S. pyogenes, pneumococcus
  • autosomal recessive
  • Tx: BMT
A

Chediak-Higashi

51
Q
  • phagocytes cannot kill bugs they ingest
  • recurrent bacterial and fungal infx in skin, lungs, GI, liver, spleen
  • staph or gram negative infx
  • Dx: NTB test measures the oxidative burst
  • infx PPx, interferon, and BMT
A

Chronic Granulomatous Disease

52
Q
  • WBCs do not adhere well
  • delayed separation of the umbilical cord, impaired wound healing, severe periodontal disease
  • high WBC
  • Tx: BMT
A

Leukocyte Adhesion Deficiency

53
Q
  • autosomal dominant
  • low WBC x1 wk, reappears every month or so
  • oral lesions in a child less than 10 yo
  • Dx: CBC twice a week
  • infx with Clostridium perfringens
  • Tx: G-CSF if symptomatic
A

Cyclic Neutropenia

54
Q
  • autosomal recessive
  • arrest in the development of neutrophils
  • low ANC
  • Tx: G-CSF
A

Kostmann Syndrome

55
Q
  • pancytopenia, EPI
  • short statures, diarrhea, recurrent infx, skeletal abnl, clinodactyly/syndactyly
  • monitor for leukemic transformation
A

Shwachman-Diamond Syndrome

56
Q
  • recent viral illness, low plts, ecchymoses, petichiae
  • big plts
  • Tx: IVIG, WinRho
  • transfuse if plts <20K and bleeding
A

Immune Thrombocytopenic Purpura

57
Q
  • immunodeficiency only seen in boys (X-linked)-baby boy with really bad diaper rash, bruising, ear infx, and pneumonia
  • low plts, low MPV, or small plts
  • Tx: BMT
  • at risk for other malignancies
A

Wiskott-Aldrich Syndrome

58
Q
  • hemangioma
  • thrombocytopenia caused by consumptive coagulopahty
  • at risk for DIC
  • Tx: control hemangioma and transfuse
A

Kasabach-Merritt Syndrome

59
Q
  • thrombocytopenia, absent radius
  • high WBC, nl Hb
  • symptomatic in the first week of life
A

TAR Syndrome

60
Q
  • plts cannot aggregate

- nl plts ct and coags

A

Glanzmann Thrombasthenia

61
Q

-baby born at home who is breast fed and bleeding

A

Vit K Deficiency

62
Q
  • bruising, bleeding from circ, or site of venipuncture, deep joint bleeds
  • prolonged pTT
  • boys with a FHx in men on mom’s side
  • X-linked recessive
A

Hemophilia

63
Q
  • excessive bleeding after a dental procedure or T&A
  • epistaxis
  • menorrhagia in a female
  • abnl pTT, normal PT
  • prolonged bleeding time
  • Tx: DDAVP, amicar, factor VIII
  • autosomal dominant
A

Von Willebrand Disease

64
Q
  • h/o sepsis, malignancy, or burns
  • low plts, fibrinogen, coags
  • elevated D-dimer, thrombin
  • Tx: plts, FFP, cryo, RBCs
A

DIC

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