Brainscape's Knowledge GenomeTM

Browse over 1 million classes created by top students, professors, publishers, and experts.


See full index

OS 216 - Hematology Exam > Congenital Hemolytic Anemia (Eufrosina A. Melendres, MD) > Flashcards

Congenital Hemolytic Anemia (Eufrosina A. Melendres, MD) Flashcards

1
Q

Enumerate the three common congenital hemolytic anemias.

A

Hereditary Spherocytosis
Thalassemias
G6PD Deficiency

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

Congenital Hemolytic Anemia is an inborn defect in one of what three main components?

A

RBC Membrane
Hemoglobin
Enzymes

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

Number one cause of anemia in children below 6

A

Congenital Hemolytic Anemia

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

T/F: Extracorpuscular factors are usually inherited, while intracorpuscular factors are usually hereditary.

A

False

It’s the other way around.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

Treatment for hereditary spherocytosis and elliptocytosis

A

Splenectomy

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

Examples of disorders with RBC membrane defects

A

Hereditary Spherocytosis

Heredtiary Elliptocytosis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

Examples of disorders with hemoglobin defects

A

Thalassemias

Hemoglobinopathies

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

Examples of disorders with enzyme defects

A

G6PD Deficiency

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

Most commonly detected hereditary intracorpuscular defect in newborn screening

A

G6PD Deficiency

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

Examples of acquired disorders involving intracorpuscular defects

A

Acquired Autoimmune Hemolytic Anemia
Lead Poisoning
Paroxysmal Nocturnal Hemoglobinemia

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

Characteristics of Acquired Autoimmune Hemolytic Anemia

A

Splenomegaly
IgG-mediated
Responsive to corticosteroids
Blood transfusion is difficult

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

Paroxysmal Nocturnal Hemoglobinemia predisposes a person to what other hematologic disorder?

A

Aplastic Anemia

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

Clinical triad for hemolytic anemia

A

Pallor or anemia
Jaundice
Splenomegaly (except in newborn and G6PD deficient)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

Increase in this component in the serum and urine reflects ongoing hemolytic process

A

Bilirubin

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

Positive Coomb’s test favors diagnosis of this disease

A

Autoimmune Hemolytic Anemia

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

Leftward shift in fragility curve favors diagnosis of this disease

A

Hereditary Spherocytosis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
17
Q

Supplement given for Congenital Hemolytic Anemia

A

Folic acid (NOT IRON)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
18
Q

T/F: Packed RBCs should only be transfused for patients with symptomatic or pathologic anemia.

A

True

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
19
Q

T/F: You can still perform etiologic tests after blood transfusion.

A

False

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
20
Q

Recommended age range for splenectomy

A

5 – 9 (earlier if with severe hypersplenism)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
21
Q

Consideration to make for splenectomy

A

All lobes should be removed

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
22
Q

RBC morphology in Congenital Hemolytic Anemia

A

Hypochromic
Microcytic
Anisocytosis (size)
Poikilocytosis (shape)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
23
Q

Red cell fragments present in peripheral blood smear of Congenital Hemolytic Anemia

A

Schistocytes

Helmet cells

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
24
Q

Describe the extent of central pallor of a normal RBC

A

1/3 of the diameter

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
25
Q

T/F: There is increased reticulocytosis and polychromasia in Congenital Hemolytic Anemia

