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Summer Disciplines 2 > Peds Heme-Onc > Flashcards

Peds Heme-Onc Flashcards

1
Q

What is the hallmark of sickle cell disease

A

Vaso-occlusive phenomenon and hemolysis

vaso-occlusion causes pain known as sickle cell crisis

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2
Q

Sickle cell can lead to

A

acute and chronic pain
tissue ischemia/infarct
Splenic infarct, which leads to hyposplenism in early life

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3
Q

Where is the sickle point mutation

A

Beta globin gene (HbS)

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4
Q

Where is sickle cell mostly prevalent

A

Areas of Africa with heavy presence of malaria; it is thought that people developed sickle cell disease to prevent infection of malaria
Parents who carry the sickle cell trait give their child the trait, but also the immunity to malaria

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5
Q

What are complications of sickle cell disease

A

Infections
Severe anemia (2/2 splenic sequestration, aplastic crisis, Parvoo B19, or hyperhemolysis)
Vaso-occlusive phenomenons
Chronic: pain, anemia, neuro deficits, Sz d/o, pulmonary d/o, renal impairment, HTN, osteoporosis, cardiomyopathy, hepatotoxicity, pigmented gallstones, delayed puberty, chronic leg ulcers, proliferative retinopathy

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6
Q

Symptoms of Vaso-occlusive phenomenons include

A 
acute ain 
stroke 
acute chest syndrome 
renal infarct 
Dactylitis* or bone infarct 
MI
pregnancy complications 
priapism 
VTE
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7
Q

How do you manage sickle cell disease

A

Comprehensive healthcare maintenance
Vaccinations (strep pneumo, flu, N. meningitidis, HIB, HBV)
Antibiotic prophylaxis in first 3 months of life-5 y/o
Folic acid (no iron or vitamin D)
Hydroxyurea (inhibit riboucleotide reductase= more HbF, less HbS)
Routine evaluations (detect and prevent end organ damage)

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8
Q

How do you manage acute chest syndrome 2/2 sickle cell disease

A 
Blood transfusion (NOT for uncomplicated pain in absence of Sx anemia) 
Provider attitudes play a role in Tx 
Prevention 
Acute pain (as per pt) 
Demerol and Toradol are NOT helpful 
**IVF, hydration**
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9
Q

What is Henoch-Schonlein purpura

A 
Inflammatory vasculitis (IgA) primarily in 3-15 y/o
Unknown etiology, but suspected viral, drugs, or vaccinations
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10
Q

Tetrad of Sx associated with Henoch schonlein purpura is

A

Distinct rash (non-blanching purpura on LE and lower trunk)
Arthritis (LE)
GI (abd pain, N/V, bloody stools)
Renal (proteinuria, hematuria)

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11
Q

What workup should you get for Henoch Schonlein purpura

A

CBC, CMP, ESR, Coags, UA

Rare skin Bx or renal Bx

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12
Q

How do you treat Henoch Schonlein

A 
Sx pain control for arthralgias and anti-pyretics
Nephrology consult (renal involved) 
Surgical consult (if worried about intussusception
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13
Q

What is idiopathic thrombocytic purpura

A

Immune thrombocytopenia AKA IgG directed to patient’s own platelets, usually occurring after a viral infx
MCC of thrombocytopenia in children
MC in 2-5 y/o, M>F

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14
Q

ITP presents with

A

Sudden appearance of petechial rash, bruising, bleeding (platelets are ridiculously low <100, w/ otherwise nl CBC)
Always check mucosa and gingiva)

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15
Q

Watch out for these BAD signs on CBC that may indicate malignancy as a cause of thrombocytopenia

A 
Systemic Sx (fever, bone pain, joint pain, weight loss) 
Hepatosplenomegaly 
LAD
Leukocytosis 
Anemia
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16
Q

How do you manage ITP

A

Heme consult
Activity restriction
Avoid antiplatelet meds
If SEVERE bleeding ot plt <30, may use steroids or IVIG
Life threatening bleed: transfuse platelets

