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Peds > peds36 > Flashcards

peds36 Flashcards

1
Q

isoimmune neutropenia

A

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

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2
Q

leading cause of death from disease in childhood

A

cancer

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3
Q

most common childhood cancers

A

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

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4
Q

wiskott-aldrich syndrome is associated with what cancers?

A

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

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5
Q

EBV associated with what cancer?

A

lymphoma

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6
Q

most common malignant abdominal tumors

A

wilms tumor and neuroblastoma

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7
Q

down syndrome associated with what type of cancer?

A

leukemia

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8
Q

turner syndrome associated with what cancer

A

gonadoblastoma

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9
Q

trisomy 13 associated with what cancer

A

leukemia, teratoma

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10
Q

trisomy 18 associated with what cancer

A

wilms tumor, neurogenic tumors

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11
Q

klinefelter syndrome associated with what tumor

A

leukemia, germ cell tumors, breast cancer

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12
Q

fanconi anemia associated with what kind of cancer

A

leukemia

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13
Q

xeroderma pigmentosa associated with what

A

basal and squamous cell carcinoma, melanoma

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14
Q

ataxia telangiectasia assoc with what cancer

A

lymphoma, leukemia, sarcoma

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15
Q

bloom syndrome associated with what cancer

A

leukemia, lymphoma, GI malignancies, solid tumors

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16
Q

beckwith-wiedemann syndrome associated with what cancers

A

wilms tumor, hepatoblastoma, rhabdomyosarcoma, adrenocortical carcinoma

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17
Q

NF type 1 associated with what cancer

A

brain tumors, lymphoma, leukemia, malignant schwannoma

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18
Q

NF type 2 associated with what cancers

A

acoustic neuroma

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19
Q

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

A

2-6 years olds, boys

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20
Q

most common childhood cancer

A

ALL

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21
Q

ALL classification

A

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

22
Q

ALL- t cells or b cells or both?

A

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

23
Q

ALL on CBC?

A

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
Q

does a normal CBC rule out leukemia?

A

nope! Confirmation by bone marrow evaluation

25
Q

management of leukemia involves what?

A

three stages: induction, consolidation, and maintenance

26
Q

indiction for leukemia treatment

A

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
Q

consolidation for leukemia treatment

A

systemic chemo and prophylaxis to prevent CNS involvement

28
Q

what treatments are used for consolidation

A

intrathecal metotrexate is continued; cranial radiaion may be given to high risk kids but avoided in kids less than 5

29
Q

maintenance therapy for leukemia

A

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

30
Q

what to do for leukemia patients who relapse after remission?

A

bone marrow transplant

31
Q

metabolic complications from spontaneous or therapy induced cell lysis (leukemia was example)

A

aka tumor lysis syndrome; hyperuricemia may lead to renal insuff; hyperkalemia may lead to cardiac dysrhythmias, hyperphosphatemia my lead to hypocalcemia with tetany

32
Q

longterm survival for ALL patients

A

Eighty five percent

33
Q

acute myelogenous leukemia (AML)- how diff from ALL

A

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

34
Q

how do they classify AML?

A

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

35
Q

Clinical features of AML vs ALL?

A

CNS involvment more common in AML

36
Q

auer rods

A

think AML

37
Q

management of AML

A

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

38
Q

prognosis for AML

A

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

39
Q

chronic myelogenous leukemia (CML)

A

least common type of leukemia

40
Q

how is CML classified

A

adult type and juvenile type

41
Q

adult ctype CML

A

twice as common as juvenile; older kids; philadelphia chromosome

42
Q

juvenile CML

A

infants and kids; no philly chrom

43
Q

phily chorm

A

reciprocal translocation between the long arms of choms 9 and 22, leading to fusion of BCR/ABL1 that produces BCR-ABL fusion protein

44
Q

clinical features of adult CML

A

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

45
Q

clinical features of juvenile CML

A

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

46
Q

treatment of CML

A

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

47
Q

prognosis of adult CML

A

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

48
Q

prognosis of juvenile CML

A

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

49
Q

hodgkin’s disease

A

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

50
Q

hodgkins disease is associated with what infection?

A

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

51
Q

presenting features in hodgkins

A

painless lymphadenopathy; most commonly in supraclavicular or cervical regions

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