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NICU Board Review > Heme > Flashcards

Heme Flashcards

1
Q

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

A

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+)

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2
Q

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

A

ITP

Think autoimmune.

Milder neonatal thrombocytopenia

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

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3
Q

Neonates have _____ clotting time as compared to adults

A

Shorter clotting time

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4
Q

Male with prolonged PTT and bleeding. Dx?

A

Hemophilia A, Deficiency factor VIII

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5
Q

Bleeding in a noonan infant. Which dx?

A

AR, Factor XI

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6
Q

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

A

Factor XIII

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7
Q

Reason for anemia of prematurity

A

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

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8
Q

What is the role of hepcidin in iron storage?

A

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.

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9
Q

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

A

Random donor plts

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10
Q

Enzyme responsible for enterohepatic bilirubin circulation

A

Β glucuronidase

  • deconconjugates bilirubin which allows for its réabsorption in the intestine
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11
Q

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

A

Biliverdin reductase

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12
Q

Thrombocytopenia is inversely correlated with gestational age.
T or F

A

True

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13
Q

Irradiation of prbcs is done to prevent _______?

A

Graft vs host disease

Side effect: hyperkalemia

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14
Q

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

A

Resistance to acid elution

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15
Q

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

A

7.3ml/kg/min

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16
Q

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

A

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

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17
Q

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

T or F

A

TRUE

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18
Q

Stem cells originate from ______

A

Mesoderm

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19
Q

Where does early activé hematopoiesis occur?

A

Secondary yolk sac
(2-6 weeks)

Déclines after 8 weeks

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20
Q

When does fetal liver hematopoiesis begin?

When does the liver become the primary site for hematopoiesis?

A

Begins: 5-6 weeks gestation

Primary site: 6-22 weeks’ gestation

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21
Q

Which red blood cell indices INCREASE with increasing gestational age?

A

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

22
Q

Which red blood cell induces DECREASE with increasing gestational age?

A

MCV

Nucleated RBC

23
Q

Complications of hemangiomas

A

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

24
Q

Describe the triad in Kasaback-Merritt Syndrome

A

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

25
Q

What does leukocyte reduced RBC blood prevent?

Irradiated RBC?

A

CMV

Graft vs host reaction

26
Q

Delayed cord clamping decreases _______

A

Need for transfusion

Risk for IVH and NEC

27
Q

What percent of iron stores are accrued in 3rd trimester

A

80%

Iron content in term baby 75mg/kg body weight

28
Q

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

A

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

29
Q

2d 32 weeks Neonate with PLT 100k

A

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

30
Q

Whst is the differential for late onset thrombocytopenia

A

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

31
Q

What is the role of hepcidin in fetal iron transfer?

A

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

32
Q

Which conditions are associated with low iron stores?

A

Fetal overgrowth
Male sex
Preterm birth
Multiple gestation

33
Q

Low fetal iron stores occur with what maternal conditions ?

A 
Placental insufficiency 
Diabetes
Severe stressors
Obesity 
Chronic illness

*increased maternal hepcidin levels —> decreased placental iron transfer (endowment)

34
Q

What is the MOA and concerning side effects of tin mesoporphrin

A

Photosensitization induced cytoxicity

35
Q

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

A

Chromium mesoporphyrin

zinc bis glycol

36
Q

TRansient myeloproliferative disorder affects which cell lines?

A

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

37
Q

What metabolic derrangement is associated with polycythemia?

A

Hypoglycemia

38
Q

What are the features of hepatoblastoma?

A 
Abdominal mass - MC in RIGHT lobe 
Thrombocytosis 
Elevated AFP (>90%) - low levels can indicate poor prognosis
39
Q

How to interpret KB test?

A

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

40
Q

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

A

Fanconi Anemia

Test chromosomes with mitomycin C with increased chromosomal breaks

Can predispose to AML

41
Q

What is the bilirubin:albumin molar ratio?

A

Measure of bili binding capacity of albumin

Surrogate to estimate free bili

Increases with increasing GA

<0.8 in healty term neonates

42
Q

How to test for G6PD?

A

Spectrophotometric quantitative enzyme assay based on NADPH (nicotinsmide adenine dinucleotide phosphate)

Enzyme active <30% of normal is confirmatory

43
Q

Whai is neonatal auto immune thrombocytopenia?

A

Mother + baby with decreased platelet

Caused by maternal auto antibodies (MC in Mommy’s with ITP or lupus)

44
Q

MC tumor of the kidney

A

Congenital mesoblastic nephroma

45
Q

What explains low plasma concentration of epo

In preemies,

A

Increased metabolic clearance of epo

46
Q

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

A

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

47
Q

5 or more cutaneous hemangiomanas. Next best step?

A

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

48
Q

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

A

> 6.8 if preterm w risk factors

> 8 if term w risk factors

49
Q

What affects albumin binding ability to albumin ?

A 
Sepsis 
Acidosis 
Hypoxia 
Free fatty acid 
Albumin binding drugs (ceftriaxone, sulfonamides, indomethacin)
50
Q

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

T or F

A

True

As part of multi factorial analysis

51
Q

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

A

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

NICU Board Review (19 decks)

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