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17
Q

What are the 4 types of leukemia

A

Acute Lymphoblastic
Acute Myelogenous
Chronic lymphoblastic
Chronic Myelogenous

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18
Q

What is the MC malignancy of childhood

A

ALL; proliferation of immature lymphocytes, considered leukemia when >25% of marrow is malignant blasts
peak is 4 y/o, M>F, white>black
5 year survival is 85%
If with down’s syndrome, 14x higher risk

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19
Q

Sx of ALL include

A 
Fever 
bleeding 
bone pain (esp long bones) 
LAD 
Hepatosplenomegaly 
Rare: testicular enlargement, peripheral blood abn, low WBC, atypical cells, blasts
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20
Q

How can you tell a physiologic vs pathologic lymph node

A

Smaller than 2cm, swollen, tender, or red are ok
Fixed and non-tender, be worried
Supraclavicular and epitrochlear nodes being palpable is worrisome; start digging!

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21
Q

How do you diagnose ALL

A

Bone marrow examination showing immature lymphoblasts infiltrating the bone marrow

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22
Q

How do you treat ALL

A

Chemotherapy; cure rates >85% but there are still many challenges

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23
Q

What are Sx and Dx for AML

A

Sx same as ALL

Diagnose with bone marrow Bx

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24
Q

AML is associated with

A

children w/ exposure to ionizing radiation, benzenes, and down’s syndrome have 7x higher risk of type M7 AML

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25
Q

AML may result in

A

venous stasis and sludging of blast cells

infarct, hypoxia, and hemorrhage

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26
Q

How do you Tx AML

A

Aggressive!
Chemotherapy
+/- HCT (hemopoetic cell transplant)
High rates of acute toxicity, less responsive to chemo, BUT, 60-75% 5 year survival

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27
Q

What is CML associated with

A 
Philadelphia chromosome (translocation of 9&amp;22) leading to fusion of BCR gene on 22, and ABL gene on 9 
MC in teens, more rare in kids
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28
Q

Fusion proteins cause

A

Deregulation of cellular proteins, decreased adherence of cells to extracellular matrix, resistance to apoptosis

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29
Q

Sx of CML include

A 
bone pain 
night sweats 
fever
fatigue
asymptomatic 
Severe Sx: dyspnea, priapism, neurologic findings
30
Q

How do you diagnose CML

A

Predominance of myeloid cells on peripheral smear, high basophils, low blasts
But, “gold standard” is demonstration of philly chromosome or underlying 9:22 translocation, or BCR-ABL fusion gene

31
Q

How do you treat CML

A

Hydroxyurea
Marrow transplant
Tyrosine kinase inhibitor

32
Q

What is lymphoma

A

malignant proliferation of lymphoid cells arising from lymphoid tissues (nodes, thymus, spleen)

33
Q

What is Hodgkins lymphoma

A 
MC in 15-19 y/o. Bimodal peak (teens and 50 y/o) 
97% occur above the diaphragm 
Can be; 
1. Nodular Sclerosis 
2. Mixed cellularity 
3. Lymphocyte depleted 
4. Lymphocyte rich
34
Q

What are Sx oh hodgkins disease

A

Unexplained weight loss (>10% TBW)
Unexplained recurrent fever (>38C)
Recurrent drenching night sweats
MC: painless cervical or supraclavicular adenopathy
Mediastinal mass causing SOB and cough
+/- hepatic or splenic enlargement if advanced

35
Q

What is the Ann Arbor staging system for Hodgkins lymphoma

A

Stage I: single node or extra-lymphatic organ w/ no nodes
II: 2+ nodes on same side of diaphragm
III: nodes on both sides of diaphragm
IV: spread to extra-lymphatic site

