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Neonatology Boards > Hematology > Flashcards

Hematology Flashcards

1
Q

Triad for renal vein thrombosis

A

Flank mass
Hematuria
Thrombocytopenia

Risk factors: Dehydration, hyperviscosity, asphyxia, hypercoagulable states, diabetes, indwelling catheters
Presentation: Hypotension then hypertension days/weeks later
Dx: US with Doppler

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2
Q

Most common intraerythrocytic pathogen transmitted by transfusion

A

Babesia!

intra-erythrocyte pathogen!
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3
Q

Fetal Hgb is composed of which types of globin chains?

A

Alpha and gamma

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4
Q

How do globin chains develop in utero and once baby is born?

A
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5
Q

% fetal hemoglobin at birth

A

80%

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6
Q

Hematopoeisis at gestational ages

A

Conception-6 week: 2nd yolk sac
6-22 weeks: fetal liver
after 22 weeks: bone marrow

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7
Q

Characteristics of anemia of prematurity

A

Normocytic, monochromic, normal MCV, decreased retic

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8
Q

Neonatal leukemia

A

RARE

Increased risk: Fanconi’s, Diamond-Blackfan, T21

decreased platelets, anemia, increased WBC > 100, erythroid/lymphoid blasts
palpable cutaneous nodules

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9
Q

stem cells originate from where?

A

mesoderm

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10
Q

RBC indices and how they change over development:

RBC number
Hematocrit
MCV
Reticulocytes
Nucleated RBC

A

increases
increases
decreases
peaks at 26-27 wks, then declines
decreases

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11
Q

alpha globin genes on which chromosome

A

16
total of 4 genes

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12
Q

beta globin genes on which chromosome?

A

11
total of 2 genes

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13
Q

alpha thalassemia

severity based on the # of genes expressed

chinese subcontinent, malaysia, indochina, africa

A

absence of 1 gene: normal

absence of 2 genes (alpha thal trait): normal to inc Hgb Barts
- mild microcytosis, hypochromia
- erythrocytosis
- may be asymptomatic

absence of 3 genes (Hgb H): infant 20-30% Barts, child 4-20% Hgb H;
- moderately severe hemolytic anemia –> Hgb H is unstable
- Heinz bodies, +/- splenectomy
- susceptible to same drugs that cause hemolysis in G6PD

none (Alpha thal major): 80-90% Barts, no Hgb A or F
- FETAL HYDROPS SYNDROME

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14
Q

Hgb Barts

A

4 gamma chain hemoglobin present in alpha thalassemia

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15
Q

beta thalassemia

not usually detected in 1st month- beta not needed for fetal Hgb

A

blood smear- mild microcytic, hypochromic anemia, target cells

mediterranean, africa, china, pakistan, india, middle east

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16
Q

types of beta thalassemia

A

beta thal trait: mild increased A2/F

beta thal disease:

Beta zero: severe anemia in 1st yr, splenomegaly, poor growth, thalassemia bony changes, chronic transfusions
- increased Hgb F (>90%)
- increased Hb A2
- no change or decreased Hgb A

Beta+ : 20-40% A, 60-80% F
- moderate anemia, may not require transfusions

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17
Q

most common hemoglobinopathy in the world

A

Hemoglobin E

abnormal transcription message in the beta-globin chain –> dec production of beta chains –> chronic mild microcytic anemia

southeast asia

nucleotide substitution: GLU –> LYS

if inherited with beta-thal, more severe anemia

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18
Q

FS hemoglobin on newborn screen

A

differential: sick cell anemia, sickle-B thal, sick with hereditary persistence of fetal hgb

start PCN ppx
hematology referral

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19
Q

FSC on newborn screen

A

sickle C disease

start penicilin
hematology referral

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20
Q

FSA on newborn screen

A

sickle B+ thalassemia

start PCN ppx
hematology referral

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21
Q

Kleihauer Betke

A

done on maternal blood–> detects fetal blood

blood treated with citric acid buffer –> adult Hgb soluble –> Hgb F remains and stained with eosin –> appear red on slide

mothers/Hgb A appears as “ghosts”

