Heme

Question 1

Baby with severe thrombocytopenia with normal maternal platelets. Next step?

Answer 1

Get HUS, Plt transfusion (ideally HPS-1B) and IVIG

Neonatal Alloimmune Thrombocytopenia
Production and transfer of maternal alloantibodies against paternally inherited antigens on fetal platelets
Intracranial hemorrhage in 10 to 15% of cases
› Normal coagulation testing
› Most common cause: Incompatibility in HPA-1 (PLA-1)
• Mother HPA-1b (PLA-1)
• Father HPA-1a (PLA1+)
• Fetus HPA-1a (PLA1+)

Question 2

Baby and mother with thrombocytopenia. Which is the most common disease in mother?

Answer 2

ITP

Think autoimmune.

Milder neonatal thrombocytopenia

Tx IVIG
Only give platelets random donor if bleeding (avoid if possible)

Question 3

Neonates have _____ clotting time as compared to adults

Answer 3

Shorter clotting time

Question 4

Male with prolonged PTT and bleeding. Dx?

Answer 4

Hemophilia A, Deficiency factor VIII

Question 5

Bleeding in a noonan infant. Which dx?

Answer 5

AR, Factor XI

Question 6

Bleeding in a neonate, umbilical stump. Clotting studies are normal. Dx?

Answer 6

Factor XIII

Question 7

Reason for anemia of prematurity

Answer 7

Low epo production
Insufficient placental transfer of iron
Short survival of fetal and neonatal RBC
Phlebotomy losses

Question 8

What is the role of hepcidin in iron storage?

Answer 8

High hepcidin blocks release of iron from enterocytes into the circulation.

Hepcidin expression is decreased in response to iron deficiency or increased erythropoeisis leading to increased iron absorption in interstine and increase circulating iron.

Question 9

Which type of platelet transfusion should be ordered for pt with suspected NAIT?

Answer 9

Random donor plts

Question 10

Enzyme responsible for enterohepatic bilirubin circulation

Answer 10

Β glucuronidase

• deconconjugates bilirubin which allows for its réabsorption in the intestine

Question 11

What enzyme catabolizes biliverdin to unconjugated bilirubin in the RES of spleen, liver and bone marrow?

Answer 11

Biliverdin reductase

Question 12

Thrombocytopenia is inversely correlated with gestational age.
T or F

Answer 12

True

Question 13

Irradiation of prbcs is done to prevent _______?

Answer 13

Graft vs host disease

Side effect: hyperkalemia

Question 14

How does hbg F differ from maternal hgb in KB test?

Answer 14

Resistance to acid elution

Question 15

What is the critical threshold for oxygen delivery in adults for which we develop tissue hypoxia?

Answer 15

7.3ml/kg/min

Question 16

What is the clinical presentation of TMD (Transient Myeloproliferative Disorder)

Answer 16

10% of T21 patients
Hepatomegaly - MC presenting sign (50%)
Asymptomatic —->fulminant with hydrops —-> respiratory or ♥️ failure
Rash on face

Question 17

Fetal megakaryocytes are smaller than adult megakaryocytes, but there are a larger number circulating in fetuses compared with adults

T or F

Answer 17

TRUE

Question 18

Stem cells originate from ______

Answer 18

Mesoderm

Question 19

Where does early activé hematopoiesis occur?

Answer 19

Secondary yolk sac
(2-6 weeks)

Déclines after 8 weeks

Question 20

When does fetal liver hematopoiesis begin?

When does the liver become the primary site for hematopoiesis?

Answer 20

Begins: 5-6 weeks gestation

Primary site: 6-22 weeks’ gestation

Question 21

Which red blood cell indices INCREASE with increasing gestational age?

Answer 21

RBC Number
Hématocrit
Reticulocytes (peaks at 26-27 weeks) then declines

Question 22

Which red blood cell induces DECREASE with increasing gestational age?

Answer 22

MCV

Nucleated RBC

Question 23

Complications of hemangiomas

Answer 23

Hypothyroidism- if involves thyroid (2/2 ⬆️ in type 3 iodothyronine deiodinase) or liver
High output ❤️ failure - if involves liver

Question 24

Describe the triad in Kasaback-Merritt Syndrome

Answer 24

High output heart failure, DIC and thrombocytopenia in a pt that has multiple or single large hemangioma-like lesions

Question 25

What does leukocyte reduced RBC blood prevent? Irradiated RBC?

Answer 25

CMV Graft vs host reaction

Question 26

Delayed cord clamping decreases _______

Answer 26

Need for transfusion | Risk for IVH and NEC

Question 27

What percent of iron stores are accrued in 3rd trimester

Answer 27

80% Iron content in term baby 75mg/kg body weight

Question 28

How can a neonate have a normal G6PD enzyme levels yet be deficient later in life?

Answer 28

Significant Hemolysis leads to increased reticulocytes that have normal enzyme levels.

