Brainscape's Knowledge GenomeTM

Browse over 1 million classes created by top students, professors, publishers, and experts.


See full index

PEDS- Clinical Disciplines Exam 2 > Peds Hem/Onc > Flashcards

Peds Hem/Onc Flashcards

1
Q

What is sickle cell disease?

A

Vaso-occlusive phenomena and hemolysis are the clinical hallmarks of sickle cell disease (SCD). Can lead to acute and chronic pain and tissue ischemia or infarction. Splenic infarction leads to functional hyposplenism early in life.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

Presentation of sickle cell disease?

A

recurrent painful episodes (previously called sickle cell crisis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

abnormality seen in sickle cell disease?

A

sickle point mutation in the beta globin gene results in the production of sickle hemoglobin. HbS

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

Complications of sickle cell disease?

A

infxs

severe anemia

vaso-oculsive phenomena: stroke, MI, dactylitis, lots more

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

Tx of sickle cell disease?

A

vaccinations

abx prophylaxis: first 3 months of life- 5yrs

folic acid, MVI without iron replacement vit D and Ca

Hydroxyurea

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

When would you give a blood transfusion to a child with sickle cell disease?

A

therapy (typically for life-threatening SCD related comp) or for prophylaxis, to decrease the incidence of specific SCD related complications

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

What is the gold standard for assessment of pain in sickle cell disease?

A

individual’s report

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

What is henoch-schonlein purpura?

A

Inflammatory vasculitis, Immunoglobulin A vasculitis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

Presentation of henoch-schonlein purpura?

A

usually 3-15 y/o

distinct rash, arthritis, GI, renal

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

Work up for Henoch-Schönlein purpura?

A

CBC, CMP, ESR, Coags, UA

Rare Skin Bx or Renal Bx

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

Tx of Henoch-Schönlein purpura?

A

sxs pain control

nephro consult if renal involvement

surg consult if worrisome abd pain (intussusception common)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

What is Idiopathic Thrombocytopenic Purpura?

A

aka Immune ThrombocytoPenia (ITP)

immune-mediated thrombocytopenia (peripheral blood platelet count <100,000/microL)
IgG directed to patient’s own platelets

MC cause of thrombocytopenia in children

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

Epidemiology of ITP?

A

peak in kids 2-5yrs

can present at any age

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

Presentation of ITP?

A

sudden appearance of a petechial rash, bruising, and/or bleeding in an otherwise healthy child

look at mucosa & gingiva

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

ITP is usually seen after…

A

viral infection, in otherwise healthy child

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

If you see low platelets + other abnormalities on CBC, should you suspect ITP?

A

NO, not if there are other abn. on CBC

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
17
Q

management of ITP?

A

Hem consult

activity restriction

avoid antiplatelets/anticoags

regular monitoring of platelets

+/- steroids, IV IG

(usually spontaneously recovering 3 mos)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
18
Q

What is the MC malignancy of childhood? epidemiology?

A

ALL

peak 4 yrs

M >F

caucasians > African American

14x risk in Down’s pts

survival 85&

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
19
Q

pathogenesis of ALL

A

Proliferation of immature lymphocytes.

Leukemia = >25% malignant hematopoetic cells (blasts) in the bone marrow aspirate.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
20
Q

s/s of ALL?

A

Fever, bleeding, bone pain (esp. long bones), lymphadenopathy

Hepatosplenomegaly

rare: testicular enlargement

unexplained cytopenias, atypical cells, blasts

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
21
Q

Dx of ALL? Tx?

A

bone marrow examination: immature lymphoblasts

chemo

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
22
Q

Risk factors for AML?

A

Children w/exposure to ionizing radiation, benzenes, Down’s at increased risk

less common than ALL

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
23
Q

S/s of AML?

A

same as AML

Fever, bleeding, bone pain (esp. long bones), lymphadenopathy

Hepatosplenomegaly

rare: testicular enlargement

unexplained cytopenias, atypical cells, blasts

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
24
Q

Dx of AML?

A

bone marrow biopsy

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
25
Q

Tx of AML?

A

very aggressive and includes intense chemotherapy, and in some cases HCT, and has high rates of acute toxicity, especially infections

less responsive to chemo, px 65-75% 5 yr survival

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
26
Q

Epidemiology of CML?

A

MC in teens and adults

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
27
Q

genetic abnormality seen in CML?

A

Philadelphia chromosome (translocation of chromosomes 9 & 22) fusion of BCR gene on 22 & ABL gene on 9 > fusion protein

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
28
Q

s/s of CML?

A

bone pain, nt sweats, fever, fatigue or Asymptomatic

Severe Sx: dyspnea, priapism, neurologic findings

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
29
Q

Dx of CML?

A

predominance of myeloid cells on peripheral smear, basophils high, low blasts

30
Q

Tx of CML?

A

hydroxyurea (eliminates Ph+ cells)

bone marrow transplants; Imatinib mesylate (Gleevec)

tyrosine kinase inhibitor (TKI);

targeted TKIs: dastinib, erlotinib, nilotinibm, ponatinib

31
Q

px of CML?

A

20 yrs. old with bone marrow transplant from matched donor – 70%-80%, unmatched 50%-60%

32
Q

What is lymphoma?

A

Refers to a malignant proliferation of lymphoid cells

Usually arising from lymphoid tissues (lymph nodes, thymus, spleen

33
Q

epidemiology of hodgkin’s disease?

A

Rare in kids < 5

most common childhood cancer in the 15- to 19-year-old age group

bimodal peak: adolescents and >50yo

34
Q

What are the 4 types of hodgkin’s disease?

