Peds Heme/Onc Flashcards

1
Q

What are the clinical hallmarks of sickle cell disease?

A

vaso-occlusive phenomena and hemolysis

can lead to acute and chronic pain and tissue ischemia or infarction

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2
Q

What is the mutation involved in sickle cell disease?

A

point mutation in the beta globin gene

results in sickle hemoglobin= HbS

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3
Q

Acute complications of sickle cell dz

A
  • infections
  • severe anemia
  • aplastic crisis-Parvo B19
  • Vaso-occlusive phenomena
  • Dactylitis
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4
Q

What are some of the recommendations for sickle cell dz?

A
  • antibiotic prophylaxis (w/in first 3 mo until age 5)
  • Folic acid w/o iron, Vit D and calcium
  • Hydroxyurea
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5
Q

What should and should not be used in sickle cell crisis?

A

Should: IVF, hydration

NOT= demerol and toradol

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6
Q

What is Henoch-Schonlein purpura?

A

inflammatory vasculitis

Immunoglobulin A vasculitis

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7
Q

What is the tetra of sx in Henoch-Schonlein purpura?

A
  • Distinct rash (purpuric non-blanching on legs/trunk)
  • Arthritis
  • GI- abd pain, N/V
  • Renal- proteinuria, hematuria
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8
Q

Sudden appearance of petechial rash, bruising, and/or bleeding in an otherwise healthy child, what do you suspect?

A

Idiopathic Thrombocytopenic purpura

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9
Q

What is the normal platelet count in Idiopathic Thrombocytopenic purpura

A

<100,000 (LOW!)

need to watch and eval for spontaneous bleed

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10
Q

MC malignancy of childhood?

A

Acute lymphocytic leukemia

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11
Q

Who is at 14x risk of having Acute lymphocytic leukemia

A

Pt’s w/ Down Syndrome

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12
Q

Sx of Acute lymphocytic leukemia

A
  • Supraclavicular-epitrochlear lymphadenopathy*
  • fever, bleeding, bone pain
  • hepatosplenomegaly
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13
Q

Dx and tx for Acute lymphocytic leukemia

A

dx= bone marrow biopsy

tx= chemo

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14
Q

What chromosome abnormality is involved in Chronic Myelocytic Leukemia?

A
Philadelphia chromosome
(translocation of chromosomes 9 and 22)
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15
Q

What is the “Radiologist sign”

A

swollen lymph node in neck that is visible when the pt turns head?

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16
Q

MC childhood CA in 15-19 yos

A

Hodgkins Lymphoma

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17
Q

MC sign of Hodgkins Disease?

A
  • Painless cervical or supraclavicular adenopathy*

- mediastinal mass causing cough/SOB

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18
Q

What are the 3 “B” sx that indicate bad prognosis

(In Hodgkins Disease slide?)

A
  1. Unexplained weight loss
  2. unexplained persistent fever
  3. recurrent drenching night sweats
19
Q

What staging system is used for Hodgkins disease?

A

Ann Arbor staging system

20
Q

What is used for dx in Hodgkins disease? (pathology)

A

tissue biopsy= Reed-Sternberg cells!!

21
Q

Signs and sx of non-Hodgkins lymphoma

A

enlarging, non-tender lymphadenopathy

22
Q

Burkitt lymphoma mimics…

A

acute appendicitis or intussusception

23
Q

Sx of Neuroblastoma?

A

presenting sx reflect the location of the primary tumor

periorbital ecchymosis “Raccoon eyes

24
Q

Average age of pt with Wilms tumor?

A

2-5 yo

25
Q

How are Wilms tumors normally found?

A

Incidentally

25% of pts have HTN

26
Q

For any pediatric pt, an abdominal mass is considered??

A

Malignant until proven otherwise

27
Q

What type of cell does rhabdomyosarcoma arise from?

A

primitive mesenchymal cell

28
Q

Histologic appearance of rhabdomyosarcoma?

A

small round blue cell

29
Q

Peak age of osteosarcoma

A

13-16

30
Q

At time of dx, is metastasis common in osteosarcoma?

A

yes, 10-20% have metastases. MC to the lung

31
Q

Where in the bones do osteosarcomas have a predilection for?

A

metaphyseal region of long bones

MC= distal femur, prox tibia

32
Q

localized pain, atraumatic in any child in one bone/extremity=
what do you do?

A

XRAY

33
Q

what are the 2 characteristic XRAY findings in osteosarcoma?

A
  • Codman’s triangle

- “Sunburst” pattern

34
Q

Ewing’s sarcoma- MC arise from what bones?

A

Long bones of the extremities

35
Q

Common sx of a pt w/ Ewing’s Sarcoma?

A
  • nighttime bone pain**
  • localized pain or swelling
  • fever, fatigue, weight loss
36
Q

MC sign of Retinoblastoma?

A

White pupillary reflex (leukocoria)

37
Q

Philadelphia chromosome

A

CML

38
Q

Reed Sternburg Cells

A

Hodgkins

39
Q

Raccoon eyes

A

Neurobastoma

40
Q

Any pediatric mass in abdomen=?

A

malignant until proven otherwise

41
Q

Small blue cell

A

Rhabdo

42
Q

Long bones

A

Ewings

43
Q

White eye

A

Retinoblastoma