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Anemia 3 Flashcards

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1
Q

ANEMIAS OF ABNORMAL GLOBIN DEVELOPMENT

A

THE THALASSEMIAS

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2
Q

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

A

THE THALASSEMIAS

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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
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4
Q

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

A

11

16

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5
Q

differentiates thalassemia from Hereditary Persistence of Fetal Hemoglobin (HPFH)

A

Acid elution testfor Hb F:

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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

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7
Q

Most important test to differentiate Iron deficiency from thalassemia

A

Ferritin:

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8
Q

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

A

a thalassemia-1 or 1° thalassemia

a thalassemia-2

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9
Q

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

A

BART’S HYDROPS FETALIS

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10
Q

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

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

A

HbH DISEASE

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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)

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12
Q

Heterozygous a° (- -/aa)

Homozygous a+ (-a/-a)

A

a THALASSEMIA MINOR

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13
Q

Heterozygous a+ thalassemia

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

A

THE SILENT CARRIER

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14
Q

b THALASSEMIA MAJOR

Four genotypes

A

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

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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

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16
Q

Hb Lepore consists of 3 types:

A

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

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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

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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°)

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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

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20
Q

Test for Hb H Inclusions

A

Brilliant Cresyl Blue (BCB)

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21
Q

Test for Hb F

A

Acid Elution Slide

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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

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23
Q

ANEMIA OF INCREASED ERYTHROCYTE DESTRUCTION

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A

THE HEMOLYTIC ANEMIAS

24
Q

Classification of Hemolytic Anemias
INTRINSIC HEMOLYTIC ANEMIAS

--Hereditary
Membrane Defects
Enzyme Defects
Hemoglobinopathies
Acquired
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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
EXTRINSIC HEMOLYTIC ANEMIAS Hereditary Acquired
LCAT deficiency Abetalipoproteinemia Immune mediated Mechanical, thermal, chemical damage Infectious agents
26
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)
EXTRINSIC DEFECT
27
is one in which the patient’s erythrocytes would not survive normally when transfused to a normal recipient (the erythrocytes themselves are defective)
INTRINSIC DEFECT
28
Hemolytic anemia are often due to alterations in __ (either DEFICIENCY or DEFECTIVENESS)
STRUCTURAL PROTEINS
29
forms dimmers linked end-to-end to form tetramers
Spectrin
30
links together spectrin dimers
Actin
31
Mediates the spectrin:actin linkage an integral membrane protein
Protein 4.1 – Protein 3 –
32
binds the spectrin fiber
Ankyrin –
33
Is a hemolytic disorder characterized by numerous MICROSPHERIC erythrocytes in the blood film Autosomal dominant inheritance and therefore manifests in heterozygotes
HEREDITARY SPHEROCYTOSIS
34
Confirmatory test for HS
OSMOTIC FRAGILITY TEST
35
MCV is normal but MCHC is increased
HEREDITARY SPHEROCYTOSIS
36
The _ hemolytic indicators of HEMOGLOBINEMIA, HEMOGLOBINURIA and HEMOSIDERINURIA DO NOT APPEAR IN !
intravascular | HEREDITARY SPHEROCYTOSIS
37
Is a heterogenous group of disorders characterized by large number of elliptocytes on the blood film
HEREDITARY ELLIPTOCYTOSIS
38
Protein membrane defects seen in HE: Deficiency in Protein 4.1 (also called ) Deficiency in s__ interaction Defective ankyrin-Protein 3 interactions (aka )
Spherocytic HE spectrin dimer-dimer Atypical HE
39
The mechanism of hemolysis involves: Membrane loss Decreased red cell “deformability” Shortened red cell survival due to splenic destruction
HE
40
Is an extremely rare hemolytic disorder characterized by extreme anisocytosis and micropoikilocytosis
HEREDITARY PYROPOIKILOCYTOSIS
41
Are a heterogenous group of red cell disorders caused by increased membrane lipids, particularly phosphatidylcholine
HEREDITARY STOMATOCYTOSIS (HYDROCYTOSIS) and HEREDITARY XEROCYTOSIS (CELL DEHYDRATION)
42
Enzymopathies in EM pathway are referred collectively as
CONGENITAL NONSPHEROCYTIC HEMOLYTIC ANEMIAS
43
Is responsible for AEROBIC GLYCOLYSIS which generates 10% of the energy of the cell
THE PENTOSE PHOSPHATE PATHWAY or HEXOSE MONOPHOSPHATE SHUNT
44
leads to a marked reduction in ATP production, whicg is necessary for the maintenance of the membrane Na-K pump.
PK deficiency
45
Screening test for PK Deficiency and G6PD Deficiency
AUTOHEMOLYSIS TEST
46
– Autohemolysis is slightly to moderately increased but is partially corrected by glucose. Seen in: G6PD deficiency HS Unstable Hb Disease
TYPE I
47
Autohemolysis is greatly increased and glucose has no effect, however, ATP corrects the hemolysis. Seen in Pyruvate kinase deficiency.
TYPE II
48
Autohemolysis is greatly increased but can be corrected either by glucose or ATP. This pattern is also seen in triose phosphateisomerase deficiency
HEREDITARY SPHEROCYTOSIS
49
is the MOST COMMON enzymopathy associated with hemolysis
G-6-PD Deficiency
50
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.
GSH peroxide methemoglobin HEINZ BODIES
51
protects enzymes and hemoglobin against oxidation by reducing hydrogen peroxide and oxygen radicals.
REDUCED GLUTATHIONE (GSH)
52
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
METHEMOGLOBIN REDUCTASE TEST Little or no fluorescence
53
This test measures the ability of normal cells to detoxify hydrogen peroxide when incubated with ascorbate. + g6pd
ASCORBATE CYANIDE TEST | brown color
54
In this group of disorders, red cell survival is affected by abnormalities in lipid metabolism. 2
HEREDITARY PLASMA CONSTITUENT ABNORMALITIES Abetalipoproteinemia Lecithin-Cholesterol Acyltransferase (LCAT) deficiency
55
Also known as Hereditary Acanthocytosis
ABETALIPOPROTEINEMIA
56
ABETALIPOPROTEINEMIA
Malabsorption of fat Retinitis pigmentosa Neurologic damage Acantho
57
A rare inherited disorder characterized by mild normocytic, normochromic anemia Found in Scandinavian families
LECITHIN-CHOLESTEROL ACYL TRANSFERASE (LCAT) DEFICIENCY

Hematology (5 decks)

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