peds36 Flashcards
isoimmune neutropenia
passive transfer of antineutrophil antibodies from mom to fetus; resolves by 8 weeks of life
leading cause of death from disease in childhood
cancer
most common childhood cancers
leukemia, brain tumors, lymphoma, neuroblastoma, soft tissue sarcomas, Wilms tumor, and bone tumor
wiskott-aldrich syndrome is associated with what cancers?
leukemia and lymphoma; w-a is characterized by B and T cell dysfunciton
EBV associated with what cancer?
lymphoma
most common malignant abdominal tumors
wilms tumor and neuroblastoma
down syndrome associated with what type of cancer?
leukemia
turner syndrome associated with what cancer
gonadoblastoma
trisomy 13 associated with what cancer
leukemia, teratoma
trisomy 18 associated with what cancer
wilms tumor, neurogenic tumors
klinefelter syndrome associated with what tumor
leukemia, germ cell tumors, breast cancer
fanconi anemia associated with what kind of cancer
leukemia
xeroderma pigmentosa associated with what
basal and squamous cell carcinoma, melanoma
ataxia telangiectasia assoc with what cancer
lymphoma, leukemia, sarcoma
bloom syndrome associated with what cancer
leukemia, lymphoma, GI malignancies, solid tumors
beckwith-wiedemann syndrome associated with what cancers
wilms tumor, hepatoblastoma, rhabdomyosarcoma, adrenocortical carcinoma
NF type 1 associated with what cancer
brain tumors, lymphoma, leukemia, malignant schwannoma
NF type 2 associated with what cancers
acoustic neuroma
acute lymphocytic leukemia (ALL) more common in what age and gender
2-6 years olds, boys
most common childhood cancer
ALL
ALL classification
by cell mophology; L1, L2, L3; L1 is most common;
ALL- t cells or b cells or both?
t cell phenotype in 25%, B cell in less than 5%; pre-B cell in 70%
ALL on CBC?
anemia with thrombocytopenia; the bone marrow is overwhelmed by producing these abnormal B or T cells; total WBC can be high, normal or low
does a normal CBC rule out leukemia?
nope! Confirmation by bone marrow evaluation
management of leukemia involves what?
three stages: induction, consolidation, and maintenance
indiction for leukemia treatment
aims to destory as many cancer cell as possible; drugs vary; intrathecal methotrexate is given to all children during induction; remission is induced in 95% of kids
consolidation for leukemia treatment
systemic chemo and prophylaxis to prevent CNS involvement
what treatments are used for consolidation
intrathecal metotrexate is continued; cranial radiaion may be given to high risk kids but avoided in kids less than 5
maintenance therapy for leukemia
involves daily and periodic chemo during remission for up to 3 years
what to do for leukemia patients who relapse after remission?
bone marrow transplant
metabolic complications from spontaneous or therapy induced cell lysis (leukemia was example)
aka tumor lysis syndrome; hyperuricemia may lead to renal insuff; hyperkalemia may lead to cardiac dysrhythmias, hyperphosphatemia my lead to hypocalcemia with tetany
longterm survival for ALL patients
Eighty five percent
acute myelogenous leukemia (AML)- how diff from ALL
ALL is b and t cells; AML is grnaulocytes like neutrophils, eosinophils, mast cells
how do they classify AML?
M1 through M7; based on cell morphology; M7 is assoc with down syndrome; auer rods in M3
Clinical features of AML vs ALL?
CNS involvment more common in AML
auer rods
think AML
management of AML
very intensive myeloblative therapy to induce remission; bone marrow transplant is recommended once patient is in remission
prognosis for AML
chemo works in 50%; BMT works in 70%; therapy more effective in Down syndrome for unknown reason
chronic myelogenous leukemia (CML)
least common type of leukemia
how is CML classified
adult type and juvenile type
adult ctype CML
twice as common as juvenile; older kids; philadelphia chromosome
juvenile CML
infants and kids; no philly chrom
phily chorm
reciprocal translocation between the long arms of choms 9 and 22, leading to fusion of BCR/ABL1 that produces BCR-ABL fusion protein
clinical features of adult CML
nonspec symptoms; masssive splenomegaly leading to abdominal distension; extremely high WBC
clinical features of juvenile CML
fever, chronic eczemalike facial rash, suppurative lymphadenopathy, petechiae and purpura; moderate leuocytosis, anemia, and thrombocytopenia
treatment of CML
bone marrow transplant in both adult and juvenile forms; imatinib; radiation therapy is not effective
prognosis of adult CML
biphasic course, which chemo working initially but then patient deteriorates and is not responsive
prognosis of juvenile CML
often fatal; relapse occures in 50% of cases, even with bone marrow transplan
hodgkin’s disease
cancer of the antigen processing cells in the lymph nodes or spleen
hodgkins disease is associated with what infection?
EBV; if you get EBV-assoc mono, you have a 2-4 times greater risk of hodgkins
presenting features in hodgkins
painless lymphadenopathy; most commonly in supraclavicular or cervical regions