36
Q

How do you diagnose Hodgkins lymphoma

A 
Hx
PE 
CXR, CT, PET scan 
Tissue Bx (excisional, not FNA): *Reed Sternberg cells on lymph node Bx 
Lab evals: CBC, ESR, LFT, Renal, DH, UA
37
Q

How do yuo Tx hodgkins lymphoma

A

Refer to comprehensive peds-onc center

Chemo +/- radiation

38
Q

What is non-hodgkins lymphoma

A

Group of malignant neoplasms derived from B or T cell progenitors, or mature B or T cells
Aggressive clinical behavior
Mean age at Dx is 10 y/o

39
Q

What are signs of oncologic emergencies 2/2 non-hodgkins lymphoma

A 
Superior or inferior vena cava obstruction 
acute airway obstruction 
intestinal obstruction, intussusception 
spinal cord compression 
pericardial tamponade 
Lymph meningitis, CNS mass lesion 
hyperuricemia, tumor lysis syndrome 
ureteral obstruction, uni or b/l hydronephrosis 
VTE disease
40
Q

What are S/Sx of non-hodgkins lymphoma

A

Varies depending on type and area
Commonly, enlarged, non-tender LAD
but CBC, LDH and uric acid may be normal!

41
Q

What are types of non-hodgkins lymphoma

A

Burkitt: mimics acute appendicitis or intussusception, M>F
Diffuse large B cell: rapidly enlarging symptomatic mass, MC neck or abdomen
T cell lymphoblastic: peripheral LAD, respiratory distress, wheezing, SVC syndrome
Anaplastic large cell: painless LAD

42
Q

How do you treat non-hodgkins lymphoma

A

Depends on the type! MC combination chemo therapy

85% five year survival

43
Q

What is a neuroblastoma

A

Spectrum of neuroblastic tumors arising from primative sympathetic ganglion cells
Can arise anywhere throughout the sympathetic nervous system
Mets are common to lymph nodes, bone marrow, cortical bone, dura, orbits, liver, and skin (LESS common to pulm or intracranial sites)

44
Q

What are some presenting symptoms of neuroblastoma (depending on location)

A 
*peri-orbital ecchymosis 
abd mass/ pain 
proptosis 
horner syndrome (miosis, ptosis, anhidrosis) 
localized back pain and weakness
Scoliosis, bladder dysfunction
palpable non-ttp subQ nodules 
systemic Sx, bone pain, anemia, HTN, unilateral nasal obstruction
45
Q

Workup for neuroblastoma should include

A 
Complete H&amp;P 
CBC
CMP
Urine or serum catecholamine levels (vanillymandelic acid) 
Homovanillic acid (elevated in ?90% of cases) 
Ferritin
LDH 
BIOPSY!!
46
Q

How are neuroblastomas staged

A

Based on international neuroblastoma risk group staging system
CT or MRI
I123 MIBG

47
Q

How do you treat neuroblastomas

A

Low risk: surgery
Intermediate risk: Chemo and surgical resection
High risk: chemo, surgery, high dose chemo with stem cell rescue, radiation and immunologic therapy
(prognosis depends on type)

48
Q

What is a wilm’s tumor

A

Developed from nephrogenic cells that contain blastemal, stromal, and/or epithelial cells
Cells are anaplastic with enlarged nuclei, hyperchromastia, and abnormal mitosis
Avg age: 2-5 years
Mostly discovered incidentally

49
Q

Patients with Wilm’s tumor also have

A

HTN (2/2 renal compression and high renin)
Hepatomegaly/varicosities (2/2 extension into IVC or renal veins)
Life threatening situation if rupture occurs s/p abdominal trauma

50
Q

What must you always get in evaluating Wilm’s tumor

A

AFP and hCG
Homovanillic acid and Vanillymandelic acid
*Coags to assess for acquired von Willebrand factor

51
Q

How do you stage and treat wilm’s tumor

A

Stage based on Fifth national wilms tumor study group

Treat based on staging, and determined by children’s oncology group recommendations

