Myeloma/peds

Front 1

what is the most common type of childhood cancer

Back 1

ALL (more common in whites and males)

Front 2

presentation of ALL in a kid

Back 2

diffuse sx, like viral illness:
fever, purpura, fatigue, organomegaly, adenopathy, HA, CN palsy, aches and pains, skin rash, bleeding, prolonged "viral illness

Front 3

cytogenetics of childhood ALL that are associated with a good prognosis

Back 3

hyperdiploid, trisomy 4, 10, TEL-AML (t(12;21))

Front 4

cytogenetics of childhood ALL that are associated with a poor prognosis

Back 4

hypodiploid, t(9;22), t(4;11)

Front 5

risk factors for childhood ALL

Back 5

>50k WBC, <1y or >10y, T cell, rapidity of BM response, min residual ds, cytogenetics

Front 6

what syndrome is a major risk factor for AML in children

Back 6

down syndrome (although these pts tend to do better)

Front 7

bad cytogenetics in AML

Back 7

monosomy 5, 7, and FLT3 internal tandem duplication

Front 8

good cytogenetics in AML

Back 8

t(8;21), t(15;17)-APL, inv(16)

Front 9

is secondary AML assoc with a better or worse prognosis

Back 9

worse

Front 10

leukemia emergencies

Back 10

1. bleeding- thrombocytopenia or DIC
2. tumor lysis
3. leukostasis- WBC>100k
4. fever/infection/neutropenia!!! (esp ANC<500)

Front 11

what is wilms tumor

Back 11

most common kidney tumor in kids, highly curable

Front 12

how does wilms tumor present

Back 12

ab mass, hematuria, hemihypertrophy, aniridia (absense of the iris)

Front 13

neuroblastoma presentation

Back 13

1. ab mass and pain
2. bone pain
3. diarrhea
4. black eyes
5. congenital presentation

Front 14

rhabdomyosarcoma presentation

Back 14

mass at any location, proptosis, vaginal mass, pain at site

Front 15

what are two bone tumors in kids

Back 15

ewings and osteogenic

Front 16

where are osteogenic sarcomas located

Back 16

ends of long bones (adolescents)

Front 17

where are ewings sarcomas located

Back 17

mid shaft long bones, flat bones (younger kids)

Front 18

presentation of brain tumors

Back 18

HA, n/v- esp in the am, papilledema, ataxia, unilateral weakness, head tilt, strabismus

Front 19

what are the late effects of radiation

Back 19

effect growth/develp, cognitive, endocrine

Front 20

what are the late effects of ITs

Back 20

growth/develp, cognitive

Front 21

what are the late effects of alkylators

Back 21

infertility, secondary malignancies

Front 22

which chemo drugs have hepatotoxicity late effects

Back 22

6MP, 6TG, MTX

Front 23

what late effect does VP16 (etopiside) cause

Back 23

secondary malignancies

Front 24

what has late effects on bone

Back 24

steroids

Front 25

what is the most impt risk factor for developing myeloma

Back 25

time. its a ds of those >50 yo. Note: takes mult factors to generate myeloma

Front 26

what are some established risk factors for myeloma

Back 26

age>65, male, black

Front 27

when does the first transformation event occur in myeloma

Back 27

shortly after class switching when they go to the marrow and produce Ig (they have same proportion of Ig classes as someone w/o the ds). the cells are nl in GC

Front 28

what should you do when you suspect myeloma

Back 28

order a PEP to see if Ig increased

Front 29

what should you do if your pt has an abnl PEP

Back 29

do an IEF to see if monoclonal (use Abs to look for kappa and lambda)

Front 30

how do myeloma cells effect the bone

Back 30

turn on osteoclasts and turn off osteoblasts (lesions are lytic and not seen on bone scan)

Front 31

what is key to the proliferation and survival of myeloma cells

Back 31

IL-6

Front 32

what are the manifestations/sx of myeloma

Back 32

1. anemia
2. kidney injury
3. increased risk for infections
4. hyperviscosity syndrome
5. bone disease

Front 33

describe the RBCs in myeloma associated anemia (chromic, cytic)

