Brainscape's Knowledge GenomeTM

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


See full index

Hematology > Anemia 3 > Flashcards

Anemia 3 Flashcards

1
Q

ANEMIAS OF ABNORMAL GLOBIN DEVELOPMENT

A

THE THALASSEMIAS

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

are a diverse group of genetic disorders characterized by a primary, QUANTITATIVE reduction in globin chain synthesis for hemoglobin.

A

THE THALASSEMIAS

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

The thalassemias are usually classified according to the particular globin chain whose synthesis is suppressed. The types are:

A 
a-thalassemia
b -thalassemia
g -thalassemia
d-thalassemia
db-thalassemia
gdb-thalassemia
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

The genetics of thalassemia is centralized to the structures of CHROMOSOME _ and _

A

11

16

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

differentiates thalassemia from Hereditary Persistence of Fetal Hemoglobin (HPFH)

A

Acid elution testfor Hb F:

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

HEMATOLOGY
Microcytic hypochromic anemia
WBC and platelets are not involved

SPECIAL HEMATOLOGY TESTS
Bone marrow: Hyperplastic red cell precursors
Hemoglobin electrophoresis on cellulose acetate at alkaline pH
Citrate agar electrophoresis (acid pH)

A

THALASSEMIAS

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

Most important test to differentiate Iron deficiency from thalassemia

A

Ferritin:

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

a-thalassemia
a DOUBLE gene deletion (- - )
a SINGLE gene deletion (- a)

A

a thalassemia-1 or 1° thalassemia

a thalassemia-2

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

Caused by a deletion of all 4 a globin chains (- -/- -)

A

BART’S HYDROPS FETALIS

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

Deletion of 3 or 4 a globin genes (- -/- a)

Non deletional forms exists (aaT/aaT and aaT/- -)

A

HbH DISEASE

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

CS consists of 2 normal b chains, one normal a chain and one abnormal a chain that has 172 amino acids as opposed to normal 141.
Occurs frequently in Orientals (SE Asia)

A

Hb H – CONSTANT SPRING DISEASE (Hb CS)

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

Heterozygous a° (- -/aa)

Homozygous a+ (-a/-a)

A

a THALASSEMIA MINOR

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

Heterozygous a+ thalassemia

Associated with one a gene deletion (-a/aa)

A

THE SILENT CARRIER

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

b THALASSEMIA MAJOR

Four genotypes

A

b°/b°
b+/b+ (Mediterranean form)
b°/b+

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

is composed of normal a chains and two abnormal a chains formed by fusion of N-terminal end of the d chain and the C-terminal of the b chain

A

Hb Lepore

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

Hb Lepore consists of 3 types:

A

Hb Lepore-Baltimore
Hb Lepore-Boston (most common)
Hb Lepore-Hollandia

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

b THALASSEMIA INTERMEDIA

Less impairment of b chain synthesis than the homozygous b+ thalassemia

A

Homozygous b+ thalassemia (b+/b+) Mild Black Form

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

b THALASSEMIA INTERMEDIA

Caused by deletion on the d and b structural genes on chromosome 11
More efficient synthesis of g chains; the g chain bind with a chains to form Hb F, which constitutes 100% of hemoglobins in patients)

A

Homozygous db thalassemia (db°/db°)

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

Describes a heterogenous group of inherited disorders characterized by increased levels of Hb F in a dults in the absence of usual clinical and hematologic features of thalassemia

Characterized either by deletion or inactivation of the b and d structural gene complexes

A

HEREDITARY PERSISTENCE OF FETAL HEMOGLOBIN

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

Test for Hb H Inclusions

A

Brilliant Cresyl Blue (BCB)

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

Test for Hb F

A

Acid Elution Slide

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

Cells containing HbF will be stained ___ whereas normal adult cells appear as “ghost cells” (only the outer membranes are visible.

A

BRIGHT PINK TO RED

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

ANEMIA OF INCREASED ERYTHROCYTE DESTRUCTION

A

THE HEMOLYTIC ANEMIAS

24
Q

Classification of Hemolytic Anemias
INTRINSIC HEMOLYTIC ANEMIAS

--Hereditary
Membrane Defects
Enzyme Defects
Hemoglobinopathies
Acquired
A

Hereditary Spherocytosis
Hereditary Elliptocytosis
Hereditary Pyropoikilocytosis

G6PD Deficiency
Pyruvate kinase Deficiency
Glutathione reductase deficiency

Hemoglobin SS, CC, SC, and S-b thalassemia

Paroxysmal Nocturnal Hemoblobinuria (PNH)

25
Q

EXTRINSIC HEMOLYTIC ANEMIAS
Hereditary
Acquired

A

LCAT deficiency
Abetalipoproteinemia

Immune mediated
Mechanical, thermal, chemical damage
Infectious agents

26
Q

is defined as one in which the life span of normal red cells would be shortened if they were transfused to a patient under investigation but the patient’s red cells would survive normally if given to a normal recipient (i.e. abnormal environment factors that damage normal red cells)

A

EXTRINSIC DEFECT

27
Q

is one in which the patient’s erythrocytes would not survive normally when transfused to a normal recipient (the erythrocytes themselves are defective)

A

INTRINSIC DEFECT

28
Q

Hemolytic anemia are often due to alterations in __ (either DEFICIENCY or DEFECTIVENESS)

