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Pediatric Board Prep > Heme/Onc > Flashcards

Heme/Onc Flashcards

0
Q

Disorders at increased risk if ALL:

A

Downs, ataxia telengiectasia, Bloom syndrome, fanconi anemia

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1
Q

What must a bone marrow biopsy show to diagnose ALL?

A

25%blasts

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2
Q

Drug- name the alkylating agents and their SE:

A

Cyclophosphamide: inhibits DNA synthesis (hemorrhagic cystitis)

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3
Q

Drugs- name the antimetabolites and their SE:

A

MTX: folic acid antagonist (myelosuppression, hepatotoxic, oral and GI ulcers)
6-MP: inhibits purine synthesis (myelosuppression, hepatotoxic)
Ara-C: inhibits DNA polymerase (myelosuppression)

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4
Q

Drugs- name the chemo antibiotics and their SE:

A

Doxorubicin & daunorubicin: bind to DNA (cardiomyopathy)

Bleomycin: binds to DNA (pulmonary fibrosis)

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5
Q

Drugs- name the chemo enzymes and their SE:

A

L-asparaginase: depletes L-asparagine (pancreatitis, increased glucose)

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6
Q

Drugs- name the vinca alkaloids and their SE:

A

Vincristine: inhibits micro tubule formation (peripheral neuropathy, SIADH)
Vinblastine: inhibits micro tubule formation (leukopenia)

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7
Q

What is M3 AML also called?

A

Acute promyelocytic leukemia- t(15;17), DIC at presentation. Treated with chemo + retinoic acid

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8
Q

Drugs- SE of cisplatin & etoposide (VP-16):

A

Cisplatin: inhibits DNA synthesis (nephro toxic, ototoxic, neurotoxic)

VP-16: topoisomerase inhibitor (secondary leukemias)

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9
Q

What findings in blasts on a bone marrow biopsy is associated with AML?

A

Auer rods

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10
Q

What is the Philadelphia chromosome?

A

CML with t(9;22) translocation -> bcr-abl abnormal protein

Tmt: BMT, hydrourea, interferon alpha, tyrosine kinase inhibitors

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11
Q

How does JMML present?

A

Juvenile myelomonocytic leukemia: <2 yrs of age, massive splenomegaly, leukocytosis/thrombocytopenia, ski findings (xanthoma, cafe au lait, eczema)

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12
Q

What is the most common presentation for Hodgkin disease?

A

Asymptomatic cervical or supraclavicular LAN

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13
Q

What are Pel-Ebstein fevers?

A

Classically associated with Hodgkin lymphoma, several days of fever followed by afebrile episodes (periodic type fevers)

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14
Q

What are the chemotherapies commonly used in Hodgkins?

A

MOPP = nitrogen Mustard, vincristine(Oncovin), Procarbazine, Prednisone

ABVD = Adriamycin, Bleomycin, Vincristine, Dacarbazine

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15
Q

What lab values are seen with tumor lysis? Prevention?

A

Hyperkalemia
Hyerphosphatemia
Renal insufficiency

Hydration, allopurinol (inhibits urate toxicity)

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16
Q

What is the most common malignancy in infants?

A

Neuroblastoma

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17
Q

Which ages of children have the best prognosis in Neuroblastoma?

A

< 1 year

4S staging has a good prognosis and is <1 with small tumor and dissemination in only liver and skin

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18
Q

What two metabolites can be useful in Neuroblastoma diagnosis and screening?

A

HVA and VMA

19
Q

What disorders are associated with Wilma tumor?

A

WAGR (Wilms, aniridia, GU abn, mental Retardation)

BWS

Denys Drash: Wilms, nephropathy, male pseudo hermaphroditism

20
Q

Most common soft tissue tumor of childhood:

A

Rhabdomyosarcoma

21
Q

What is the radio graphic appearance of osteosarcoma v Ewing’s sarcoma?

A

Osteosarcoma: metaphysis, sunburst

Ewing’s: diaphysis, lytic, onion skinning, more likely to have systemic symptoms

22
Q

What chromosomal abnormality is associated with Ewing’s sarcoma?