A

Nodular sclerosis (NS)

Mixed cellularity (MC)

Lymphocyte depleted (LD)

Lymphocyte rich (LR)

35
Q

Hodgkin’s disease almost always presents at…

A

the site above the diaphragm

36
Q

s/s of hodgkin’s disease?

A

unexplained weight loss of more than 10% body weight

persistent/recurrent fever

night sweats

MC sign: painless cervical or supraclavicular adenopathy

mediastinal mass causing cough/SOB

37
Q

What is used to stage hodgkin’s disease?

A

ann armor staging system

38
Q

Dx of hodgkin’s disease?

A

H&P

imaging: CXR, CT chest/abd/pelvis, PET

tissue bx: reed sternberg cells*

labs: CBC w/ dif, ESR, renal, LFTs, LDH, UA

39
Q

tx of hodgkin’s disease?

A

comprehensive peds oncology center

chemo +/- radiation

40
Q

What is non-hodgkin lymphoma?

A

consists of a diverse group of malignant neoplasms of the lymphoid tissues variously derived from B cell progenitors, T cell progenitors, mature B cells, or mature T cells

41
Q

NHL may present with?

A

potentially emergency complications

i.e. pericardial tamponade, acute airway obstruction

42
Q

s/s of NHL?

A

Varies

sxs develop quickly, usually over one to three weeks

commonly presents as enlarging, non-tender lymphadenopathy or as sxs due to the compression of surrounding structures

43
Q

What are the different types of non-hodgkin’s lymphoma?

A

burkitt lymphoma

diffuse large b cell lymphoma

t cell lymphoma

anaplastic large cell lymphoma

44
Q

What is a neuroblastoma?

A

spectrum of neuroblastic tumors (including neuroblastomas, ganglioneuroblastomas, and ganglioneuromas) that arise from primitive sympathetic ganglion cells

have the capacity to synthesize and secrete catecholamines

45
Q

Where do neuroblastomas arise from?

A

anywhere throughout the sympathetic nervous system

46
Q

s/s of neuroblastomas?

A

presenting sxs reflect the location

abd mass, abd pain

periorbital ecchymosis

anemia

bone pain

etc.

47
Q

work up for neuroblastoma?

A 
CBC 
CMP
urine or serum catecholamine metabolite levels 
VMA 
HVA 
Ferritin 
lDH 
Biopsy
48
Q

Staging of neuroblastoma?

A

International Neuroblastoma Risk Group Staging System (INRGSS)

need: CT or MRI, I123 MIBG

49
Q

Avg. age of pt presenting with wilms tumor?

A

2-5 yrs

50
Q

How are wilms tumors discovered?

A

usually incidentally by child/parent

HTN- 25%

can rupture after minor/major abd trauma, causing life threatening situation

51
Q

Work up for Wilm’s tumor?

A 
US 
CT/MRI 
UA
CBC
CMP, LDH, Uric acid 
AFP, hCG
Coags
52
Q

Rhamdomyosarcomas: Head and neck RMS are MC in ____.Extremity tumors present MCly in ___.

A

younger children

adolescents

53
Q

Histology of rhabdomyosarcoma?

A

to that of other small round blue cell tumors of childhood that involve bone and soft tissue

54
Q

Dx of rhabdomyosarcoma?

A

tissue dx

no FNA - not enough tissue

55
Q

Describe osteosarcomas

A

uncommon primary malignant tumor

characterized by production of osteoid or immature bone by the malignant cells

56
Q

Epidemiology of osteosarcoma?

A

bimodal

early teens (13-16 y/o) -adults over 65

57
Q

Presentation of osteosarcoma?

A

localized pain, typically of several months’ duration

PE: soft tissue mass

10-20% have metastatic disease, most often in the lung

58
Q

Dx of osteosarcoma?

A

xray: 1st dx test
- Codman’s triangle
- “sunburst” pattern

biopsy- definitive

59
Q

tx for osteosarcoma

A

surg

chemo

(radiation usually not helpful)

60
Q

Where do Ewing’s sarcomas usually arise?

A

in the long bones of the extremities

61
Q

Presentation of Ewing’s sarcoma?

A

Localized pain or swelling of a few weeks’ or months’ duration. Nighttime bone pain.

trauma

fever, fatigue, weight loss, anemia

work up same as osteosarcoma

62
Q

S/s of retinoblastoma

A

White pupillary reflex (leukocoria) is most common sign.

1/3 are bilateral

may seen strabismus

deadily if untreated, rapid mets

63
Q

tx of retinoblastoma?

A

Enucleation (if large tumor)

chemo, laser reduction

px overall 95% survival

64
Q

What is important about sickle cell crisis?

A

it HURTS

be liberal with pain meds

65
Q

HSP can have…

A

renal failure and or surgical abdomen

66
Q

labs seen in ITP?

A

low PLTs, at risk for life threatening bleeding

67
Q

lymphadenopathy where is never normal?

A

SUPRACLAVICULAR

EPITROCHLEAR

68
Q

Philadelphia chromosome is seen in___

A

CML

69
Q

Reed sternberg cells are seen in ___

A

Hodgkins

70
Q

When might you see a child with raccoon eyes?

A

Neuroblastoma

71
Q

What may cause incidental acquired von willebrand?

A

wilms tumor

PEDS- Clinical Disciplines Exam 2 (5 decks)

Brainscape helps you reach your goals faster, through stronger study habits.
© 2024 Bold Learning Solutions. Terms and Conditions