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問題一覧
1
The most common hemolytic anemia in people of Northern European extraction
HEREDITARY SPHEROCYTOSIS
2
What are the 3 Membrane Protein Defects that may cause HS?
■ Spectrin deficiency ■ Defective binding of spectrin to protein 4.1 ■ Actin
3
What is the confirmatory test for HS?
Osmotic fragility tests
4
What are the 3 Characteristics clinical features of HS?
○ Anemia ○ Splenomegaly ○ Jaundice
5
What is the treatment to HS?
Splenectomy
6
Heterogeneous group of disorders characterized by a large numbers of elliptical erythrocytes
HEREDITARY ELLIPTOCYTOSIS
7
What are the 3 skeletal defects in HE? And describe the last one.
○ Deficiency of protein 4.1 (spherocytic HE) ○ Abnormal skeletal protein interaction ○ Atypical HE: ■ Spectrin dimer-dimer interaction ■ Ankyrin-protein 3 interaction
8
Most common red cell enzymopathy associated with hemolysis
GLUCOSE-6-PHOSPHATE DEHYDROGENASE (G6PD)
9
It is the most common one associated with hemolysis
G6PD A
10
How many genetic variations in G6PD?
>150
11
Laboratory Findings: ○ Hb concentration are decreased ○ Anemia is normocytic normochromic ○ Bite cells are often seen (but are not diagnostic) ○ Heinz bodies: precipitation of denatured Hgb
GLUCOSE-6-PHOSPHATE DEHYDROGENASE (G6PD)
12
Most common enzyme deficiency of the E-M pathway
PYRUVATE KINASE DEFICIENCY ANEMIA
13
What is the recommended screening test for PKDA?
PK fluorescent spot test
14
Signs & symptoms: chronic hemolysis (anemia, splenomegaly, jaundice, gallstone)
PYRUVATE KINASE DEFICIENCY ANEMIA
15
Point mutation of beta-globin gene at the 6th position
Sickle Cell Anemia (Glutamic acid to Valine)
16
Sickling of red cells in the deoxygenated stated or lower oxygen tension
vaso-occlusion
17
What gene is defective in PAROXYSMAL NOCTURNAL HEMOGLOBINURIA (PNH)?
PIGA gene
18
Screening test and Confirmatory test for PAROXYSMAL NOCTURNAL HEMOGLOBINURIA (PNH)?
● Screening test: Sucrose lysis test ● Confirmatory test: Ham test
19
Optimal reaction temperature: 37 deg C Immunoglobulin type: IgG Blood group specificity: Rh, Kell Treatment: Steroids, splenectomy, immunosuppresants
Warm-Antibody
20
Optimal reaction temperature: 37 deg C Immunoglobulin type: IgG Blood group specificity: Rh, Kell Treatment: Steroids, splenectomy, immunosuppresants
Warm-Antibody
21
Optimal reaction temperature: 0-4 deg C Immunoglobulin type: IgM Blood group specificity: Ii Treatment: Avoid Cold
Cold-Antibody
22
Optimal reaction temperature: 0-4 deg C (antibody binds to cell) 37 deg C (hemolysis takes place) Immunoglobulin type:IgG (Donath-Landsteiner autoantibody) Blood group specificity: Pp Treatment: Avoid cold
Paroxysmal Cold Hemaglobinuria