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22
Q

KB equation

A

% Hgb F = (# feta cells/maternal cells) x 100

1% Hgb F = 50ml fetal blood

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23
Q

Rhogam

A

ml fetal blood/15

1 vial = 300mcg

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24
Q

Diamond Blackfan

congenital hypoplastic anemia

A

congenital erythroid asplasia- pure red cell asplasia

macrocytic anemia with reticulocytopenia in first year
leukocyte/plts normal
Bone marrow = absence of erythroid precursors
increased serum erythropoietin
increased RBC adenosine deaminase levels
increased RBC Hgb F and I antigen

features:
- low birthweight
- short stature
- abnormal facies
- TRIPHALANGEAL THUMBS
- cardiac/renal systems

increased risk for aplastic anemia, myelodysplastic syndrome, acute leukemia

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25
Q

Fanconi anemia
constitutional aplastic anemia

AR

A

chromosomal instability with breaks –> test chromosomes with mitomycin C

TRIPHALANGEAL THUMBS
hyperpigmentation
microcephaly
mental deficiency
strabismus
malformed ears
renal anomalies
marrow hypoplasia–> pancytopenia
macrocytosis, incr Hgb F, i antigen

at risk for malignancy: AML, lymphoma

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26
Q

I:i antigen system

A

based on a gene on chromosome 6

I = cell membrane on RBCs in adults
i = cell membrane on RBCs in fetuses and newborns until 13 - 20 months then switches to I antigen

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27
Q

hereditary spherocytosis

A

AD, Northern European, North America

issue with RBC cell membrane: absent spectrin, ankyrin –> sphere shape of RBC instead of disk –> increased hemolysis –> splenic sequestration

classic triad: jaundice, anemia, splenomegaly

Neonate: jaundice with signs of hemolysis without Coombs +

positive osmotic fragility test, low MCV, high MCHC

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28
Q

microcytic anemia

A

thalassemia
iron deficiency
lead poisoning

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29
Q

macrocytic anemia

A

methylmalonic aciduria
folate/vitamin B 12 def
acquired aplastic anemia
Diamond-Blackfan anemia
Fanconi anemia
Hypothyroidism
Down Syndrome

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30
Q

Mentzer index = MCV/#RBC

A

greater than 13.5 = iron deficiency
less than 11.5 = thalassemia major

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31
Q

anemia of prematurity

A

normocytic, normochromic
decreased retic count

physiologic drop caused by low EPO output in response to anemia

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32
Q

physiologic hgb nadir

A

term = 10-12 at 8-10 wks

preterm:
7-8 if < 1.2 kg –> 5-6 wks
9 if > 1.2 kg –> 5-6 wks

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33
Q

erythropoietin source

A

Predominant = fetal liver
3rd trimester = kidneys

hepatic > renal at birth

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34
Q

pharmacokinetics of EPO in neonates

A

plasma EPO levels drop rapidly post exogenous administration
increased volume of distribution
increased metabolism or clearance
EPO increased by iron supplementation

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35
Q

direct coombs

A

performed on INFANT’s blood
antibodies noted DIRECTLY on infant’s RBCs

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36
Q

indirect coombs

A

performed on MOTHER’s blood
detects ANTIBODIES in SERUM

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37
Q

ABO incompatibility

A

mothers who are A/B –> produce IgM which DO NOT cross placenta

mother’s who are O –> produce IgG which CROSSES placenta –> isoimmune hemolytic anemia

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38
Q

alloimmune = isoimmune

A

immune response to non-self antigens

39
Q

G6PD

A

most frequently inherited enzyme defect –> XR, M>F

anemia, reticulocytosis, hyperbilirubinemia
decreased NADPH levels
exacerbation with exposure to oxidant stress

40
Q

pyruvate kinase deficiency

A

2nd MC inherited RBC enzyme defect
AR
Northern European ancestry, AMISH

labs: anemia, hyperbilirubinemia
normocytic, normochromic, abnormal RBC shapes, reticulocytosis

41
Q

blood volume term vs preterm

A

term = 80ml/kg
preterm = 100ml/kg

42
Q

methemoglobinemia

A

Hgb oxidized to ferric state (Fe3+)
decreased blood oxygen capacity and transport –> normal PaO2 but dec oxygen saturation
blood = brown when exposed to oxygen

TX: METHYLENE BLUE = incr activity of NADPH-MET Hgb reductase –> urine blue/green

43
Q

neonatal alloimmune thrombocytopenia
NAIT

A

production and placental transfer of MATERNAL alloantibodies against PATERNALLY inherited antigens on fetal platelets
MC = HPA 1a

first infant can be severely affected

INFANT plts low AT BIRTH, decline over first several days then increase over next 4 weeks, positive for HPA (as well as father)
MATERNAL plts normal, negative for HPA
intrauterine intracranial bleeding, death
bleeding diathesis /petechiae at birth
20% mortality

management: platelet antigen testing in parents, HUS, random donor plts, consider IVIG

