Pediactric Bullshit

Question 1

5 day old, rash, mother from central america, UTI pregnancy complication, erythematous rash at birth on face, periorbital swelling, to trunk and diaper area, hepatosplenomegaly, WBC elevated, 52% blast forms, elevated platelets, LDH high

Answer 1

DDx - syphilis, chlamydia, herpes, neuroblastoma, letterer siwe, MPD

Question 2

erythema toxicum neonatorum

Answer 2

rash on face

not too much worse

Question 3

neutrophil stages

Answer 3

promyelocyte
myelocyte
metamyelocyte
band
segmented PMN

Question 4

herpes infection

Answer 4

can cause lots of problems so test for this in babies

Question 5

tzanck test

Answer 5

for herpes

Question 6

lymphocytosis in infant

Answer 6

viral - EBV - mirror cells

-or upper resp infection - cough, whoops - pertussis

Question 7

letterer-siwe disease

Answer 7

langerhans cell histiocytosis

accumulation of dendritic cells

no known cause

peak age - 2 yo

skin rash lesions - vesicular

Question 8

leukamoid rxn

Answer 8

> 50,000 leukocytes in blood

must distinguish from CML

Question 9

basophilic leukocytosis

Answer 9

MPD - chronic myeloid leukemia

Question 10

leukemic hiatus

Answer 10

majority of blasts
-not progressing to mature cells as they should

also elevated LDH - suggest rapid proliferation - neoplastic disorder

Question 11

thrombocytosis in newborn

Answer 11

acute inflammation
blood loss
nutrition conditions
asplenia
MPD
neuroblastoma
mother substance abuse

Question 12

transient myelproliferative disorder

Answer 12

trisomy 21 - possible mosaicism

only distinguishing factor - resolves by 12 weeks of age**

clinically identical to acute myeloid or megakaryocytic leukemia

MUST be followed by pediatric oncology physician

Question 13

rash of true infant leukemia

Answer 13

nodular

Question 14

FISH probe for chromosome 21q22

Answer 14

trisomy 21

Question 15

3yo boy pallor, bruising, intermittent fever 3 weeks, low WBC, low platelets, BM shows myeloblasts, pancytopenia

Answer 15

ALL or AML

Question 16

aplastic anemia BM

Answer 16

nothing there

Question 17

13yo F malaise fever 1 week, bilateral nontender cervical nodes, splenomegaly, EBV titers high, WBC 135,000, low platelets, LDH high, uric acid high, 95% WBC blasts, mediastinal widening, BM lymphoblasts - T cells (CD2/7)

Answer 17

presumptive mono dx
-not mono when see high WBC blasts and marrow

high risk ALL - rapid onset - high tumor burden - WBC > 50,000

Question 18

5yo F, bilateral leg and wrist pain, fever 103 three weeks, MCV 77, ESR 35 (sed rate), dx rheumatoid arthritis, but gets worse, elevated LDH, BM pre-B lymphocytes

Answer 18

bone pain - cytokine from lymphoblasts and marrow expansion

Question 19

ALL

Answer 19

most common pediatric cancer - 25%

peak 2-5yo males

Question 20

AML

Answer 20

4% of all childhood cancers

-predisposition with chemo and radiation - in older children

Question 21

CML

Answer 21

can happen in kids, rare

myeloproliferative disorder

Question 22

syndromes with acute leukemias

Answer 22

down syndrome
fanconi
klinefelter
shwachman diamond

Question 23

WBC > 200,000

Answer 23

microemboli in brain or lungs in children
stroke or PE

more commonly in AML

Question 24

resp distress, cough, facial edema

Answer 24

suggest SVC syncrome

-mediastinal compression in ALL

Question 25

life threatening bleeding

Answer 25

secondary to DIC in AML

Question 26

evan syndrome

Answer 26

anemic and low platelets

Question 27

DDx of leukemia malignancies

Answer 27

neuroblastoma retinoblastoma rhabdomyosarcoma

Question 28

confirm Dx of leukemoa

Answer 28

BM studies flow cytometry cytogenics

Question 29

CSF

Answer 29

must rule out CNS leukemia need to be done under optimal conditions to prevent traumatic tap that introduces leukemic cells to CNS

Question 30

80-90% ALL

Answer 30

cured in children

Question 31

50% AML

Answer 31

cured in children

Question 32

lower risk fx

Answer 32

age 1-9 and low to normal WBC at diagnosis

Question 33

high risk fx

Answer 33

age > 10yo, infants with high WBC and those with mature B cell leukemia

Question 34

hyperdiploidy

Answer 34

better prognosis

Question 35

t9:22

Answer 35

higher risk

Question 36

ALL therapy

Answer 36

aggressive first 6-12 months CNS prophylactics - intrathecal chemotherapy therapy extends 2-3 years

Question 37

AML therapy

Answer 37

intensive chem for about 1 year bone marrow transplant if possible

Question 38

risk with Tx

Answer 38

greatest risk - infection manage fever critical live virus vaccines - CONTRAINDICATED**

Question 39

avascular necrosis of joints

Answer 39

with corticosteroids

Question 40

tumor lysis

Answer 40

``` hyperuricemia hyperkalemia renal failure fatal arrhythmia P/Ca metabolism messed up ``` to tx this - diuretics and lots of fluids**

Question 41

pallor, jaundice, splenomegaly

Answer 41

hemolytic process

Question 42

cold Abs

Answer 42

IgM - require complement

Question 43

warm Abs

Answer 43

IgG - no complement

Question 44

differentiate AHA from other causes of anemia

Answer 44

direct coombs

Question 45

tx of autoimmune hemolytic anemias

Answer 45

corticosteroids increase in Hg within few days of therapy with prednisone RBC transfusions - if anemia serious

Question 46

donath landsteiner Ab

Answer 46

cause of hemolysis uncommon IgG cold hemolysin after viral infection and syphilis

Question 47

tx of sickle cell

Answer 47

hydroxyurea - enhances production of HgF

Question 48

ABE

Answer 48

acute bilirubin encephalopathy

Question 49

BIND

Answer 49

bilirubin induced neuro dysfunction kernicterus

Question 50

blueberry muffin baby

Answer 50

purpura as result of EMH