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KG - Pediatrics Exam 2 > Heme-Onc > Flashcards

Heme-Onc Flashcards

1
Q

Which malignancy is associated w/ Bence-Jones protein in 75% of patients, an M-spike, and bone pain?

A

multiple myeloma

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2
Q

which malignancy is associated w/ enlarged nodes 90% of time and unique presence of Reed-Sternberg cells?

A

Hodgkins-Lymphoma

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3
Q

Which malignancy is associated w/ B-symptoms and follicular and diffuse large B cell subtypes?

A

non-hodkins lymphoma

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4
Q

what are “B-symptoms”

A

constitutional symptoms = chills, night sweats, fever, weight loss

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5
Q

which malignancy has a 13q deletion, 90% of patients are over age 50 yrs old, and a hallmark is the clonal expansion of cells expressing CD5+ B cells?

A

Chronic lymphocytic leukemic

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6
Q

which malignancy has patients w/ Philadelphia chromosome in 85-95% of the time?

A

chronic myelogenous leukemia

remember Roch is from Philly & “cheesesteaks me likes!

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7
Q

which malignancy features more than 30% peripheral blasts (5% is normal) that stain myeloperoxidase + and the median age of onset is 68 years of age?

A

acute myelogenous leukemia

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8
Q

two most common findings of anemia

A

pallor & jaundice

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9
Q

petechiae & purpura indice abnormalities of _____.

A

platelets, coagulation factors, consumptive coagulopathy

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10
Q

first test to determine anemia?

A

hemoglobin level

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11
Q

second test to look at for anemia?

A

MCV (mean corpuscular volume)

hypochromic, microcytic –> iron deficiency, thalessemai
normochromic, normocytic –> anemia of chronic disease
macrocytic –> vit b12, folate deficiency

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12
Q

signs/symptoms of anemia

A

increased pulse rate
HA
excessive sleepiness
poor feeding

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13
Q

congenital aplastic anemia

info & presentation

A

aka FANCONI ANEMIA - congenital disease manifested by defective DNA repair

  • begins w/ thrombocytopenia or neutropenia, progresses to pancytopenia
  • may see short stature, cafe au lait spots, mental retardation
  • most cases acquired, not inherited

DDX = idiopathic thrombocytopenic purpura

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14
Q

congenital aplastic anemia

treatment

A

bone marrow transplant

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15
Q

acquired aplastic anemia

info & presentation

A
  • peripheral pancytopenia w/ hypo cellular bone marrow
  • 50% idiopathic, some related to drugs, some related to viral infections (Hep most common)
  • certain viruses will affect ONE blood line
  • low reticulocyte count (low bc problem in bone marrow)
  • normal response to decreased blood cells = increased reticulocytes
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16
Q

when will acquired aplastic anemia have complications?

A
  • with OVERWHELMING INFECTION (due to low WBCs)

- with SEVERE HEMORRHAGE (due to low platelets)

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17
Q

acquired aplastic anemia

treatment

A
  • stop offending agent
  • antibiotics for infection
  • blood transfusions for severe anemia
  • may consider bone marrow transplant (w/ pancytopenia)
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18
Q

iron deficiency anemia

info & presentation

A
  • MOST COMMON cause of anemia in ids
  • usu poor dietary intake of iron ages 6-24 months
  • may see glossitis, angular stomatitis, fatigue
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19
Q

iron deficiency anemia

diagnostic

A
  • labs = microcytic, hypochromic anemia, low serum iron, elevated TIBC (TIBC = total iron binding capacity)
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20
Q

what should be considered along w/ iron def anemia ir the patient is African, Mediterranean, or Asian?

what test should be done?

A

Thalassemia - do a hemoglobin electrophoresis

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21
Q

megaloblastic anemia

info & presentation

A
  • caused by deficiency of fit B12, folic acid (or both)

- see elevated MCV, MCH

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22
Q

megaloblastic anemia

treatment

A

replacement therapy or either B12, folic acid, or both

  • always replace B12 bc it can lead to peripheral neuropathy
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23
Q 
hereditary spherocytosis (anemia)
(info & presentation)
A
  • inherited hemolytic anemia
  • may see jaundice and splenomegaly
  • will see unusual cells on peripheral smear and elevated reticulocyte count
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24
Q 
heriditary spherocytosis (anemia)
(treatment)
A

may need splenectomy

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25
Q

thalassemia

info & presentation

A
  • alpha or beta

- beta can be minor or major

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26
Q

If someone has thalassemia, what will labs show?