A

STRUCTURAL PROTEINS

29
Q

forms dimmers linked end-to-end to form tetramers

A

Spectrin

30
Q

links together spectrin dimers

A

Actin

31
Q

Mediates the spectrin:actin linkage

an integral membrane protein

A

Protein 4.1 –

Protein 3 –

32
Q

binds the spectrin fiber

A

Ankyrin –

33
Q

Is a hemolytic disorder characterized by numerous MICROSPHERIC erythrocytes in the blood film
Autosomal dominant inheritance and therefore manifests in heterozygotes

A

HEREDITARY SPHEROCYTOSIS

34
Q

Confirmatory test for HS

A

OSMOTIC FRAGILITY TEST

35
Q

MCV is normal but MCHC is increased

A

HEREDITARY SPHEROCYTOSIS

36
Q

The _ hemolytic indicators of HEMOGLOBINEMIA, HEMOGLOBINURIA and HEMOSIDERINURIA DO NOT APPEAR IN !

A

intravascular

HEREDITARY SPHEROCYTOSIS

37
Q

Is a heterogenous group of disorders characterized by large number of elliptocytes on the blood film

A

HEREDITARY ELLIPTOCYTOSIS

38
Q

Protein membrane defects seen in HE:
Deficiency in Protein 4.1 (also called )

Deficiency in s__ interaction

Defective ankyrin-Protein 3 interactions (aka )

A

Spherocytic HE
spectrin dimer-dimer
Atypical HE

39
Q

The mechanism of hemolysis involves:

Membrane loss

Decreased red cell “deformability”

Shortened red cell survival due to splenic destruction

A

HE

40
Q

Is an extremely rare hemolytic disorder characterized by extreme anisocytosis and micropoikilocytosis

A

HEREDITARY PYROPOIKILOCYTOSIS

41
Q

Are a heterogenous group of red cell disorders caused by increased membrane lipids, particularly phosphatidylcholine

A

HEREDITARY STOMATOCYTOSIS (HYDROCYTOSIS) and HEREDITARY XEROCYTOSIS (CELL DEHYDRATION)

42
Q

Enzymopathies in EM pathway are referred collectively as

A

CONGENITAL NONSPHEROCYTIC HEMOLYTIC ANEMIAS

43
Q

Is responsible for AEROBIC GLYCOLYSIS which generates 10% of the energy of the cell

A

THE PENTOSE PHOSPHATE PATHWAY or HEXOSE MONOPHOSPHATE SHUNT

44
Q

leads to a marked reduction in ATP production, whicg is necessary for the maintenance of the membrane Na-K pump.

A

PK deficiency

45
Q

Screening test for PK Deficiency and G6PD Deficiency

A

AUTOHEMOLYSIS TEST

46
Q

– Autohemolysis is slightly to moderately increased but is partially corrected by glucose.

Seen in:
G6PD deficiency

HS

Unstable Hb Disease

A

TYPE I

47
Q

Autohemolysis is greatly increased and glucose has no effect, however, ATP corrects the hemolysis. Seen in Pyruvate kinase deficiency.

A

TYPE II

48
Q

Autohemolysis is greatly increased but can be corrected either by glucose or ATP. This pattern is also seen in triose phosphateisomerase deficiency

A

HEREDITARY SPHEROCYTOSIS

49
Q

is the MOST COMMON enzymopathy associated with hemolysis

A

G-6-PD Deficiency

50
Q

When G6PD is deficient, red cells cannot generate sufficient _ to detoxify _. Hemoglobin is oxidized to __ (Fe+3). Heme is liberated from globin. And globin denatures, forming _ . attach to membrane sulfhydryl groups, inducing cell rigidity. Lysis eventually occurs even before reaching the spleen.

A

GSH
peroxide
methemoglobin
HEINZ BODIES

51
Q

protects enzymes and hemoglobin against oxidation by reducing hydrogen peroxide and oxygen radicals.

A

REDUCED GLUTATHIONE (GSH)

52
Q

This screening test INDIRECTLY detects NADPH generation by G6PD. This test is sensitive and can detect G6PD deficiency even in the presence of hemolysis
+ g6pd

A

METHEMOGLOBIN REDUCTASE TEST

Little or no fluorescence

53
Q

This test measures the ability of normal cells to detoxify hydrogen peroxide when incubated with ascorbate.
+ g6pd

A

ASCORBATE CYANIDE TEST

brown color

54
Q

In this group of disorders, red cell survival is affected by abnormalities in lipid metabolism. 2

A

HEREDITARY PLASMA CONSTITUENT ABNORMALITIES

Abetalipoproteinemia

Lecithin-Cholesterol Acyltransferase (LCAT) deficiency

55
Q

Also known as Hereditary Acanthocytosis

A

ABETALIPOPROTEINEMIA

56
Q

ABETALIPOPROTEINEMIA

A

Malabsorption of fat
Retinitis pigmentosa
Neurologic damage
Acantho

57
Q

A rare inherited disorder characterized by mild normocytic, normochromic anemia
Found in Scandinavian families

A

LECITHIN-CHOLESTEROL ACYL TRANSFERASE (LCAT) DEFICIENCY

Hematology (5 decks)

Brainscape helps you reach your goals faster, through stronger study habits.
© 2025 Bold Learning Solutions. Terms and Conditions