52
Q

The stages of a wilm’s tumor are

A 
I: unilateral kidney 
II: tumor invades renal vessels/capsule 
III: a lot of shit- involves nodes, not resectable, vital surfaces, etc.  
IV: distant mets 
V: bilateral renal involvement
53
Q

What is a rhabdomyosarcoma

A

Soft tissue tumor arising from a primitive mesenchymal cell
MC soft tissue tumor in peds but RARE
MC <6 y/o

54
Q

What are the 4 major histologic types of rhabdomyosarcoma

A

Embryonal
Botryoid
Alveolar
Undifferentiated

55
Q

Where are rhabdomyosarcomas more commonly present

A

Young: head and neck
Teens: extremities

56
Q

Morphologically, rhabdomyosarcomas are

A

similar to small round blue cell tumors of childhood (lymphoma, small cell osteosarcoma, mesenchymal chondrosarcoma, and Ewing sarcoma)- bone cancers@

57
Q

What do you need for tissue Dx of rhabdomyosarcoma

A

Enough tissue for a routine light microscopic; FNA will not get enough tissue!

58
Q

What is osteosarcoma

A 
Primary malignant tumor characterized by production of osteoid or immature bone by malignant cells 
Bimodal distribution (13-16) and (65+)
59
Q

Majority of patients with osteosarcoma present with

A

localized pain, typically over several months
10-20% have mets, MC involving the lung*
Usually s/p injury
NO generalized Sx (wt loss, fever, malaise)

60
Q

Osteosarcomas have an affinity for

A

metaphyseal region of long bones

Distal femur, proximal tibia, proximal humerus, middle and proximal femur, etc.

61
Q

If a child presents with these findings, X-RAY asap!!

A

Localized pain in one bone and atraumatic

62
Q

Characteristics of an osteosarcoma seen on x-ray include

A 
destruction of normal trabecular bone pattern 
indistinct margins 
no endosteal bone response 
Codman's triangle 
Sunburst pattern
63
Q

What do you need for definitive diagnosis of an osteosarcoma

A

Biopsy!

Once diagnosis is established, get an MRI, CT, oe PET to stage it

64
Q

How do you treat osteosarcomas

A

*Surgery!
Chemo prior to surgery
-radiation is NOT helpful-

65
Q

What is Ewing’s sarcoma

A

a spectrum of neoplastic diseases with mesenchymal progenitor cell origin
Most often arise in long bones of extremities

66
Q

Ewing’s sarcomas are more likely to present

A 
Axial skeleton (54%) 
Appendicular skeleton (42%
67
Q

Symptoms that indicate Ewing’s sarcoma include

A

Localized pain or swelling
Night time bone pain
Fever, fatigue, weight loss, and anemia present in small portion

68
Q

How do you work up and treat Ewing’s sarcoma

A

Same as osteosarcoma!

69
Q

What is a retinoblastoma

A

MC intraocular malignancy of childhood

1/3 are bilateral

70
Q

With retinoblastoma you may see

A

White pupillary reflex! (instead of red)

+/- strabismus

71
Q

How do you treat Retinoblastoma

A

Enucleation (if large)
Radioactive plaques (plaque on sclera at base of tumor to preserve vision)
Chemo, laser radiation

72
Q

Take home pearls include***

A 
Sickle cell HURTS 
HSP: renal failure, surgical abdomen 
ITP: low platelets and risk for life threatening bleed
ALL and AML: epitrochlear and supraclavicular LAD 
CML: philidelphia chromosome 
Petechiae are NOT NORMAL ever** 
Hodgkins: reed sternberg cells 
Neuroblastoma: racoon eyes 
Wilms: incidental acquired VWF
Any abd mass in peds is malignant until proven otherwise! 
Rhabdo: small blue cell 
Osteosarcoma: get an x-ray 
Ewing's: long bones 
Retinoblastoma: white eye

Summer Disciplines 2 (8 decks)

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