A
  • microcytic anemia, but out of proportion to degree of anemia
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27
Q

thalassemia, beta major

treatment

A

may be bone marrow transplant

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28
Q

G6PD

A
  • infants show hyperbilirubinemia

- symptoms appear under oxidative stress

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29
Q

What is the most common red cell enzyme defect? (anemia)

A

G6PD - causes hemolytic anemia

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30
Q

What is seen in G6PD labs?

A

Heinz bodies

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31
Q

Sickle Cell anemia

info & presentation

A
  • highest incidence = central African origin
  • may see jaundice, PAIN overwhelming
  • high risk for overwhelming bacterial sepsis
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32
Q

sickle cell anemia

labs

A
  • elevated reticulocyte count

- abnormal types of Hb on Hb electrophoresis lab test

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33
Q

sickle cell anemia

treatment

A
  • education
  • management of acute problems (can be pain, may need oxygen)
  • may need blood transfusion
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34
Q

What is the most common red cell enzyme defect? (anemia)

A

G6PD - causes hemolytic anemia

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35
Q 
lead poisoning
(characteristics)
A
  • history of pica

- common in older homes w/ lead paint

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36
Q

lead poisoning lab results

A

on peripheral blood smear will see BASOPHILIC STIPPLING

37
Q

lead poisoning treatment

A

chelation therapy

38
Q

what is polycythemia?

A

increased red blood cells

39
Q

if bleeding occurs w/ idiopathic thrombocytopenic purpura, what should be used?

A

Prednisone

40
Q

primary polycythemia

treatment

A

phlebotomy

41
Q

secondary polycythemia

info & presentation

A
  • most common cause is cyanotic congenital heart disease
42
Q

secondary polycythemia

treatment

A

correction of underlying disorder (fix defect, phlebotomy)

43
Q

what are the two types of cyanotic congenital heart disease?

A

tetralogy of Fallot, transposition of the great vessels

44
Q

idiopathic thrombocytopenic purpura

info & presentation

A
  • most common bleeding disorder in kids ages 2-5
  • immune process of antibodies to own platelets
  • OFTEN follows viral infection
  • 90% kids have spontaneous remission
45
Q

what labs should be ordered if a bleeding disorder is suspected?

A
  • platelet count
  • bleeding time
  • PT (monitors warfarin)
  • aPTT (monitors heparin)
45
Q

name 2 inherited bleeding disorders and the deficiency for each

A

1) hemophilia A - factor VIII deficiency

2) hemophilia B - factor IX deficiency

46
Q

how should vitamin K deficiency be treated?

A

replacement of vit K

47
Q

what triggers Disseminated Intravascular Coagulation?

A

something such as an injury to tissue or vessels, hypoxia

48
Q

is liver disease an inherited or acquired bleeding disorder?

A

acquired

49
Q

vasculitis - Henoch Schonlein Purpura

treatment

A

no treatment necessary

50
Q

is disseminated intravascular coagulation an inherited or acquired bleeding disorder?

A

acquired

51
Q

name 4 thrombotic disorders

A
  • protein c deficiency
  • protein s deficiency
  • antithrombin III deficiency
  • factor V leiden mutation
52
Q

what are common info & presentation of thrombotic disorders?

A
  • may see DVTs of lower extremities
  • may see pulmonary embolus
  • positive family history of clots
53
Q

vasculitis - Henoch Schonlein Purpura

info & presentation

A
  • most common small vessel vasculitis in kids ages 2-7 yrs

- 66% have preceding URI

54
Q

disseminated intravascular coagulation

info & presentation

A
  • widespread activation of coagulation mechanism
  • may see bleeding as well as clotting
  • usu associated w/ sepsis or malignancy
55
Q

disseminated intravascular coagulation

labs

A
  • show thrombocytopenia

- elevated PT, aPTT, FDP (fibrin degradation products), D-Dimer)

56
Q

disseminated intravascular coagulation

treatment

A

multifactorial, depends on person - chemo/antibiotics

57
Q

which neoplastic disease could become a medical emergency?