A

T(11,22) mostly, some t(21,22)

23
Q

What is an ostechondroma?

A

Benign bone tumor, occurs in metaphysis, stalk projection with cartilage cap

24
Q

What is an osteoid osteoma?

A

Benign tumor with unremitted pain, worse at night, relieved with aspirin. Lucency with sclerotic bone

25
Q

What tumor is associated with tuberous sclerosis?

A

Subependymal giant cell tumor

26
Q

What is the most common malignant CNS tumor?

A

Primitive neurrctodermal tumors (medulloblastoma, pineoblastoma, central Neuroblastoma)

27
Q

What is the most common CNS tumor?

A

Glioma (cerebellar astrocytoma & brainstem gliomas & high grade astrocytoma)

28
Q

What are some germ cell CNS tumors? What so they often secrete?

A

Pineal tumor, germinomas, teratoma. Alpha feto protein and hcg

29
Q

What is parinaud syndrome? What is it seen with?

A

Triad of (1) impaired upward gaze (2) dilated pupils with better reactivity to accommodation than light (3) conversion/retraction nystagmus. Seen with pineal germ cell tumors or pinealblastoma

30
Q

What disease is meningioma associated with?

A

NF2

31
Q

Where are most teratomas located?

A

Sacrococcyx, ovaries, testes, anterior mediastinum

32
Q

What teratoma is most prone to malignant transformation?

A

Sacrococcygeal teratoma in infants over two months

33
Q

Name the germ cell tumors and their associated tumor markers:

A 
Teratoma- typically none
Germinoma- marker negative
Embryonal carcinoma- AFP & Bhcg
Yolk sac (endodermal)- AFP +
Choriocarcinoma- Bhcg +
Gonadoblastoma- marker neg
Sex cord tumors- leydig ->androgens, sertoli->estrogen
34
Q

What tissue if found in a chorio carcinoma?

A

Syncytiotrophoblast tissue

35
Q

What virus is associated with nasopharyngeal carcinoma?

A

EBV

36
Q

What pancreatic tumor is associated with AD MEN-1?

A

Insulinoma and gastrinoma

37
Q

What is the most common benign liver tumor?

A

Hemangioendothelioma, if under two, multiple lesions and AFP normal, think this.

38
Q

Common findings with Hand-Schuller-Christian disease:

A 
Mulitfocal LCH:
Skull lytic lesions
DI
Exopthalmos
Erosion of lamina dura or premature eruption of teeth
Gingival hemorrhage
Oral mucosa irritation
39
Q

anterior mediastinal tumors

A

thymoma, teratoma, thyroid, t-cell lymphoma

*high risk of airway compromise, do not anesthetize

40
Q

Mentzer index:

A

MCV/red blood cell count

<13 suggests beta thal trait

41
Q

macrocytic anemia buzzwords

A
  1. B12 def: vegan, pernicious anemia
  2. Folate: goats milk, dx with erythrocyte folic acid level and r/o B12 def prior to tx
  3. Diamond Blackfan: triphalyngeal thumbs
  4. Fanconi: AR, short stature, café au laits, absent thumb/radial anomalies, HgF high
42
Q

microcytic anemia buzzwords

A
  1. iron deficiency: high RDW
  2. beta thalassemia: high Hgb A2, Mentzer Index decreased, major (Cooleys)/intermedia/minor, target cells
  3. alpha thal: carrier, trait/minor, HBH, hydrops fetalis
  4. lead poisoning: ringed sideroblast, tx penicillamine/dimercaprol or EDTA
  5. anemia of chronic disease: TIBC low, high ferritin, tx underlying illness
43
Q

normocytic anemia buzzwords

A
  1. SCD: AR, val instead of glutamic acid, howell jolly bodies when asplenic
  2. HS: AD, spectrin def, tx with folate and splenectomy
  3. G6PD: xlinked, Heinz bodies, test several weeks after an episode
  4. PK def-AR and AD forms
44
Q

definition of neutropenia

A

ANC<500

Pediatric Board Prep (11 decks)

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