Hematology, Bilirubin

Question 1

DDx neonatal thrombocytopenia

early 72 hrs
lateafter 72 hours

Answer 1

Question 2

Bleeding disorders
- isolated high PT
- isolated high PTT
- both

Answer 2

Question 3

blood vol for exchange transfusion

Answer 3

Question 4

Hemolytic anemias

• pyruvate kinase deficiency
• glucose 6 phosphate deficiency

Answer 4

Question 5

Other ddx hemolytic anemia

Answer 5

Question 6

More ddx NB thrombocytopenia

Answer 6

Question 7

Physiologic mechanisms of neonatal jaundice

Answer 7

Question 8

FActors that influence efficacy of phototherapy

Answer 8

Question 9

Contributions to anemia of prematurity

Answer 9

Question 10

What has been associated with neuroblastoma

Answer 10

Question 11

presentation of acute bilirubin encephalopathy

Answer 11

Question 11

Describe hemorrhaghic disease of newborn

• time periods of presentation
• causes
• presentation
• mgmt

Answer 11

Question 12

stages of kernicterus

Answer 12

Question 13

Bilirubin metabolism

Answer 13

Question 14

Review thalassemias

Answer 14

Question 15

Review thalassemias

Answer 15

Question 16

Changes that lead to RBC storage lesions

Answer 16

Question 17

Continuing to rise BR - on ptx

Answer 17

Question 18

Answer 18

Question 19

HyperBR, phototherapy risk factors

Answer 19

Question 20

Review thalassemias
- clinical presentation

Answer 20

Question 20

chromosome - encodes alpha globin

chromosome - encodes beta globin

Answer 20

chromosome 16

chromosome 11

Question 21

most common worldwide hemoglobinopathy

Answer 21

Hgb E - decrease PRODUCTIONof beta chains - chronic, mild hemolytic anemia; esp SE asia

21glu ->lys

can still form tetramer with alpha 2 chains

Question 22

KB test calc

Answer 22

% Hgb F = # fetal cells / # maternal cells x 100 (assumes maternal cells 500 mL)

Ask to calculate fetal blood loss by KB test -> take % hgb fetus and x 5000

Rhogam vials = mL blood / 15

Question 23

Congenital erythrocyte underproduction

• Diamond Blackfan
• Fanconi Anemia

Answer 23

Diamond Blackfan - AD, AR
- pure RED CELL aplasia
- may have increased Hgb F
- Increased RBC adenosine deaminase levels
- MACROcytoci anemia at 2 months, low retic,BM no erythroid precursors + TRIPHALANGEAL THUMBS, abnormal facial, maybe cardiac, renal

Increase risk MDS, AML; chronic transfusions, steroid, BM tx possible

Fanconi anemia
- chromosomal INSTABILITY - lots of breaks
- hypoplastic BONE MARROW - anemia then pancytopenia
- MACROCYTOSIS, TRIPHALANGEAL thumbs, RADIAL hypoplasia, renal, ear malformations, strabismus

Test chromosome with mitomycin for BREAKS; Rx ANDROGENS, GF, BM tx

Question 24

RBC membrane defect

RBC biochemical defect

Answer 24

Her spherocytosis - AD, membrane instability, aplastic crisis, vaccinate ENCAPSULATED ORGANISMS, +osmotic fragility test

G6PD defn - #1enzyme deficiency, XLR -> cannot make NADPH so decrease levels, worse with oxidant STRESS; HEINZ bodies; FN if not in crisis

Pyruvate kinase deficiency - AR, not convert PEP to pyruvate so not generate ATP; chronic anemia;

Question 25

Question 26

Question 27

Question 28

most common solid tumor

second most common

Answer 28

Teratoma - 50% SCT, malignant potential <10% neonates

Neuroblastoma - adrenal, neural crest (horner HTN, flushing, dd, subcutaneous nodules), < 12 mo = better prognosis

Prognosis depends on stage, age, N-myc amplification; Stage IV S - < 1 yo better prognosis (although mets)

Ax NF, central hypoventilation, Hirschsprung

Question 29

Other tumors

• pheochromocytoma
• Wilms
• hepatoblastoma
• retinoblastoma – inheritance
• rhabdomyosarcoma

Answer 29

P - von hippel lindau, catecholamine
W - GU, hemihypertrophy ASS - mets - good prognosis - BW, Denys Drash, WAGR, pearlman
H - increased AFP, thrombocytosis
R - sporadic > AD; 45% passing on if bilateral, 6% unilateral; 2nd sibling if 1st bilateral - 3%

Question 30

FFP contains…

Cryo contains…

Answer 30

FFP - all factors + gamma globulins + albumin + protein factors

Cryo - VIII, vWF, fibrinogen, XIII, fibronectin

Question 31

Congenital leukemia

Histiocytosis

Answer 31

Langerhans - F,pancytopenia, multi organ infiltration

HLH - F, pancytopenia, low fibrinogen, high ferritin/high TG, HSM, adenopathy

Question 32

BR - increase production or increase circulating BR

Answer 32

Production usually 2/2 increased RBC breakdown

Increased enterohepatic circulation due to bowel obstruction, delayed enteral feeds, HD, mec ileus

OTHER - cong hypothyroidism, tyrosinemia, liver failure, sepsis

Question 33

Drugs that uncouple BR from albumin

Answer 33

ceftriaxone (although confusion in Q - uncouples but also decreases unconjugated BR?)

Question 34

Drugs where hyperbilirubinemia will influence its dose