A

acute myeloid leukemia (AML)

58
Q

acute lymphoblastic leukemia (ALL)

diagnostic

A
  • WBC > 200,000
  • elevated LDH
  • bone marrow examination (shows leukemic blasts replacing marrow)
  • 95% patients show decrease in at least one cell line, commonly two cell lines
59
Q

acute lymphoblastic leukemia (ALL)

treatment & side effects

A
  • chemo

- should anticipate tumor lysis syndrome –> hyperkalemia, hyperuricemia, hypocalcemia, renal failure

60
Q

brain tumors

info & presentation

A
  • most common solid tumor of childhood
  • may see vomiting, lethargy, irritability
  • older kids have HA, visual changes
61
Q

how to diagnose brain tumor/what to do if tumor suspected?

A
  • measure head circumference & observe gait for eval of young children
  • do MRI for best definition of tumor
62
Q

acute myeloid leukemia (AML)

treatment

A

chemo

63
Q

which neoplastic disease could become a medical emergency?

A

acute myeloid leukemia (AML)

64
Q

chronic myelogenous leukemia (CML)

info & presentation

A
  • only 5% leukemias in children

- look for Philadelphia chromosome

65
Q

chronic myelogenous leukemia (CML)

treatment

A
  • interferon alpha

- bone marrow transplant (more common w/ leukemia)

66
Q

non-hodgkin lymphoma

treatment

A

chemo

67
Q

hodgkin disease

info & presentation

A
  • neoplastic disease
  • will show B-cell symptoms
  • anorexia, weight loss, fever, fatigue, night sweats
  • painless cervical or supraclavicular adenopathy, mediastinal mass
68
Q

hodgkin disease

diagnostic

A
  • stage w/ CXR, CTs, bone marrow biopsy

- requires presence of REED-STERNBERG cells (owl’s eyes)

69
Q

hodgkin disease

treatment

A
  • chemotherapy

kids respond better to treatment than adults

70
Q

non-hodgkin lymphoma

info & presentation

A
  • neoplastic disease
  • cough, dyspnea, orthopnea, lymphadenopathy, abdominal pain, vomiting, fever
  • in children… N-HL RAPIDLY PROLIFERATING, HIGH GRADE, DIFFUSE MALIGNANCIES
71
Q

non-hodgkin lymphoma

diagnostic

A

biopsy

72
Q

neuroblastoma

info & presentation

A
  • in sympathetic nervous system
  • 90% diagnosed before age 5
  • fever, weight loss, irritability, ABDOMINAL MASS, bone pain
73
Q

neuroblastoma

diagnostic

A

need to do skeletal survey and bone scan to stage

74
Q

neuroblastoma

treatment

A
  • surgery
  • radiation
  • chemo
75
Q

nephroblastoma (Wilms Tumor)

info & presentation

A
  • must look for asymptomatic abdominal mass

- fever, hematuria, hypertension

76
Q

Ewing Sarcoma

treatment

A
  • chemo

- maybe surgery

77
Q

osteosarcoma

info & presentation

A
  • peak occurrence during adolescent growth spurt

- occurs in LONG BONE

78
Q

osteosarcoma

diagnostic

A
  • bone scan
79
Q

osteosarcoma

treatment

A
  • surgery (70% getting surgery alone develop pulmonary metastatic disease w/in 6 months of surgery)
  • chemo
80
Q

Ewing sarcoma

info & presentation

A
  • long bones or soft tissue
  • NIGHT TIME bone pain
  • white males
81
Q

Ewing sarcoma

diagnostic

A

CT or MRI to define lesion

82
Q

retinoblastoma

info & presentation & treatment

A
  • 90% diagnosed before age 5
  • WHITE PUPILLARY REFLEX most common sign
  • needs ophthamologic referral
83
Q

rhabdomyosarcoma

info & presentation

A
  • most common soft tissue sarcoma in childhood
  • 70% diagnosed before age 10 yrs
  • signs/symptoms depends on where tumor located - usu head/neck region
84
Q

rhabdomyosarcoma

treatment

A
  • surgery
  • chemo
  • radiation
    (or any combo of this)
85
Q

hepatic tumors

info & presentation

A
  • 57% found in childhood = malignant

- usu present w/ enlarging abdomen

86
Q

hepatic tumor

diagnostic

A
  • check AFP (serum alpha fetal protein)
87
Q

hepatic tumor

treatment

A
  • surgery

- chemo

KG - Pediatrics Exam 2 (3 decks)

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