A

True

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
26
Q

Iron-deficiency Anemia produces these RBC morphologies

A

Teardrop cells

Pencil cells

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
27
Q

RBC morphology in Thalassemia

A

Hypochromic
Microcytic
Target cell (3 humps)
Fragmented into different shapes

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
28
Q

Differentiate IDA and Thalassemia peripheral blood smears

A

More reticulocytes in Thalassemia blood smear

29
Q

Normal MCV

A

80 – 100 fL

30
Q

Normal MCH

A

27 – 31 pg

31
Q

Normal MCHC

A

320 – 360 g/L

32
Q

Normal RDW

A

11 – 16%

33
Q

Describe the red cell indices for hemolytic anemia

A

MCV decreased
MCH decreased
MCHC normal to increased
RDW increased

34
Q

Good tool to diagnose IDA

A

Nutritional history

35
Q

Hereditary Spherocytosis is due to defects in these components

A

Spectrin

Ankyrin

36
Q

Mode of inheritance of hereditary spherocytosis

A

75% autosomal dominant

25% autosomal recessive

37
Q

T/F: Hereditary spherocytosis predisposes an individual to infection

A

True

38
Q

Process of RBC membrane loss in Hereditary Spherocytosis

A

Vesiculation

39
Q

T/F: There is presence of pigmented gallstomes in Hereditary Spherocytosis

A

True

40
Q

Describe the red cell indices for Hereditary Spherocytosis

A

MCV normal to slightly decreased
MCH decreased
MCHC increased
RDW increased

41
Q

Describe RBC morphology in Hereditary Spherocytosis

A

Abnormally small and lack central pallor

42
Q

Test used to diagnose Hereditary Spherocytosis

A

Osmotic Fragility Test

43
Q

Spherocytes are sequestered in this organ in Hereditary Spherocytosis

A

Spleen

44
Q

Types of Thalassemia

A

Alpha Thalassemia

Beta Thalassemia

45
Q

T/F: Thalassemia is a qualitative defect.

A

False

It is quantitative. Hemoglobinopathies are qualitative.

46
Q

Type of Thalassemia that behaves like Thalassemia Major

A

HbE-Beta Thalassemia

47
Q

Describe dyserythropoiesis in Thalassemia

A

Increased production of RBCs that are defective (ineffective erythropoiesis)

48
Q

New expanded newborn screening process includes this, which allows early detection of Thalassemia

A

Detection of HbH

49
Q

Describe the beta hemoglobin chain in Beta Thalassemia

A

Defective via mechanism other than deletion

50
Q

Type of Alpha Thalassemia where three alleles are lost and displaying the clinical triad

A

Hb H disease (beta 4)

51
Q

Type of Alpha Thalassemia that is incompatible with life

A

Hydrops Fetalis

52
Q

Type of Beta Thalassemia with stormy clinical course and involving multiple transfusions

A

Thalassemia Major or Cooley’s Anemia

53
Q

Mode of inheritance of Thalassemia Major

A

Autosomal Recessive

54
Q

Shift from fetal Hb to Hb A occurs highest at what age?

A

6 months

55
Q

Craniofacial changes associated with Thalassemia Major

A

Frontal bossing
Maxillary overgrowth
Micrognathia

56
Q

T/F: There is growth stunting in Thalassemia Major patients.

A

True

57
Q

On top of the clinical triad, this is also present in Thalassemia Major

A

Hepatomegaly

58
Q

Test to diagnose Thalassemia Major

A

Hb Electrophoresis

59
Q

Increase in these types of Hb in Thalassemia Major

A

HbF

HbA2

60
Q

Describe hair-on-end appearance on skull X-ray in Thalassemia Major

A

Spikes running perpendicular to surfaces

61
Q

Presentation of Thalassemia Major in the Osmotic Fragility Test

A

Rightward shift (resistant to hemolysis)

62
Q

When is iron chelation indicated for Thalassemia Major?

A

Children aged 5 and above to increase Fe excretion

63
Q

Gold standard of oral chelators

A

Desferrioxamine

64
Q

Types of bone marrow transplantation

A

Autologous

Allogeneic

65
Q

Mode of inheritance of G6PD Deficiency

A

X-linked

66
Q

Most common type of G6PD Deficiency

A

G6PD A-

67
Q

Dots attached to RBC membrane that reduce cell flexibility and increase susceptibility to destruction by splenic macrophages

A

Heinz bodies

68
Q

Exposure of infant to this chemical merits consideration of G6PD Deficiency

A

Naphthalene (moth balls)

OS 216 - Hematology Exam (8 decks)

Brainscape helps you reach your goals faster, through stronger study habits.
© 2024 Bold Learning Solutions. Terms and Conditions