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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
AML may result in
venous stasis and sludging of blast cells | infarct, hypoxia, and hemorrhage
26
How do you Tx AML
Aggressive! Chemotherapy +/- HCT (hemopoetic cell transplant) High rates of acute toxicity, less responsive to chemo, BUT, 60-75% 5 year survival
27
What is CML associated with
``` Philadelphia chromosome (translocation of 9&22) leading to fusion of BCR gene on 22, and ABL gene on 9 MC in teens, more rare in kids ```
28
Fusion proteins cause
Deregulation of cellular proteins, decreased adherence of cells to extracellular matrix, resistance to apoptosis
29
Sx of CML include
``` bone pain night sweats fever fatigue asymptomatic Severe Sx: dyspnea, priapism, neurologic findings ```
30
How do you diagnose CML
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
How do you treat CML
Hydroxyurea Marrow transplant Tyrosine kinase inhibitor
32
What is lymphoma
malignant proliferation of lymphoid cells arising from lymphoid tissues (nodes, thymus, spleen)
33
What is Hodgkins lymphoma
``` 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
What are Sx oh hodgkins disease
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
What is the Ann Arbor staging system for Hodgkins lymphoma
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
How do you diagnose Hodgkins lymphoma
``` 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
How do yuo Tx hodgkins lymphoma
Refer to comprehensive peds-onc center | Chemo +/- radiation
38
What is non-hodgkins lymphoma
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
What are signs of oncologic emergencies 2/2 non-hodgkins lymphoma
``` 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
What are S/Sx of non-hodgkins lymphoma
Varies depending on type and area Commonly, enlarged, non-tender LAD but CBC, LDH and uric acid may be normal!
41
What are types of non-hodgkins lymphoma
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
How do you treat non-hodgkins lymphoma
Depends on the type! MC combination chemo therapy | 85% five year survival
43
What is a neuroblastoma
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
What are some presenting symptoms of neuroblastoma (depending on location)
``` *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
Workup for neuroblastoma should include
``` Complete H&P CBC CMP Urine or serum catecholamine levels (vanillymandelic acid) Homovanillic acid (elevated in ?90% of cases) Ferritin LDH BIOPSY!! ```
46
How are neuroblastomas staged
Based on international neuroblastoma risk group staging system CT or MRI I123 MIBG
47
How do you treat neuroblastomas
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
What is a wilm's tumor
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
Patients with Wilm's tumor also have
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
What must you always get in evaluating Wilm's tumor
AFP and hCG Homovanillic acid and Vanillymandelic acid *Coags to assess for acquired von Willebrand factor
51
How do you stage and treat wilm's tumor
Stage based on Fifth national wilms tumor study group | Treat based on staging, and determined by children's oncology group recommendations
52
The stages of a wilm's tumor are
``` 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
What is a rhabdomyosarcoma
Soft tissue tumor arising from a primitive mesenchymal cell MC soft tissue tumor in peds but RARE MC <6 y/o
54
What are the 4 major histologic types of rhabdomyosarcoma
Embryonal Botryoid Alveolar Undifferentiated
55
Where are rhabdomyosarcomas more commonly present
Young: head and neck Teens: extremities
56
Morphologically, rhabdomyosarcomas are
similar to small round blue cell tumors of childhood (lymphoma, small cell osteosarcoma, mesenchymal chondrosarcoma, and Ewing sarcoma)- bone cancers@
57
What do you need for tissue Dx of rhabdomyosarcoma
Enough tissue for a routine light microscopic; FNA will not get enough tissue!
58
What is osteosarcoma
``` Primary malignant tumor characterized by production of osteoid or immature bone by malignant cells Bimodal distribution (13-16) and (65+) ```
59
Majority of patients with osteosarcoma present with
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
Osteosarcomas have an affinity for
metaphyseal region of long bones | Distal femur, proximal tibia, proximal humerus, middle and proximal femur, etc.
61
If a child presents with these findings, X-RAY asap!!
Localized pain in one bone and atraumatic
62
Characteristics of an osteosarcoma seen on x-ray include
``` destruction of normal trabecular bone pattern indistinct margins no endosteal bone response Codman's triangle Sunburst pattern ```
63
What do you need for definitive diagnosis of an osteosarcoma
Biopsy! | Once diagnosis is established, get an MRI, CT, oe PET to stage it
64
How do you treat osteosarcomas
*Surgery! Chemo prior to surgery -radiation is NOT helpful-
65
What is Ewing's sarcoma
a spectrum of neoplastic diseases with mesenchymal progenitor cell origin Most often arise in long bones of extremities
66
Ewing's sarcomas are more likely to present
``` Axial skeleton (54%) Appendicular skeleton (42% ```
67
Symptoms that indicate Ewing's sarcoma include
Localized pain or swelling *Night time bone pain* Fever, fatigue, weight loss, and anemia present in small portion
68
How do you work up and treat Ewing's sarcoma
Same as osteosarcoma!
69
What is a retinoblastoma
MC intraocular malignancy of childhood | 1/3 are bilateral
70
With retinoblastoma you may see
White pupillary reflex! (instead of red) | +/- strabismus
71
How do you treat Retinoblastoma
Enucleation (if large) Radioactive plaques (plaque on sclera at base of tumor to preserve vision) Chemo, laser radiation
72
Take home pearls include***
``` 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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