Test 3 Flashcards
What is sweet syndrome associated with?
myelodysplasia
what is sweet’s syndrome?
acute neutrophilic dermatosis, pyoderma gangrenosum
is latency for AML longer for alkylating agents or topoisomerase inhibitors?
alkylating agents - 3-7yrs
topoisomerase inhibitors - 1-2 yrs
response to tx for ppl who have MDS after being on alkylating agents?
highly resistant
what growth factors can be used to tx MDS?
G-CSF, GM CSF
EPO (can only use it epo level
what type of chemotx agent is azacytidine and what does it tx?
hypomethylating agent
tx MDS
benefits of azacytidine?
- decr transfusion dependence 50%
2. prolonge time to leukemia: 21 months vs 12 mon
Which phase of CML is this? peripheral and bone marrow blasts
chronic phase of CML
Which phase of CML is this? Progressive splenomegaly, weight loss, fevers, bone pain. Blood or bone marrow blasts >10%, increasing peripheral counts or cytopenias unresponsive to therapy
accelerated phase of CML
which phase of CML is this? Blood or marrow blasts >20%
blast phase of CML
why does imatinib most often fail in tx of CML?
resistance to ABL kinase domain
what is the 1st drug of choice of rtx of polycytheia vera?
hydrea (reduces thrombosis rate–>can normalize platelet and spleen size)
how to tx smoldering multiple myeloma?
watch and wait
what characteristics indicates that a patient with palsmacytoma will have a graeter of chance?
disappearance of M protein and NO active disease = longest stability
is there a correlation b/w amt of amyloid & degree of impairment of organ fxn?
no
what disease do 10% myeloma patients concurrently have?
amyloidosis
what is the greatest predictor of poor outcome in amyloidosis?
cardiac involvement = median survival of 6 months
when is the risk of waldenstroms most likely to progress?
within 1st 5 yrs
difference b/w tx’ing waldenstroms asymptomatic vs symptomatic?
asymptomatic: observe
sx: chemo, plasmapheresis
which familial/genetic diseases are assc’d with incr cancer risk?
neurofibromatosis, familial polyposis, li-fraumeni syndrome
immune deficiencies, metabolic disorders, disorders of chromosome stability
what is a common sx that a kid with a brain tumor presents with?
wakes up in the middle of the night with a HA, then vomits
when are lymph nodes considered large?
when >10 mm
exceptions:
epitrochlear >5 mm; inguinal >15 mm
what type of adenopathy is more likely malignant?
- generalized
- regional not involving the head or neck
- medistinal
describe the texture of lymph nodes that are more likely to be more malignant?
hard/rubbery, non-tender, matted, non-mobile
when is biopsy recommended for LN?
- if nodes are enlarging after 2-3 weeks of antibiotic tx
- nodes that are not enlarging, but have not shrunk in 6-8 weeks
- when present with abnormal CXR
- when present with weight loss, hepatosplenomegaly, fevers, NS
- if LN is posterior auricular, epitrochlear, supraclavicular
is bone pain and early or late presentation?
late sx of cancer (unless Eqings sarcoma or osterosarcoma in which bone pain presents earleir in course of disease)
when to evaluate bone pain?
- persistent pain
- associated swelling/mass
- limited mobility
- wakes at night
- no relief from NSAIDS
which kids are at increased risk for developing ALL?
- Downs
- prenatal exposure to xray
- postnatal exposure to high doses radiation
what is the best predictor of outcome in ppl with ALL?
poor response within 28 days
high WBC (>50k) is prognostic of what?
prognostic in precursor B-ALL, not t-ALL
what age grps have a prognosis of higher risk in ALL?
> 10 years
when to do f/u after chemotx for ALL?
5 yrs post-chemo, then annually for 10 yrs
what % of malignancies do brain tumors comprise?
20%
what is the most common brain tumor?
astrocytoma
parinaud syndrome is associated with what?
incr ICP “setting sun”
which type of brain tumor can you not use surgery for?
optic glioma
can use surgery to tx which types of brain tumors?
medulloblastoma, low grade astro (cerebral), high grade astro, ependymoma
can use XRT to tx what types of brain tumors?
brain stem glioma, ependymoma, germ cell tumor
can use chemo for what types of brain tumors?
medulloblastoma, ependymoma, germ cell tumor
which types of brain tumors do you not need to use surgrery for diagnosis?
GCT, BSG
what type of radiation to use if tumor disseminates to CSF?
neuro axis prophylaxis
what disease are astrocytomas associated with?
NF-1, li-fraumeni syndrome
what is the leading cause of morbidity & mortality in pediatric cancers?
brain tumors
what is the 2nd most common abdominal malignant tumor in children?
wilms