44
Q

autoimmune

A

body can’t tell the difference between self and foreign

45
Q

neonatal autoimmune thrombocytopenia
ITP

A

MATERNAL antiplatelet antibodies caused to autoimmune disease

MOTHERS plts count LOW
INFANTs plts count LOW –> mildler than NAIT, lower risk of IVH

maternal plt count is not a good indicator of neonatal platelet count/severity

management: platelet tx, IVIG, steroids

46
Q

minor blood group antigens that can cause hemolytic disease

A

Kell = K and k
Duffy = Fya
Kidd = Jka, Jkb

47
Q

common Rh antigens that cuase hemolytic disease

A

D, c, C, e, E

48
Q

Thrombocytopenia with absent radii syndrome
TAR

A

AR
M:F = 1:2

severe thrombocytopenia with absent/reduced megakaryocytes –> blockage of early differentiation
leukemoid reactions, eosinophilia
bilateral absent radii and ulnar abnormalities
thumbs/digits formed - contast to Fanconi
30-35% = cardiac disease: TOF, ASD
facial capillary hemangioma

25% mortality, MC IVH

Management: platelet transfusiosn unti spontaneous resolution

49
Q

oxygen dissociation curve

A
50
Q

amegakaryocyte thrombocytopenia

A

BM absent or scarce megakaryocytes

M:F = 2:3

SEVERE ISOLATED thrombocytopenia

50% = aplastic anemia, risk of leukemia
HIGH MORTALITY

51
Q

clotting factor development

A

maternal clotting factors DO NOT cross placenta = endogenous production for infant

clotting proteins synthesized during 1st trimester
levels comparable to adult ~ 6 months

52
Q

clotting cascade

A
53
Q

PT = extrinsic pathway

(< 28 wks 14.5-20.9 sec)
(28-34 wks 13.9-20.6 sec)
~10-13 sec

A

inherited factor VII def
liver disease
DIC
vit K def
coumadin/warfarin
inherited/acquired factor V, X, II if also prolonged PTT

54
Q

PTT = intrinsic pathway

(< 28 wks 27-64 sec)
(28-34 wks 30-57 sec)

A

hemophilia A (VIII)
hemophilia B (IX)
VWf (if also prolonged bleeding time)
heparin
lupus anticoagulant
inherited/acquired factor V, X, II defect (if also prolonged PT)

55
Q

vitamin K dependent factors

A

II, VII, IX, X
protein C & S

56
Q

hemophilia A
factor VIII deficiency

A

XR, 70% of hemophilia

increased bleeding after blood draws, circ, hemarthrosis, IVH

prolonged PTT
give factor VIII

correlation between level of deficiency and sx

57
Q

hemophilia B
factor IX deficiency
Christmas disease

A

XR, 30% hemophilia

given factor IX

correlation between level of deficiency and sx

58
Q

hemophilia C
factor XI deficiency

A

AR, Ashkenazi jews

Noonan syndrome
increased risk of GU bleeding
give FFP
NO correlation b/w level of deficiency and sx

59
Q

factor XIII def

A

bleeding with normal coag studies

give cryo or factor XIII

60
Q

vonWillebrand disease

A

AD/R

component of factor VIII functioning as a ligand b/w plt and vessel

mucous membrane bleeding
abnormal bleeding time
+/- prolonged PTT, PT/plts NORMAL

Dx: ristocetin factor

Give CRYO, factor VIII with vWF, DDAVP

61
Q

hemorrhagic disease of the newborn

A

early - first 24 hours, maternal drugs
classic - 2-7 days, inadequate vit K
late - 2 wks to 6 mo, inadequate intake of hepatobiliary disease

PROLONGED PT, NORMAL PTT

GIVE IM VIT K- oral doesnt prevent this

62
Q

histiocytosis

A

infant < 2 yrs old
fever, pancytopenia, multiorgan infiltration

63
Q

familial hemophagocytic lymphohistiocytosis

fHLH

A

AR
abnormally hyper-activated T-cells and macrophages
fever, pancytopenia, HSM, adenopathy
FATAL

Dx: find hemophagocytosis in samples of bone marrow, spleen, lymph nodes

decreased NK cells, neutropenia

64
Q

virus-associated hemophagocytic lymphohistiocytosis

A

fever
pancytopenia
HSM

viruses: CMV, EBV, HSV, adenovirus

65
Q

MC solid tumor in the neonatal period

A

teratoma

2nd is neuroblastoma

66
Q

retinoblastoma

A

most frequent eye tumor

AD in 40%
unilateral (70%), bilateral (30%)
sxs: leukocoria, strabismus, decreased vision, secondary glaucoma

