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Pediatrics Board Exam > Heme/Onc > Flashcards

Heme/Onc Flashcards

0
Q

14 yo boy with painful swelling of mid-femur. Multiple layers of periosteal bone formation.

A

Ewing Sarcoma

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1
Q

16 yo with painless firm mass at proximal tibia. What radiology findings? And when is this the most common secondary malignancy?

A

Osteogenic Sarcoma. XR findings are sunburst pattern and codman triangle. Most common secondary malignancy in retinoblastoma.

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2
Q

Li Fraumeni syndrome has increased incidence of what cancer

A

Osteogenic sarcoma

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3
Q

12 yo boy with unilateral thigh pain at night. Relieved by NSAIDs. X-ray findings? Tx?

A

Osteoid osteoma. XR findings are central radiolucent area surrounded by sclerotic bone. Tx is supportive care

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4
Q

6 yo girl with cranial nerve palsies, ataxia, head tilt

A

Brainstem glioma

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5
Q

Poor prognostic signs for LCH

A

Age < 2, male gender, organ involvement

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6
Q

Most frequent secondary malignancy in Hodgkin survivors

A

AML

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7
Q

ALL poor prognostic signs

A

Age 10, WBC >50,000, steroid tx, hypodiploidy, MLL rearrangement t(4;11)

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8
Q

What cancer to think about in a 16 yo otherwise healthy male with isolated splenomegaly. Treatment?

A

CML; txt is BMT

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9
Q

Auer rods, leukoerythroblastic reaction (nRBC). Is there testicular involvement?

A

AML; No testicular involvement

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10
Q

Philadelphia chromosome t(9;22). BCR-abl gene. Associated with what cancer? Tx?

A

CML; tx with imatinib (tyrosine kinase inhibitor)

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11
Q

Teen with non-tender cervical or supraclavicular node. What is it and what do you see on bx

A

Hodgkin lymphoma; Reed-Sternberg cells

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12
Q

6 yo boy with RLQ mass, pain, and LAD below the diaphragm. Negative px indicators?

A

Burkitt’s lymphoma; neg px: CNS involvement, BM involvement, high LDH > 1000

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13
Q

Cancer associated with intussusception

A

Burkitt’s lymphoma

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14
Q

T(8;14) c-myc oncogene

A

Burkitt’s lymphoma

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15
Q

Serum markers for Hodgkin’s disease

A

ESR and serum copper

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16
Q

Most common tumor in infants? Px factors?

A

Neuroblastoma, px good for age <18 mos, stage localized, histopathology, hyperdiploidy

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17
Q

2 yo black girl with painless abdominal mass and hematuria. Prognostic factor that is most important?

A

Wilm’s tumor; histology (bad=clear cells! a aplastic cells)

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18
Q

Child with constipation and rectal mass

A

Rhabdomyosarcoma

19
Q

What factors shift the oxygen dissociation curve to the right (higher affinity)?

A

2,3 DPG, acidosis, hypoxia, fever

20
Q

Buchanans rule to determine Hgb

A

11+ (age in yrs x 0.1)

21
Q

Buchanans rule to determine MCV

A

70 + (age in years)

22
Q

Hair on end appearance of skull X-ray

A

Beta thalassemia; indicates extra medullary hematopoiesis

23
Q

8 yo girl with repeated episodes of pallor, jaundice, and hemolytic anemia that is hyperchromic with high MCHC

A

Hereditary spherocytosis

24
Q

2 yo boy with recent viral illness with pallor, normochromic normocytic anemia with low retic count, normal WBC and platelets

A

Transient erythroblastopenia of childhood

25
Q

School age child with macrocytic anemia, mild to moderate thrombocytopenia, leukopenia, and upper limb abnormalities, short stature. How do you diagnose?

A

Fanconi’s anemia, dx with chromosome breakage study

26
Q

Infant with normochromic, macrocytic anemia, reticulocytopenia, triphalangeal thumbs, short stature. How do you dx? How to treat?

A

Diamond blackfan anemia. Dx with elevated RBC ADA. Tx with steroids

27
Q

5 mo old with recurrent skin infections, hypo pigmentation of skin and hair. Inheritance? Dx?

A

Chediak-Higashi. Autosomal recessive. Giant neutrophil granules on smear

28
Q

Infant with recurrent severe infections, omphalitis, and neutropenia

A

Kostmanns Syndrome (Severe congenital neutropenia)

29
Q

Impaired wound healing or delayed separation of umbilical cord, with high WBC in absence of infection. Tx?

A

Leukocyte adhesion deficiency. Tx BMT

30
Q

Most common pathogen in cyclic neutropenia? Inheritance?

A

C. Perfringens. Autosomal dominant

31
Q

Pancreatic insufficiency, short stature, recurrent infections and neutropenia. Also with clinodactyly or syndactyly

A

Shwachman-Diamond Syndrome

32
Q

Baby boy with bad diaper rash, bruising, frequent ear and lung infections. Small platelets. Inheritance? Tx?

A

Wiskott-Aldrich. X-linked ( only in boys). Tx is BMT

33
Q

Infant with massive GI bleeds and family history of similar episodes. What do the PT, PTT, and bleeding time look like? Tx?

A

Glanzmann’s thrombasthenia which is AR. PT, PTT are normal. Bleeding time prolonged. Tx with platelet transfusions.

34
Q

Infant with bleeding, normal coags, prolonged bleeding time, and giant platelets

A

Bernard-Soulier

35
Q

Newborn with petechiae, wet purpura, and platelet count of 1000. Mom with normal platelets. Tx?

A

Neonatal alloimmune ITP. Tx with maternal platelets

36
Q

ITP look a like with HSM

A

Think leukemia!

37
Q

Mucosal bleeding with positive family history, normal coags and prolonged bleeding time. Inheritance? Tx?

A

Von Willebrand disease. Autosomal dominant. Tx severe bleeding with factor 8. If unavailable, cryoprecipitate. Mild bleeding with DDAVP

38
Q

What coags are abnormal in hemophilias?

A

PTT is prolonged

39
Q

Most common complication in hemophilia?

A

Chronic hemarthroses leading to joint destruction

40
Q

Massive DIC at birth with family history of clotting. Tx?

A

Protein C or S deficiency causing neonatal purpura fulminans. Tx FFP, heparin while starting warfarin

41
Q

Recurrent severe VTE, heparin fails to anticoagulate.

A

Antithrombin III Deficiency. Tx warfarin lifelong

42
Q

Late onset thromboses, most common cause

A

Factor V Leiden

43
Q

Most common form of sickle cell disease?

A

FS

44
Q

Sickle cell trait

A

FAS

45
Q

When to start PCN ppx in sickle cell disease

A

2 months

Pediatrics Board Exam (23 decks)

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