Hematology

Question 1

1 g of hemoglobin holds how much oxygen?

Answer 1

1.34 mL

Question 2

Primary sites of hematopoiesis in utero

Answer 2

Yolk sac <6 wks
Liver 6-22 wks
Bone marrow >22 wks

Question 3

Alpha-2 beta-2 Hemoglobin

Answer 3

Adult hemoglobin

Question 4

Alpha-2 gamma-2 Hemoglobin

Answer 4

Fetal hemoglobin

Question 5

Fetal hemoglobin

Answer 5

Alpha-2 Gamma-2
Favors transfer of oxygen from maternal to fetal blood
60-80% of hemoglobin in newborn
Disorders affecting betaglobin do not affect neonates

Question 6

Erythropoietin

Answer 6

Produced in liver in fetus and newborn
Primary hormone regulating erythropoiesis
Transitions to kidney

Question 7

Why are neonates less responsive to heparin?

Answer 7

Inadequate antithrombin III
Increased clearance
Increased binding of heparin to plasma proteins
Increase concentration of fibrinogen

Question 8

Genetic disorder associated with ribosomal 60 S subunit dysfunction

Answer 8

Shwachman-Diamond syndrome

Question 9

Most common Hematologic abnormality in Schwachman diamond

Answer 9

Cyclical neutropenia

Question 10

Alloimmune hemolysis and maternal RBC antigens

Answer 10

Most -> least affected/severe
c > E > C
Kell > Daffy > Kidd > MNS

Question 11

Father intermittently cyanotic with cyanotic newborn

Answer 11

NADH methemoglobin reductase deficiency

Respond to methylene blue

Question 12

How much iron is needed for each gram of hemoglobin?

Answer 12

3.47 mg

Question 13

Why do we use irradiated blood transfusions?

Answer 13

Prevent graft versus host disease

Question 14

Why do we use leukocyte-reduced blood for transfusions?

Answer 14

Decreased febrile non-hemolytic transfusion reactions
Decreased CMV transmission
Decreased HLA alloimmunization

Question 15

Why do we used washed RBCs for blood transfusions?

Answer 15

Decreased risk for hyperkalemia

Question 16

Favorable features for neuroblastoma

Answer 16

Prenatal diagnosis
Liver/skin/bone metastases
DNA hyperploidy
No MYCN amplification

Question 17

What RBC enzyme defect can give a false positive NBS result for galactosemia?

Answer 17

G6 PD

Question 18

Causes of Purpura fulminans

Answer 18

DIC
Protein C or protein S deficiency
Factor V Leiden mutation

Question 19

Absolute contraindications for anticoagulation

Answer 19

CNS surgery or birth ischemia within 10 days
Active bleeding
Invasive procedures within three days
Seizures within 48 hours

Question 20

Relative contraindications to anticoagulation

Answer 20

Platelet < 50
Fibrinogen <100
INR >2
Severe coagulation deficiency
Hypertension

Question 21

How much bilirubin does 1 g of albumin bind?

Answer 21

7-8 mg

Question 22

Which form of Crigler Najjar syndrome responds to phenobarbital treatment?

Answer 22

Type 2

Question 23

Which parts of the brain are affected by kernicterus?

Answer 23

Basal ganglia
Hippocampus
Subthalamic nuclei
Globus pallidus
Cerebellum

Question 24

How does phototherapy decrease indirect bilirubin?

Answer 24

Geometric photoisomerization (4Z/15Z -> 4E/15Z, 4Z/15E, 4E/15E)
Intramolecular cyclization (lumirubin formation, most effective)
Bilirubin oxidation

Question 25

What affects the effectiveness of phototherapy?

Answer 25

Wavelength (Blue/green, 425-475 nm) Irradiance (Energy output, 30W/cm2/nm) Body surface area exposed Distance from light to baby

Question 26

Normal blood smear looks like…

Answer 26

``` Zone of pallor 1/3 size of RBC Minimal variation in size Visible platelets Normal segmented neutrophils RBC is 2/3 size of normal lymphocyte ```

Question 27

Siderocyte

Answer 27

RBC containing granules of iron that are not part of hemoglobin

Question 28

Howell-jolly bodies

Answer 28

Inclusions of nuclear chromatin

Question 29

Elliptocytes

Answer 29

Elliptical shape

Question 30

Spherocytes

Answer 30

Decreased central pallor

Question 31

What does an eosinophil look like on a blood smear?

Answer 31

Reddish colored, dramatic granules

Question 32

What does a monocyte look like on a blood smear?

Answer 32

Large cell with one large nucleus inside

Question 33

What does a basophil look like on a blood smear?

Answer 33

Purple granules

Question 34

What does a hypersegmented PMN look like on a blood smear?

Answer 34

Many nuclei strung together

Question 35

Pelger-Huet anomaly

Answer 35

Leukocytes with dumbbell shaped bilobed nuclei

Question 36

Increased risk of Wilms tumor

Answer 36

Beckwith Weidemann syndrome Denys-Drash syndrome Pearlman syndrome WAGR syndrome

Question 37

When is cyanosis observed?

Answer 37

>3-5g of reduced Hgb/ dL capillary blood present

Question 38

Vitamin K dependent clotting factors

Answer 38

Disco 1972 | 10, 9, 7, 2, protein c, protein s

Question 39

Patience with Shwachman-Diamond Syndrome are at increased risk of?

Answer 39

Myelodysplastic syndromes and leukemia

Question 40

Skeletal abnormalities in Shwachman-Diamond syndrome?

Answer 40

Metaphyseal dystosis Thoracic dystrophies Low turnover osteopenia

Question 41

Symptoms of Shwachman-Diamond syndrome

Answer 41

Bone marrow failure Pancreatic dysfunction Recurrent infections Skeletal abnormalities

Question 42

Symptoms of hemophagocytic lymphohistiocytosis

Answer 42

``` Fever Hepatosplenomegaly Jaundice Lymphadenopathy Elevated ferritin Elevated triglycerides Pancytopenia ```