2nd malignancies: osteosarcoma, pinealoblastoma

67
Q

components of FFP

A

ALL clotting factors
fibronectin
gamma-globulins
albumin
plasma proteins

68
Q

components of cryo

A

VIII
vWF
fibrinogen
XIII
fibronectin

69
Q

bilirubin pathway

A
70
Q

phototherapy dose dependent on…

A

spectrum of light: blue wavelength 460-490
irradiance of light source: standard = 10, high intensity = > 30
maximize exposure of skin to light
distance of infant to light: optimal is 10-15 cm

71
Q

exchange transfusion

A

double volume:
- fresh IRRADIATED whole blood or reconstituted with FFP
- HCT = 50-55%
- 5-20ml aliquots
- replaced 87% of blood volume
- removes bilirubin and any free antibody
- monitor: HR, BP, pH, K, glucose, Ca, Mg

morbidities: hypocalcemia, thrombocytopenia, NEC, air embolus, thrombosis, infection, GVH

72
Q

abnormal activity of glucuronyl transferase (UGTP1A1)

A

absent = crigler-najjar
reduced = gilbert’s

73
Q

direct vs indirect bilirubin

A
74
Q

IVIG MOA

A

binds to Fc receptor on reticuloendothelial cells so that destruction of RBCs cannot occur

DOES NOT GET RID OF BILIRUBIN ALREADY IN THE SYSTEM –> PREVENTS CONTINUED ACCUMULATION

75
Q

Gilberts syndrome

A

impairment in conjugation process (dec UGT)
intermittent increases in INDIRECT/UNCONJUGATED bilirubin

76
Q

kasabach-meritt

A

thrombocytopenia

77
Q

blood volume exchange

A

[(pts hct - desired hct) / pts hct] x infant’s blood volume

78
Q

transient myeloproliferative disease
transient leukemia

A

5-10% T21
diagnosed in neonatal period
asymptomatic but may present with HSM, effusions, bleeding/petechiae

HALLMARK = leukocytosis with neutrophilia, thrombocytopenia, blasts on blood smear

self-limited- 60-70% resolve spontaneously by 2 months

20% of T21 develop myeloid leukemia

79
Q

areas of yellow deposition/bilirubin due to kernicterus

A

basal ganglia
cranial nerve nuclei
hippocampus

80
Q

how does 2,3 DPG change oxygen affinity

A

2,3 DPG is an organic phosphate normally found in the human erythrocyte

it REDUCES oxygen affinity by stabilizing deoxyhemoglobin

81
Q

Cooley anemia

A

severe beta-thalassemia

82
Q

Why do babies get anemia of prematurity?

A

low EPO production
insufficient placental transfer of iron- occurs mostly in 3rd trimester
short survival of fetal/neonatal RBCs
phlebotomy losses

83
Q

Hepcidin

A

regulates maternal-fetal iron metabolism

Mother: decreases iron absorption and placental transfer

Fetus: downregulate placental iron delivery once its needs are met

Levels decrease throughout pregnancy to allow for more iron absorption

84
Q

enzyme responsible for enterohepatic bilirubin circulation

A

beta-glucuronidase

deconjugates bilirubin –> reabsorbed by intestine

85
Q

enzyme breaks down biliverdin to unconjugated bilirubin in RES of spleen, liver, bone marrow

A

biliverdin reductase

86
Q

irradiation

A

prevents GVH disease

WATCH for hyperkalemia

87
Q

complications of hemangiomas

A
  1. hypothyroidism: increased type 3 iodothyronine deiodinase which inactivates thyroid hormones
  2. high output heart failure: if involves liver

Think Kasabach-Meritt

88
Q

leukocyte reduced

A

prevents CMV

89
Q

DCC decreases?

A

need for transfusion

risk for IVH and NEC

90
Q

bilirubin/albumin ratio can tell you what?

A

marker for amount of unbound bilirubin

exchange:
> 6.8 - preterm with risk factors
> 8 - term with risk factors

91
Q

PTT measures all factors in pathway except for?

A

factor VII

92
Q

serum ferritin is a gauge for what?

A

total body iron stores

93
Q

what cell line can be elevated in BPD?

A

Eosinophils

94
Q

exchange transfusion volume

A

blood volume = wt x est blood volume/kg (80 or 100)

Neonatology Boards (19 decks)

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