Question 29

2d 32 weeks Neonate with PLT 100k

Answer 29

Monitor and repeat Min risk for bleeding plt count 20k-100k Mod risk for bleeding plt count <20k Severe risk for plt count <5k Mild thrombocytopenia 100-150k Mod 50-100k Severe <50k

Question 30

Whst is the differential for late onset thrombocytopenia

Answer 30

Sepsis (bacterial, viral, fungal) NEC IEM (propionoc acidemia, methylmalonic acidemia, Gauchers)

Question 31

What is the role of hepcidin in fetal iron transfer?

Answer 31

Hepcidin is a key regulator of maternal - fetal iron metabolism Maternal hepcidin exerts negative effect on iron absorption and placental transfer Fetal hepcidin can downregulate placental iron delivery once its needs are met Hepcidin levels will decrease throughout pregnancy to allow more iron absorption

Question 32

Which conditions are associated with low iron stores?

Answer 32

Fetal overgrowth Male sex Preterm birth Multiple gestation

Question 33

Low fetal iron stores occur with what maternal conditions ?

Answer 33

``` Placental insufficiency Diabetes Severe stressors Obesity Chronic illness ``` *increased maternal hepcidin levels —> decreased placental iron transfer (endowment)

Question 34

What is the MOA and concerning side effects of tin mesoporphrin

Answer 34

Photosensitization induced cytoxicity

Question 35

Which metalloporphrin can be orally administered for treatment of severe hemolytic disease?

Answer 35

Chromium mesoporphyrin | zinc bis glycol

Question 36

TRansient myeloproliferative disorder affects which cell lines?

Answer 36

Disease of megakaryocytes precursors, mutagenesis of immature myeloblast or megakaryoblast lineages

Question 37

What metabolic derrangement is associated with polycythemia?

Answer 37

Hypoglycemia

Question 38

What are the features of hepatoblastoma?

Answer 38

``` Abdominal mass - MC in RIGHT lobe Thrombocytosis Elevated AFP (>90%) - low levels can indicate poor prognosis ```

Question 39

How to interpret KB test?

Answer 39

1%HbF=50ml %HbF=#fetal cells/maternal cells x100 Remember vial of rhogam needed is determined by mL of fetal blood loss/15 where 1 vial=300mcg

Question 40

Baby with macrocytic anemia, reticulocytopenia, increased RBC adenosine deaminase, increased RBC Hb F and I antigen, triphalangeal thumb, hypoplastic radi. Dx?

Answer 40

Fanconi Anemia Test chromosomes with mitomycin C with increased chromosomal breaks Can predispose to AML

Question 41

What is the bilirubin:albumin molar ratio?

Answer 41

Measure of bili binding capacity of albumin Surrogate to estimate free bili Increases with increasing GA <0.8 in healty term neonates

Question 42

How to test for G6PD?

Answer 42

Spectrophotometric quantitative enzyme assay based on NADPH (nicotinsmide adenine dinucleotide phosphate) Enzyme active <30% of normal is confirmatory

Question 43

Whai is neonatal auto immune thrombocytopenia?

Answer 43

Mother + baby with decreased platelet Caused by maternal auto antibodies (MC in Mommy’s with ITP or lupus)

Question 44

MC tumor of the kidney

Answer 44

Congenital mesoblastic nephroma

Question 45

What explains low plasma concentration of epo | In preemies,

Answer 45

Increased metabolic clearance of epo

Question 46

Ehat is the cause of hemolysis and anemia in an jnfant with NEC?

Answer 46

T antigen activation Cause bt neuramidase in bacteria such as clostridia and Streptococcus pneumonia or from a virus In a non activated state N acetyl neuraminic acid is attached to galactose on T antigen on surface of RBC. When neuraminidase cleaves NeuAc exposing galactose residues l, T antigen is activated and naturally occuring anti-T antibodies in humans attach to activated T antigen resulting in hemolysis and anemia. It also cause platelet destruction and thrombocytopenia Washed packed RBCs are indicated if T antigen activation is confirmed

Question 47

5 or more cutaneous hemangiomanas. Next best step?

Answer 47

Hepatic US for hepatic hemangioma As AV shunting of blood through large hepatic hemangioma can lead to CHF Rarely seen is a consumptive form of hypothyroidism with HH. Excessive production of type 3 iodothyronine deiodinasr leading to inactivation of thyroid hormones

Question 48

Serum bilirubin/albumin ratio in which exchange should be performed?

Answer 48

>6.8 if preterm w risk factors >8 if term w risk factors

Question 49

What affects albumin binding ability to albumin ?

Answer 49

``` Sepsis Acidosis Hypoxia Free fatty acid Albumin binding drugs (ceftriaxone, sulfonamides, indomethacin) ```

Question 50

The ratio of B/A (bilirubin to albumin) can be used as a surrogate for unbound bilirubin T or F

Answer 50

True | As part of multi factorial analysis

Question 51

What are the 3 types of hemorrhagic disease of the newborn ?

Answer 51

Classic (DOL 2-7) Bleeding from umbilical stump or after invasive procedure Vit K stores < Vit K intake *Exclusively breastfed at ⬆️ risk Early (within 24 h) Placentally transferred drugs that inhibit Vit K production (anticonvulsants, cephalosporin, warfarin) ``` Late Onset (2w - 6mo) ⬆️ boys, in summer Poor enteral intake Vit K or liver disease ```