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Neo Board Review MAP > Hematology > Flashcards

Hematology Flashcards

1
Q

1 g of hemoglobin holds how much oxygen?

A

1.34 mL

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2
Q

Primary sites of hematopoiesis in utero

A

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

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3
Q

Alpha-2 beta-2 Hemoglobin

A

Adult hemoglobin

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4
Q

Alpha-2 gamma-2 Hemoglobin

A

Fetal hemoglobin

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5
Q

Fetal hemoglobin

A

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

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6
Q

Erythropoietin

A

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

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7
Q

Why are neonates less responsive to heparin?

A

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

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8
Q

Genetic disorder associated with ribosomal 60 S subunit dysfunction

A

Shwachman-Diamond syndrome

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9
Q

Most common Hematologic abnormality in Schwachman diamond

A

Cyclical neutropenia

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10
Q

Alloimmune hemolysis and maternal RBC antigens

A

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

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11
Q

Father intermittently cyanotic with cyanotic newborn

A

NADH methemoglobin reductase deficiency

Respond to methylene blue

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12
Q

How much iron is needed for each gram of hemoglobin?

A

3.47 mg

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13
Q

Why do we use irradiated blood transfusions?

A

Prevent graft versus host disease

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14
Q

Why do we use leukocyte-reduced blood for transfusions?

A

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

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15
Q

Why do we used washed RBCs for blood transfusions?

A

Decreased risk for hyperkalemia

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16
Q

Favorable features for neuroblastoma

A

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

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17
Q

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

A

G6 PD

18
Q

Causes of Purpura fulminans

A

DIC
Protein C or protein S deficiency
Factor V Leiden mutation

19
Q

Absolute contraindications for anticoagulation

A

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

20
Q

Relative contraindications to anticoagulation

A 
Platelet < 50
Fibrinogen <100
INR >2
Severe coagulation deficiency
Hypertension
21
Q

How much bilirubin does 1 g of albumin bind?

A

7-8 mg

22
Q

Which form of Crigler Najjar syndrome responds to phenobarbital treatment?

A

Type 2

23
Q

Which parts of the brain are affected by kernicterus?

A 
Basal ganglia
Hippocampus
Subthalamic nuclei
Globus pallidus
Cerebellum
24
Q

How does phototherapy decrease indirect bilirubin?

A 
Geometric photoisomerization (4Z/15Z -> 4E/15Z, 4Z/15E, 4E/15E)
Intramolecular cyclization (lumirubin formation, most effective)
Bilirubin oxidation
25
Q

What affects the effectiveness of phototherapy?

A

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

26
Q

Normal blood smear looks like…

A 
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
27
Q

Siderocyte

A

RBC containing granules of iron that are not part of hemoglobin

28
Q

Howell-jolly bodies

A

Inclusions of nuclear chromatin

29
Q

Elliptocytes

A

Elliptical shape

30
Q

Spherocytes

A

Decreased central pallor

31
Q

What does an eosinophil look like on a blood smear?

A

Reddish colored, dramatic granules

32
Q

What does a monocyte look like on a blood smear?

A

Large cell with one large nucleus inside

33
Q

What does a basophil look like on a blood smear?

A

Purple granules

34
Q

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

A

Many nuclei strung together

35
Q

Pelger-Huet anomaly

A

Leukocytes with dumbbell shaped bilobed nuclei

36
Q

Increased risk of Wilms tumor

A

Beckwith Weidemann syndrome
Denys-Drash syndrome
Pearlman syndrome
WAGR syndrome

37
Q

When is cyanosis observed?

A

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

38
Q

Vitamin K dependent clotting factors

A

Disco 1972

10, 9, 7, 2, protein c, protein s

39
Q

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

A

Myelodysplastic syndromes and leukemia

40
Q

Skeletal abnormalities in Shwachman-Diamond syndrome?

A

Metaphyseal dystosis
Thoracic dystrophies
Low turnover osteopenia

41
Q

Symptoms of Shwachman-Diamond syndrome

A

Bone marrow failure
Pancreatic dysfunction
Recurrent infections
Skeletal abnormalities

42
Q

Symptoms of hemophagocytic lymphohistiocytosis

A 
Fever
Hepatosplenomegaly
Jaundice
Lymphadenopathy
Elevated ferritin
Elevated triglycerides
Pancytopenia

Neo Board Review MAP (18 decks)

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