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
thalassemia | info & presentation
- alpha or beta | - beta can be minor or major
26
If someone has thalassemia, what will labs show?
- microcytic anemia, but out of proportion to degree of anemia
27
thalassemia, beta major | treatment
may be bone marrow transplant
28
G6PD
- infants show hyperbilirubinemia | - symptoms appear under oxidative stress
29
What is the most common red cell enzyme defect? (anemia)
G6PD - causes hemolytic anemia
30
What is seen in G6PD labs?
Heinz bodies
31
Sickle Cell anemia | info & presentation
- highest incidence = central African origin - may see jaundice, PAIN overwhelming - high risk for overwhelming bacterial sepsis
32
sickle cell anemia | labs
- elevated reticulocyte count | - abnormal types of Hb on Hb electrophoresis lab test
33
sickle cell anemia | treatment
- education - management of acute problems (can be pain, may need oxygen) - may need blood transfusion
34
What is the most common red cell enzyme defect? (anemia)
G6PD - causes hemolytic anemia
35
``` lead poisoning (characteristics) ```
- history of pica | - common in older homes w/ lead paint
36
lead poisoning lab results
on peripheral blood smear will see BASOPHILIC STIPPLING
37
lead poisoning treatment
chelation therapy
38
what is polycythemia?
increased red blood cells
39
if bleeding occurs w/ idiopathic thrombocytopenic purpura, what should be used?
Prednisone
40
primary polycythemia | treatment
phlebotomy
41
secondary polycythemia | info & presentation
- most common cause is cyanotic congenital heart disease
42
secondary polycythemia | treatment
correction of underlying disorder (fix defect, phlebotomy)
43
what are the two types of cyanotic congenital heart disease?
tetralogy of Fallot, transposition of the great vessels
44
idiopathic thrombocytopenic purpura | info & presentation
- 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
what labs should be ordered if a bleeding disorder is suspected?
- platelet count - bleeding time - PT (monitors warfarin) - aPTT (monitors heparin)
45
name 2 inherited bleeding disorders and the deficiency for each
1) hemophilia A - factor VIII deficiency | 2) hemophilia B - factor IX deficiency
46
how should vitamin K deficiency be treated?
replacement of vit K
47
what triggers Disseminated Intravascular Coagulation?
something such as an injury to tissue or vessels, hypoxia
48
is liver disease an inherited or acquired bleeding disorder?
acquired
49
vasculitis - Henoch Schonlein Purpura | treatment
no treatment necessary
50
is disseminated intravascular coagulation an inherited or acquired bleeding disorder?
acquired
51
name 4 thrombotic disorders
- protein c deficiency - protein s deficiency - antithrombin III deficiency - factor V leiden mutation
52
what are common info & presentation of thrombotic disorders?
- may see DVTs of lower extremities - may see pulmonary embolus - positive family history of clots
53
vasculitis - Henoch Schonlein Purpura | info & presentation
- most common small vessel vasculitis in kids ages 2-7 yrs | - 66% have preceding URI
54
disseminated intravascular coagulation | info & presentation
- widespread activation of coagulation mechanism - may see bleeding as well as clotting - usu associated w/ sepsis or malignancy
55
disseminated intravascular coagulation | labs
- show thrombocytopenia | - elevated PT, aPTT, FDP (fibrin degradation products), D-Dimer)
56
disseminated intravascular coagulation | treatment
multifactorial, depends on person - chemo/antibiotics
57
which neoplastic disease could become a medical emergency?
acute myeloid leukemia (AML)
58
acute lymphoblastic leukemia (ALL) | diagnostic
- 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
acute lymphoblastic leukemia (ALL) | treatment & side effects
- chemo | - should anticipate tumor lysis syndrome --> hyperkalemia, hyperuricemia, hypocalcemia, renal failure
60
brain tumors | info & presentation
- most common solid tumor of childhood - may see vomiting, lethargy, irritability - older kids have HA, visual changes
61
how to diagnose brain tumor/what to do if tumor suspected?
- measure head circumference & observe gait for eval of young children - do MRI for best definition of tumor
62
acute myeloid leukemia (AML) | treatment
chemo
63
which neoplastic disease could become a medical emergency?
acute myeloid leukemia (AML)
64
chronic myelogenous leukemia (CML) | info & presentation
- only 5% leukemias in children | - look for Philadelphia chromosome
65
chronic myelogenous leukemia (CML) | treatment
- interferon alpha | - bone marrow transplant (more common w/ leukemia)
66
non-hodgkin lymphoma | treatment
chemo
67
hodgkin disease | info & presentation
- neoplastic disease - will show B-cell symptoms - anorexia, weight loss, fever, fatigue, night sweats - painless cervical or supraclavicular adenopathy, mediastinal mass
68
hodgkin disease | diagnostic
- stage w/ CXR, CTs, bone marrow biopsy | - requires presence of REED-STERNBERG cells (owl's eyes)
69
hodgkin disease | treatment
- chemotherapy | kids respond better to treatment than adults
70
non-hodgkin lymphoma | info & presentation
- neoplastic disease - cough, dyspnea, orthopnea, lymphadenopathy, abdominal pain, vomiting, fever - in children... N-HL RAPIDLY PROLIFERATING, HIGH GRADE, DIFFUSE MALIGNANCIES
71
non-hodgkin lymphoma | diagnostic
biopsy
72
neuroblastoma | info & presentation
- in sympathetic nervous system - 90% diagnosed before age 5 - fever, weight loss, irritability, ABDOMINAL MASS, bone pain
73
neuroblastoma | diagnostic
need to do skeletal survey and bone scan to stage
74
neuroblastoma | treatment
- surgery - radiation - chemo
75
nephroblastoma (Wilms Tumor) | info & presentation
- must look for asymptomatic abdominal mass | - fever, hematuria, hypertension
76
Ewing Sarcoma | treatment
- chemo | - maybe surgery
77
osteosarcoma | info & presentation
- peak occurrence during adolescent growth spurt | - occurs in LONG BONE
78
osteosarcoma | diagnostic
- bone scan
79
osteosarcoma | treatment
- surgery (70% getting surgery alone develop pulmonary metastatic disease w/in 6 months of surgery) - chemo
80
Ewing sarcoma | info & presentation
- long bones or soft tissue - NIGHT TIME bone pain - white males
81
Ewing sarcoma | diagnostic
CT or MRI to define lesion
82
retinoblastoma | info & presentation & treatment
- 90% diagnosed before age 5 - WHITE PUPILLARY REFLEX most common sign - needs ophthamologic referral
83
rhabdomyosarcoma | info & presentation
- 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
rhabdomyosarcoma | treatment
- surgery - chemo - radiation (or any combo of this)
85
hepatic tumors | info & presentation
- 57% found in childhood = malignant | - usu present w/ enlarging abdomen
86
hepatic tumor | diagnostic
- check AFP (serum alpha fetal protein)
87
hepatic tumor | treatment
- surgery | - chemo