Heme/Onc Flashcards by Erica Unknown

Primary site of hematopoiesis in fetus (in order)

Secondary yolk sac (until ~10 weeks)
Liver (6-22 weeks)
Bone marrow (after 22 weeks)

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Predominant cell type in fetal liver

Mature macrophage

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HgF made of which global chains

Alpha 2 gamma 2

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HgA made of which global chains

Alpha 2 Beta 2

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Which thalassemia detected at birth

Alpha thalassemia 
(HgF has alpha chains but no beta chains so don't notice beta thal until ~6 months when HgA predominant)

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Absence of 1 alpha gene

Silent carrier

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Absence of 2 alpha genes

Alpha thal trait = mild microcytosis, hypochromia, may be asymptomatic

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Absence of 3 alpha genes

Hb H

moderately severe hemolytic anemia, Heinz bodies

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Absence of 4 alpha genes

Hemoglobin barts

hydrops fetalis

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Frontal bossing, maxillary hypertrophy, severe anemia

Beta thalassemia disease (homozygous) or Cooley anemia

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Most common hemoglobinopathy in the world

Hemoglobin E

Decreased production of beta chains, resulting in chronic, mild, microcytic anemia

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FA vs FAS vs FSA

FA normal
FAS sickle cell trait
FSA sickle B+ thalassemia

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Sickle cell anemia, sickle beta 0 thalassemia, sickle with hereditary persistence of hemoglobin

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Kleihauer-Betke vs Apt test

KB on maternal blood, detects fetal blood

Apt test on fetal sample, detects maternal blood

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Triphalangeal thumbs + macrocytic anemia

Diamond Blackfan and Fanconi anemia

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Radial hypoplasia

Fanconi anemia and TAR

in TAR, thumbs and digits are developed normally

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Hypersegmented neutrophils

Vitamin B12 and folate deficiency

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Most frequently inherited enzyme defect

G6PD deficiency

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G6PD catalyzes which process?

G6P to pentose phosphate and makes NADPH

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Pyruvate Kinase catalyzes which process?

PEP to pyruvate and makes ATP

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Normal paO2 but decreased oxygen saturation

Methemoglobinemia

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Maternal platelet count in NAIT vs Neonatal autoimmune thrombocytopenia

Normal vs low

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Bleeding with prolonged PTT

Hemophilia A or B

A in 70% cases

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Bleeding with normal coagulation studies

Factor XIII (Autosomal recessive)

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Bleeding with abnormal BT +/- prolonged PTT

vWF disease (AR or AD)

Bleeding with prolonged PT and normal PTT

Vitamin K deficiency | if extended time, PTT will also be prolonged

MC solid tumor in the neonatal period

Teratoma

What do ceftriaxone, sulfonamides and indomethacin do to bilirubin?

Bind albumin and displace bilirubin, increasing indirect hyperbilirubinemia

Hemangiomas + thrombocytopenia

Kasabach Merritt syndrome (Coagulation activated locally within the hemangioma and platelets are sequestered) Only 50% infants have visible hemangioma

chromosomal fragility and breakage

Fanconi's anemia

First line treatment for NAIT

random donor platelets IF ineffective, HPA-1a negative donor IVIG and steroids may help reduce immune mediated destruction

Early onset hemorrhagic disease of the newborn

Within 24 hours Placental transferred drugs that inhibit vit K ie anticonvulsants, cephalosporins, warfarin, barbiturates, rifampin, isoniazid

Classic hemorrhagic disease of the newborn

2-7 days

Late onset hemorrhagic disease of the newborn

2 weeks to 6 months MC in boys, summer Poor enteral intake of Vitamin K or liver disease

Leukocytosis with neutrophilic, thrombocytopenia and blasts

Transient myeloproliferative disease | 5-10% infants with T21

Typical symptoms of infants with Transient myeloproliferative disease

Typically asymptomatic | can have HSM, effusions, bleeding or petechiae

Prognosis of Transient myeloproliferative disease

60-70% cases resolve spontaneously by 2 months | 20% affected infants will develop AML

NAIT vs Rh disease, which can occur in 1st pregnancy?

NAIT | Recurrence rate >75% in subsequent pregnancies

Where is EPO made in the fetus?

Fetal liver (primarily) and kidney

When does most Fe transfer to the fetus occur?

After 30 weeks (accrued at 1.6-2 mg/kg/d)

Hypopigmented and hyperpigmented skin lesions, photosensitive malar rash, mild craniofacial dysmorphisms, high pitched voice

Bloom's syndrome

Size and amount of megakaryocytes in adult vs fetus

Fetus - smaller but greater number circulating (still only 0.03-0.1% of nucleated cells in the BM)

<12 months + neuroblastoma = favorable or unfavorable prognosis?

Favorable

MC renal malignancy of children

Wilm's tumor

Wilm's tumor associated with which syndromes

BW syndrome Denys drash (progressive renal disease, male pseudohermaphroditism) Pearlman (fetal gigantism, visceromegaly, abnormal facies, b/l renal hamartomas) WAGR (Wilm's, aniridia, genitorurinary, mental retardation)

Heme oxygenase catalyzes which step

Heme to biliverdin

Biliverdin reductase catalyzes which step

Biliverdin to bilirubin

Can placenta remove indirect bilirubin? Biliverdin?

Bilirubin but not biliverdin

MC site of bleeding in hemorrhagic disease of the newborn

GI tract

Why is irradiated blood used?

Prevents GVHD

How to decrease risk of CMV in blood?

Leucodepleted

What shifts oxyhemoglobin curve to right and does that increase or decrease affinity?

Inc temperature, acidosis (dec pH), altitude, 2,3-DPG, Bohr effect Inc offloading ie decreases affinity

Rh blood system consists of

C, c, D, E, e, G antigens

Albumin binding to bilirubin decreased by

Sepsis, acidosis, hypoxia, free fatty acids, albumin-binding drugs ie. Ceftriaxone albumin = 2.5 is a risk factor

Irreversible sign of kernicterus

Opisthonus

Most severe of minor antigens

Kell

Nitrites, aniline dyes, prilocaine, nitrous oxide

Acquired methoemloglobinemia

Congenital methemoglobinemia tx

do NOT respond to methylene blue

Family history of parent with intermittent cyanosis

Congenital methemoglobinemia (AD)

Pure red cell aplasia

Diamond Blackfan Anemia (macrocytic)

Total blood volume to be exchanged in polycythemia

([hematocrit observed - desired) x blood volume] / hematocrit observed

Bleeding disorder ass with Noonan

Hemophilia C

Purpura fulminans

Protein C or S deficiency

Term infant with IVH, look for?

Cerebral sinovenous thromboses --> get MRI

When use TPA

limited to life or limb threatening thromboses

Which criggler najjar responds to phenobarbital?

Type II

What to do when infant showing advanced stages of acute bilirubin encephalopathy

EXCHANGE- DVET (80-100 mL/kg/day x 2)

Firm subcutaneous nodules bluish gray

leukemia cutis --> neonatal leukemia