heme/onc

Question 1

What is the early active site of hematopoiesis?

Answer 1

the secondary yolk sac, blodd cells appear as early has 16-19 days; the secondary yolk sac begins to regress by week 10

Question 2

When does hematopoiesis begin in the fetal liver?

Answer 2

week 5-6 and becomes the primary site between 6-22 weeks

Question 3

When does the bone marrow in the developing long bones start hemotpoiesis and when does it become the primary site?

Answer 3

starts 8-19 week but becomes primary after 22 weeks

Question 4

a mother is Rh c- and her fetus is Rh +c., what is the likelihood of having an infant affected with significant jaundice and anemia?

Answer 4

20-30%

Question 5

An O+ mother has a B+ neonate. In addition to anemia, what else is this infant at risk for?

Answer 5

thrombocytopenia, there is aB antigen on the platelets

Question 6

What is the most common solid tumor in the neonatal period?

Answer 6

teratoma, with 50% sacrococcoygeal

Question 7

What is the second most common tumor in the neonatal period?

Answer 7

neuroblastoma with 70-% in the adrenal gland

Question 8

When do you give FFP and what are the components of it?

Answer 8

bleeding, DIC, Vit K def, Factor IX def, Factor XI def: FFP has all clotting factors, fibronectin, gamma-globulins, albumin, and plasma proteins

Question 9

when do you give cryoprecipitate and what are the components of it?

Answer 9

Factor VIII def, von Willbrande disease and Factor XIII def; it contains, VIII, vWF, fibrinogen, XIII, fibronectin

Question 10

What syndrome is a/w hemophilia C?

Answer 10

Noonan’s ; give FFP since it is Factor XI def

Question 11

What are causes of purpura fulminans?

Answer 11

DIC (GBS and Neissier men), congenital absence of protein C or Protein S; homozygous or heterozygous factor V Leiden mutations

Question 12

What is the most frequently inheritied RBC enzyme defect?

Answer 12

G6PD; x-linked recessive

Question 13

When does early HDN present?

Answer 13

within 24 hours, due to maternal medications

Question 14

When does classic HDN present?

Answer 14

2-7 dyas., due to inadequate vit K. Clinically has GI bleeding, ICH and oozing

Question 15

When does late HDN present?

Answer 15

2 weeks- 6 months; due to inadequate vit K intake or hepatobiliary disease; clincally, are at increased risk for ICH and death, more common in boys

Question 16

When does UDPGT1 reach adult levels?

Answer 16

3 months of age

Question 17

What Is the most prominent hemoglobinopathy in the world?

Answer 17

Hemoglobin E; resuilts from a gene mutation causing decreased production of beta chains; range of clnical symptoms (mild to severe anemia)

Question 18

infant with T21 that has WBC count 55,000 (high % neutrophils) with blasts in the peripheral smear, thrombocytopenia, . Whats the diagnosis and how does it usually present?

Answer 18

transient myoproliferative disease- offurs in 50% of infants with T21, affected individuals are usually asympotmatic but may present with hepatosplenomegal, pleural effusions, bleeding, or petechiae

Question 19

Wha is the first line management of a neonate with NAIT and plt 20,000?

Answer 19

random donor platelet infusion

Question 20

Whst factors limite the efficacy of phototherapy?

Answer 20

etiology of the indirect hyperbili (phototherapy is less effective if jaundice is due to hemolysis or if cholestasis is present; light source, distnace, SA exposed, TSB concentration (higher levrels will hvve a more rapid decline in TSB)

Question 21

What disorders are a/w an increased of congenital leukemia

Answer 21

Bloom’s syndrome, Diamond Blackfan syndrome, Fanconi anemia, T21,

Question 22

hypopigmented and hyperpigmented skin lesions photosensitive, malar rash, mild craniofacial dysmorphisms, high pitched voice. Name the syndrome

Answer 22

Bloom’s syndrome

Question 23

Syndrom characterized by short stature, bone marrow failure with cytopenia, radial and other anomalies.

Answer 23

Fanconi anemia

Question 24

Why are platelets low in TAR sydrome?

Answer 24

due to a blockage in differentiation of an early megakaryocyte precursor resulting in decreased platelet production. Thombopoietin level and thrrombopoietin receptor levels are normal

Question 25

Wilms tumor is the most common renal malignancy in children under 15 years old 2/3 cases are diagnosed at <5years. Name the syndrome that are a/w Wilms tumor

Answer 25

Beckwith-Wiedeman, Denys-Drash syndrome, Pearlman syndrome, WAGR syndrome

Question 26

Name the complications a/w double volume exchange?

Answer 26

electrolyte disturbances (hypocalcemia, hypomag, hypoglycemia, hyperkalemia,)NEC, thrombosis, infxn, thrombocytopenia, GVHD, apnea

Question 27

What is hepcidin?

Answer 27

It is the predominant regulator of iron uptake and storage in both the fetus and newborn infant. Hepcidin and Fe stores are in direct proportion to each other. Hepcidin regulates iron absorption and release. NR Jan 1

Question 28

Name two factors that increase the risk and volume of fetal-maternal transplacental hemorrhage?

Answer 28

c-section and manual removal of the placenta. If this happens when there is Rh incompatibility, need to screen the mom for fetal-maternal transplacental hemorrhage with Kleihauer-Betke test

Question 29

What are the most common causes of significant anemia at birth?

Answer 29

blood loss or alloimmune hemolysis

Question 30

At what time frame do internal hemorraghes and other cuases of hemorrhages manifest?

Answer 30

after 24 hours

Question 31

When do abnormalities in the synthesis of hemoglobin beta chains, hypoplastic RBC disorders and physiologic anemia of infancy or prematurity manifest>

Answer 31

several weeks after birth

Question 32

Does the indirect coombs test tell you quantititative or qualitative info?

Answer 32

It tells you the presence of alloimmunization and also the degree; A critical titer is a/w a risk of fetal hydrops

Question 33

Labs show: decreased MCV, decreased Ferritin, increased TIBC and increaed transferrin receptor. Whats the diagnosis?

Answer 33

Iron deficiency anemnia. TIBC reflects the amount of unbound trasferrin and is elevated in iron deficiency. Synthesis of soluble transferrin receptor is increased when intracellular iron is insufficient. Low serum ferrtiin is specific for iron deficiency

Question 34

What phycial exma findings characterize stage 1 of acute bilirubin encephalopathy?

Answer 34

infant with decreased tone, poor suck, decreased activity and a slightly high pitched cry. Damage reversible

Question 35

What physical exam findings characterizes stage 2 of acute bilirubin encephalopathy?

Answer 35

stage 1 + rigid extension of all 4 extremities, tight fisted posturing of the arms, crossed extension of the legs, and a high-pitched irritable cry. May have seizure activity, retrocollis amd opisthotonos. Damage likely reversible.

Question 36

What phycial exam findings characterize stage 3 of acute bilirubin encephalopathy?

Answer 36

hypertonia with marked retrocollis and opisthotonos, stupor, coma, and a shrill cry. Damage not reversible.

Question 37

What physical finding in an infant with indirect hyperbili shows that some damage to the CNS has occurred?

Answer 37

opisthotonos

Question 38

What is the recommended level for exchange transfusion in infants < 28 weeks?

Answer 38

11 to 14, recs from AAP