Back 33

normochromic, normocytic

Front 34

what are the causes of myeloma associated anemia

Back 34

1. myeloma cells and stroma interact and inhibit erythropoesis (hypoprolif anemia)
2. myeloma cells produce FasL which kills RBC precursors
3. renal problems decrease epo levels
4. overgrowth in marrow doesn't allow RBCs to grow (in adv ds)

Front 35

how does myeloma cause kidney injury

Back 35

1. lambda M proteins deposit in dist tubules and collecting ducts
2. kappa M proteins deposit in glomerlus
3. myeloma can cause hypercalcemia which can lead to acute kidney injury

Front 36

myeloma labs

Back 36

increased protein to albumin ration
decreased anion gap
rouleaux formation
increased sedimentation rate

Front 37

causes of increased infection risk in myeloma pts

Back 37

1. hypogammaglobulinemia
2. dysregulation of cell immunity (T cell response poor)

Front 38

how does myeloma cause hyperviscosity syndrome

Back 38

1. forms large If polymers (usually IgM myeloma)
2. forms Ig/RBC aggregates (seen in IgG myeloma)

Front 39

what evidence do you need to dx myeloma

Back 39

1. monoclonal expansion of plasma cells
2. sx due to plasma cells

Front 40

what are the labs and values that demonstrate monoclonal expansion of plasma cells

Back 40

M protein >= 3g/dL OR >=10% clonal plasma cells in BM

Front 41

what are the myeloma symptoms due to plasma cells

Back 41

1. hypercalcemia
2. renal insufficiency
3. anemia
4. myeloma bone ds (lytic bone lesions)
5. >2 bacterial infections/yr

Front 42

what things do you measure to quantify myeloma

Back 42

1. M protein (IEF)
2. serum levels of Ig (G, M, or A)
3. free serum light chains
4. BM biopsy- measure % of plasma cells (unreliable b/c pathcy but gold std for determining complete response)

Front 43

what measurements are used for staging myeloma

Back 43

B2 microglobuline and albumin (higher B2 and lower albumin are worse)

Front 44

cytogenetics principles in myeloma

Back 44

1. complex karyotypes are worse (except hyperdiploidy)
2. deletions of 13q and 17p are worse and t(4;14) poor
3. karyotypes change with time

Front 45

is myeloma curable

Back 45

no (therefore treatment shouldn't be worse than the ds)

Front 46

what does myeloma treatment involve (generally speaking)

Back 46

mult drugs in mult combos

Front 47

what is MGUS

Back 47

monoclonal gammopathy of unknown significance. high M protein or monoclonal PCs with no sx (its an abnl lab finding). 10-25% pts will progress to myeloma

Front 48

what is amyloid ds

Back 48

beta pleated sheets of fibrils in extracellular space. can be formed from different proteins

Front 49

list the different proteins that can form the beta pleated sheets of fibrils in amyloid ds

Back 49

1. abnl folded proteins from inherited defect
2. serum amyloid A (produced during inflammation and infection)
3. B2 microglobulin- used to build up in dialysis pts
4. Ig light chains- most common type of systemic amyloid and assoc with monoclonal PCs

Front 50

amyloid sx

Back 50

related to where amyloid is deposited: renal, nervous, cardiac, fat and subQ (no sx but good for dx) and mucocutaneous bleeding (from decreased factor X or abnl fibrinogen)

Front 51

what do you need to dx amyloid

Back 51

tissue biopsy

Front 52

what is Waldenstrom's macroglobulinemia

Back 52

cancer of B cells that produce IgM. has prop's of low grade lymphoma and PC dysrasia

Front 53

what are some manifestations/sx of waldenstrom's macroglobulinemia

Back 53

1. hyperviscosity syndrome because lg Igm remains intravasc
2. sausaging of retinal vessels (dilate except where they cross)-->optic disc edema and retinal hemorrhages
3. can prduce IgM against self Ags (because autoreactive cells haven't yet been deleted)

Front 54

what are the effects of IgM against self Ags in WM?

Back 54

1. cold agglutinin and intravasc hemolysis
2. IgM against myelin assoc glycoprotein-->peripheral neuropathy
3. against IgG-->cryoglobulinemia

Front 55

how do you dx WM?

Back 55

IgM monoclonal gammopathy and >= 10% BM with IgM and CD20 with plasma cell differentiation

Front 56

txt of WM is similar to txt of what other ds

Back 56

CLL (can use anti-CD20 rituximab)