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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.

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2
Q

Presentation of sickle cell disease?

A

recurrent painful episodes (previously called sickle cell crisis

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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

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4
Q

Complications of sickle cell disease?

A

infxs

severe anemia

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

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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

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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

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7
Q

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

A

individual’s report

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8
Q

What is henoch-schonlein purpura?

A

Inflammatory vasculitis, Immunoglobulin A vasculitis

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9
Q

Presentation of henoch-schonlein purpura?

A

usually 3-15 y/o

distinct rash, arthritis, GI, renal

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10
Q

Work up for Henoch-Schönlein purpura?

A

CBC, CMP, ESR, Coags, UA

Rare Skin Bx or Renal Bx

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11
Q

Tx of Henoch-Schönlein purpura?

A

sxs pain control

nephro consult if renal involvement

surg consult if worrisome abd pain (intussusception common)

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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

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13
Q

Epidemiology of ITP?

A

peak in kids 2-5yrs

can present at any age

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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

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15
Q

ITP is usually seen after…

A

viral infection, in otherwise healthy child

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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

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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)

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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&

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19
Q

pathogenesis of ALL

A

Proliferation of immature lymphocytes.

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

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20
Q

s/s of ALL?

A

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

Hepatosplenomegaly

rare: testicular enlargement

unexplained cytopenias, atypical cells, blasts

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21
Q

Dx of ALL? Tx?

A

bone marrow examination: immature lymphoblasts

chemo

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22
Q

Risk factors for AML?

A

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

less common than ALL

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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

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24
Q

Dx of AML?

A

bone marrow biopsy

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25
Tx of AML?
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
26
Epidemiology of CML?
MC in teens and adults
27
genetic abnormality seen in CML?
Philadelphia chromosome (translocation of chromosomes 9 & 22) fusion of BCR gene on 22 & ABL gene on 9 > fusion protein
28
s/s of CML?
bone pain, nt sweats, fever, fatigue or Asymptomatic | Severe Sx: dyspnea, priapism, neurologic findings
29
Dx of CML?
predominance of myeloid cells on peripheral smear, basophils high, low blasts
30
Tx of CML?
hydroxyurea (eliminates Ph+ cells) bone marrow transplants; Imatinib mesylate (Gleevec) tyrosine kinase inhibitor (TKI); targeted TKIs: dastinib, erlotinib, nilotinibm, ponatinib
31
px of CML?
20 yrs. old with bone marrow transplant from matched donor – 70%-80%, unmatched 50%-60%
32
What is lymphoma?
Refers to a malignant proliferation of lymphoid cells Usually arising from lymphoid tissues (lymph nodes, thymus, spleen
33
epidemiology of hodgkin's disease?
Rare in kids < 5 most common childhood cancer in the 15- to 19-year-old age group bimodal peak: adolescents and >50yo
34
What are the 4 types of hodgkin's disease?
Nodular sclerosis (NS) Mixed cellularity (MC) Lymphocyte depleted (LD) Lymphocyte rich (LR)
35
Hodgkin's disease almost always presents at...
the site above the diaphragm
36
s/s of hodgkin's disease?
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
What is used to stage hodgkin's disease?
ann armor staging system
38
Dx of hodgkin's disease?
H&P imaging: CXR, CT chest/abd/pelvis, PET tissue bx: reed sternberg cells* labs: CBC w/ dif, ESR, renal, LFTs, LDH, UA
39
tx of hodgkin's disease?
comprehensive peds oncology center chemo +/- radiation
40
What is non-hodgkin lymphoma?
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
NHL may present with?
potentially emergency complications i.e. pericardial tamponade, acute airway obstruction
42
s/s of NHL?
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
What are the different types of non-hodgkin's lymphoma?
burkitt lymphoma diffuse large b cell lymphoma t cell lymphoma anaplastic large cell lymphoma
44
What is a neuroblastoma?
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
Where do neuroblastomas arise from?
anywhere throughout the sympathetic nervous system
46
s/s of neuroblastomas?
presenting sxs reflect the location abd mass, abd pain periorbital ecchymosis anemia bone pain etc.
47
work up for neuroblastoma?
``` CBC CMP urine or serum catecholamine metabolite levels VMA HVA Ferritin lDH Biopsy ```
48
Staging of neuroblastoma?
International Neuroblastoma Risk Group Staging System (INRGSS) need: CT or MRI, I123 MIBG
49
Avg. age of pt presenting with wilms tumor?
2-5 yrs
50
How are wilms tumors discovered?
usually incidentally by child/parent HTN- 25% can rupture after minor/major abd trauma, causing life threatening situation
51
Work up for Wilm's tumor?
``` US CT/MRI UA CBC CMP, LDH, Uric acid AFP, hCG Coags ```
52
Rhamdomyosarcomas: Head and neck RMS are MC in ____.Extremity tumors present MCly in ___.
younger children adolescents
53
Histology of rhabdomyosarcoma?
to that of other small round blue cell tumors of childhood that involve bone and soft tissue
54
Dx of rhabdomyosarcoma?
tissue dx no FNA - not enough tissue
55
Describe osteosarcomas
uncommon primary malignant tumor characterized by production of osteoid or immature bone by the malignant cells
56
Epidemiology of osteosarcoma?
bimodal early teens (13-16 y/o) -adults over 65
57
Presentation of osteosarcoma?
localized pain, typically of several months' duration PE: soft tissue mass 10-20% have metastatic disease, most often in the lung
58
Dx of osteosarcoma?
xray: 1st dx test - Codman's triangle - "sunburst" pattern biopsy- definitive
59
tx for osteosarcoma
surg chemo (radiation usually not helpful)
60
Where do Ewing's sarcomas usually arise?
in the long bones of the extremities
61
Presentation of Ewing's sarcoma?
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
S/s of retinoblastoma
White pupillary reflex (leukocoria) is most common sign. 1/3 are bilateral may seen strabismus deadily if untreated, rapid mets
63
tx of retinoblastoma?
Enucleation (if large tumor) chemo, laser reduction px overall 95% survival
64
What is important about sickle cell crisis?
it HURTS be liberal with pain meds
65
HSP can have...
renal failure and or surgical abdomen
66
labs seen in ITP?
low PLTs, at risk for life threatening bleeding
67
lymphadenopathy where is never normal?
SUPRACLAVICULAR EPITROCHLEAR
68
Philadelphia chromosome is seen in___
CML
69
Reed sternberg cells are seen in ___
Hodgkins
70
When might you see a child with raccoon eyes?
Neuroblastoma
71
What may cause incidental acquired von willebrand?
wilms tumor

PEDS- Clinical Disciplines Exam 2 (5 decks)

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