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Flashcards in peds36 Deck (51):
1

isoimmune neutropenia

passive transfer of antineutrophil antibodies from mom to fetus; resolves by 8 weeks of life

2

leading cause of death from disease in childhood

cancer

3

most common childhood cancers

leukemia, brain tumors, lymphoma, neuroblastoma, soft tissue sarcomas, Wilms tumor, and bone tumor

4

wiskott-aldrich syndrome is associated with what cancers?

leukemia and lymphoma; w-a is characterized by B and T cell dysfunciton

5

EBV associated with what cancer?

lymphoma

6

most common malignant abdominal tumors

wilms tumor and neuroblastoma

7

down syndrome associated with what type of cancer?

leukemia

8

turner syndrome associated with what cancer

gonadoblastoma

9

trisomy 13 associated with what cancer

leukemia, teratoma

10

trisomy 18 associated with what cancer

wilms tumor, neurogenic tumors

11

klinefelter syndrome associated with what tumor

leukemia, germ cell tumors, breast cancer

12

fanconi anemia associated with what kind of cancer

leukemia

13

xeroderma pigmentosa associated with what

basal and squamous cell carcinoma, melanoma

14

ataxia telangiectasia assoc with what cancer

lymphoma, leukemia, sarcoma

15

bloom syndrome associated with what cancer

leukemia, lymphoma, GI malignancies, solid tumors

16

beckwith-wiedemann syndrome associated with what cancers

wilms tumor, hepatoblastoma, rhabdomyosarcoma, adrenocortical carcinoma

17

NF type 1 associated with what cancer

brain tumors, lymphoma, leukemia, malignant schwannoma

18

NF type 2 associated with what cancers

acoustic neuroma

19

acute lymphocytic leukemia (ALL) more common in what age and gender

2-6 years olds, boys

20

most common childhood cancer

ALL

21

ALL classification

by cell mophology; L1, L2, L3; L1 is most common;

22

ALL- t cells or b cells or both?

t cell phenotype in 25%, B cell in less than 5%; pre-B cell in 70%

23

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

24

does a normal CBC rule out leukemia?

nope! Confirmation by bone marrow evaluation

25

management of leukemia involves what?

three stages: induction, consolidation, and maintenance

26

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

27

consolidation for leukemia treatment

systemic chemo and prophylaxis to prevent CNS involvement

28

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

29

maintenance therapy for leukemia

involves daily and periodic chemo during remission for up to 3 years

30

what to do for leukemia patients who relapse after remission?

bone marrow transplant

31

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

32

longterm survival for ALL patients

Eighty five percent

33

acute myelogenous leukemia (AML)- how diff from ALL

ALL is b and t cells; AML is grnaulocytes like neutrophils, eosinophils, mast cells

34

how do they classify AML?

M1 through M7; based on cell morphology; M7 is assoc with down syndrome; auer rods in M3

35

Clinical features of AML vs ALL?

CNS involvment more common in AML

36

auer rods

think AML

37

management of AML

very intensive myeloblative therapy to induce remission; bone marrow transplant is recommended once patient is in remission

38

prognosis for AML

chemo works in 50%; BMT works in 70%; therapy more effective in Down syndrome for unknown reason

39

chronic myelogenous leukemia (CML)

least common type of leukemia

40

how is CML classified

adult type and juvenile type

41

adult ctype CML

twice as common as juvenile; older kids; philadelphia chromosome

42

juvenile CML

infants and kids; no philly chrom

43

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

44

clinical features of adult CML

nonspec symptoms; masssive splenomegaly leading to abdominal distension; extremely high WBC

45

clinical features of juvenile CML

fever, chronic eczemalike facial rash, suppurative lymphadenopathy, petechiae and purpura; moderate leuocytosis, anemia, and thrombocytopenia

46

treatment of CML

bone marrow transplant in both adult and juvenile forms; imatinib; radiation therapy is not effective

47

prognosis of adult CML

biphasic course, which chemo working initially but then patient deteriorates and is not responsive

48

prognosis of juvenile CML

often fatal; relapse occures in 50% of cases, even with bone marrow transplan

49

hodgkin's disease

cancer of the antigen processing cells in the lymph nodes or spleen

50

hodgkins disease is associated with what infection?

EBV; if you get EBV-assoc mono, you have a 2-4 times greater risk of hodgkins

51

presenting features in hodgkins

painless lymphadenopathy; most commonly in supraclavicular or cervical regions