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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
Fanconi anemia constitutional aplastic anemia ## Footnote AR
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
26
I:i antigen system
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
27
hereditary spherocytosis
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
28
microcytic anemia
thalassemia iron deficiency lead poisoning
29
macrocytic anemia
methylmalonic aciduria folate/vitamin B 12 def acquired aplastic anemia Diamond-Blackfan anemia Fanconi anemia Hypothyroidism Down Syndrome
30
Mentzer index = MCV/#RBC
greater than 13.5 = iron deficiency less than 11.5 = thalassemia major
31
anemia of prematurity
normocytic, normochromic decreased retic count physiologic drop caused by low EPO output in response to anemia
32
physiologic hgb nadir
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
33
erythropoietin source
Predominant = fetal liver 3rd trimester = kidneys hepatic > renal at birth
34
pharmacokinetics of EPO in neonates
plasma EPO levels drop rapidly post exogenous administration increased volume of distribution increased metabolism or clearance EPO increased by iron supplementation
35
direct coombs
performed on INFANT's blood antibodies noted DIRECTLY on infant's RBCs
36
indirect coombs
performed on MOTHER's blood detects ANTIBODIES in SERUM
37
ABO incompatibility
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
38
alloimmune = isoimmune
immune response to non-self antigens
39
G6PD
most frequently inherited enzyme defect --> XR, M>F anemia, reticulocytosis, hyperbilirubinemia **decreased NADPH levels** exacerbation with exposure to oxidant stress
40
pyruvate kinase deficiency
2nd MC inherited RBC enzyme defect AR Northern European ancestry, AMISH labs: anemia, hyperbilirubinemia normocytic, normochromic, abnormal RBC shapes, reticulocytosis
41
blood volume term vs preterm
term = 80ml/kg preterm = 100ml/kg
42
methemoglobinemia
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
neonatal alloimmune thrombocytopenia NAIT
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
autoimmune
body can't tell the difference between self and foreign
45
neonatal autoimmune thrombocytopenia ITP
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
minor blood group antigens that can cause hemolytic disease
Kell = K and k Duffy = Fya Kidd = Jka, Jkb
47
common Rh antigens that cuase hemolytic disease
D, c, C, e, E
48
Thrombocytopenia with absent radii syndrome TAR
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
oxygen dissociation curve
50
amegakaryocyte thrombocytopenia
BM absent or scarce megakaryocytes M:F = 2:3 SEVERE ISOLATED thrombocytopenia 50% = aplastic anemia, risk of leukemia HIGH MORTALITY
51
clotting factor development
maternal clotting factors DO NOT cross placenta = endogenous production for infant clotting proteins synthesized during 1st trimester levels comparable to adult ~ 6 months
52
clotting cascade
53
PT = extrinsic pathway (< 28 wks 14.5-20.9 sec) (28-34 wks 13.9-20.6 sec) ~10-13 sec
inherited factor VII def liver disease DIC vit K def coumadin/warfarin inherited/acquired factor V, X, II if also prolonged PTT
54
PTT = intrinsic pathway (< 28 wks 27-64 sec) (28-34 wks 30-57 sec)
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
vitamin K dependent factors
II, VII, IX, X protein C & S
56
hemophilia A factor VIII deficiency
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
hemophilia B factor IX deficiency Christmas disease
XR, 30% hemophilia given factor IX *correlation between level of deficiency and sx*
58
hemophilia C factor XI deficiency
AR, Ashkenazi jews **Noonan syndrome** increased risk of GU bleeding give FFP *NO correlation b/w level of deficiency and sx*
59
factor XIII def
bleeding with normal coag studies give cryo or factor XIII
60
vonWillebrand disease
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
hemorrhagic disease of the newborn
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
histiocytosis
infant < 2 yrs old fever, pancytopenia, multiorgan infiltration
63
familial hemophagocytic lymphohistiocytosis fHLH
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
virus-associated hemophagocytic lymphohistiocytosis
fever pancytopenia HSM viruses: CMV, EBV, HSV, adenovirus
65
MC solid tumor in the neonatal period
teratoma 2nd is neuroblastoma
66
retinoblastoma
most frequent eye tumor AD in 40% unilateral (70%), bilateral (30%) sxs: leukocoria, strabismus, decreased vision, secondary glaucoma 2nd malignancies: osteosarcoma, pinealoblastoma
67
components of FFP
ALL clotting factors fibronectin gamma-globulins albumin plasma proteins
68
components of cryo
VIII vWF fibrinogen XIII fibronectin
69
bilirubin pathway
70
phototherapy dose dependent on...
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
exchange transfusion
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
abnormal activity of glucuronyl transferase (UGTP1A1)
absent = crigler-najjar reduced = gilbert's
73
direct vs indirect bilirubin
74
IVIG MOA
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
Gilberts syndrome
impairment in conjugation process (dec UGT) intermittent increases in INDIRECT/UNCONJUGATED bilirubin
76
kasabach-meritt
thrombocytopenia
77
blood volume exchange
[(pts hct - desired hct) / pts hct] x infant's blood volume
78
transient myeloproliferative disease transient leukemia
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
areas of yellow deposition/bilirubin due to kernicterus
basal ganglia cranial nerve nuclei hippocampus
80
how does 2,3 DPG change oxygen affinity
2,3 DPG is an organic phosphate normally found in the human erythrocyte it REDUCES oxygen affinity by stabilizing deoxyhemoglobin
81
Cooley anemia
severe beta-thalassemia
82
Why do babies get anemia of prematurity?
low EPO production insufficient placental transfer of iron- occurs mostly in 3rd trimester short survival of fetal/neonatal RBCs phlebotomy losses
83
Hepcidin
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
enzyme responsible for enterohepatic bilirubin circulation
beta-glucuronidase deconjugates bilirubin --> reabsorbed by intestine
85
enzyme breaks down biliverdin to unconjugated bilirubin in RES of spleen, liver, bone marrow
biliverdin reductase
86
irradiation
prevents GVH disease WATCH for hyperkalemia
87
complications of hemangiomas
1. hypothyroidism: increased type 3 iodothyronine deiodinase which inactivates thyroid hormones 2. high output heart failure: if involves liver Think Kasabach-Meritt
88
leukocyte reduced
prevents CMV
89
DCC decreases?
need for transfusion risk for IVH and NEC
90
bilirubin/albumin ratio can tell you what?
marker for amount of unbound bilirubin exchange: > 6.8 - preterm with risk factors > 8 - term with risk factors
91
PTT measures all factors in pathway except for?
factor VII
92
serum ferritin is a gauge for what?
total body iron stores
93
what cell line can be elevated in BPD?
Eosinophils
94
exchange transfusion volume
## Footnote blood volume = wt x est blood volume/kg (80 or 100)

Neonatology Boards (19 decks)

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