GLOBIN SYNTHESIS

Yves Laure Pimentel · 70問 · 2年前

通報

問題一覧

how many globin (polypeptide) chains in globin synsthesis

Four

Quantitative Globin Disorder

Thalassemia

Globin Disorders Qualitative

Hemoglobinopathy

Quantitative production of globin

thalassemia

thalassemia aka

thalassic/ great sea

alpha thalassemia aka

hb bart/ hydrops fetalis syndrome

all 4 alpha genes are deleted (--/--); no normal hemoglobins are produced

alpha thalassemia major

three alpha genes deleted (--/-a)

Hemoglobin H disease

hemoglobin H

4 betw

Hemoglobin H, an unstable hemoglobin is produced

Hemoglobin H disease

inclusion present in Hemoglobin H disease

heinz bodies

% Hb H and the rest is Hb A

30%

Hb H inclusions demonstrated with a supravital stain

brilliant cresyl blue

hemolytic anemia occurs extravascular RBC appearance

Golf ball, Raspberry appearance

2 alpha genes are deleted and are usually asymptomatic

alpha thalassemia Minor/ Trait

Up to 6 % Bart's Hb is present

Alpha thalassemia Minor/ Trait

How many percent Bart's Hb is present in Alpha thalassemia Minor/ Trait

up to 6%

1 alpha gene is deleted

silent carrier

diagnosed only by gene analysis

silent carrier

beta thalassemia major aka

Cooley's anemia

Cooley's anemia aka

Beta thalassemia major

markedly decreased rate of synthesis or absence of both beta chains results in excess alpha chain

Beta thalassemia Major

no hemoglobin A can be produced

Beta thalassemia Major

Beta thalassemia Major ?% Hb F

90%

Hb A1

2 alpha and 2 beta

Hb A2

2 alpha and 2 delta

decrease rate of synthesis of one of the beta chain

beta thalassemia minor/ trait

Hb A is slightly decreased, Hb A2 is slightly increased to compensate

Beta thalassemia Minor/ Trait

Hemoglobinopathies (MCHC)

normocytic, normochromic

structurally abnormal globin chain synthesis due to amino acid substitution

Hemoglobinopathies

both globin chain affected

Homozygous/ Disease conditions

Homozygous/ Disease conditions ex.

Hb SS

Heterozygous/ trait conditions ex.

Hb AS

only one globin chain affected

Heterozygous/ trait conditions

caused when valine replaces glutamic acid at position 6 on both beta chains

Sickle cell disease (Hb SS)

mos common hemoglobinopathy and most severe

Sickle cell disease (Hb SS)

Sickle cell disease (Hb S %)

80%

Sickle cell disease (Hb F %)

20%

caused when Valine replaces glutamic acid at position 6 on one beta chain

Sickle cell trait (Hb AS)

Sickle cell trait (Hb AS) % Hb A

Sickle cell trait (Hb AS) % Hb S are produced

40%

Hemoglobin C disease aka

Hb CC, Bar of gold, clam shell, rod shape

Hemoglobin C disease inclusion

Target cell

caused when lysine replaces glutamic acid at position 6 on both beta chains

Hemoglobin C disease

hexagonal with blunt ends and stain darkly

Hb CC crystals

Hb CC, Bar of gold, clam shell, rod shape

Hb C disease

caused when lysine replaces glutamic acid at position 6 on one beta chains

Hemoglobin C trait

Hemoglobin C trait % Hb A, and % Hb X

60%, 40%

hb severity

Hb SS> Hb SC> Hb CC

S gene from one parent + C gene from one parent

Hemoglobin SC disease

symptoms are less severe than sickle anemia but more severe than Hb C disease

Hemoglobin SC disease

Hemoglobin SC disease % Hb S, and % Hb C

50%, 50%

crystalline fingerlike projection is often straight with parallel sides and blunt, pointed, protruding end

Hb Sc Crystals

Hemoglobin SC disease aka

Washington monument/ pistol shape

Washington monument/ pistol shape aka

Hemoglobin SC disease

caused when lysine replaces glutamic acid position 26 on the beta chain

Hemoglobin E

it migrates with Hb A2 C and O alkaline hemoglobin electrophoresis

Hemoglobin E

caused when glycine replaces glutamic acid at position 121 on beta chain

hemoglobin D (Punjab)

what hb in which both homozygous and heterozygous conditions are asymptomatic

hemoglobin D (Punjab)

Hb D migrates with hb ? and hb? on alkaline hemo electrophoresis

S and G

Hb D migrates with hb S and hb G on

alkaline hb electrophoresis

primary screening procedure used to detect variant hb

Cellulose Acetate Electrophoresis (alkaline ph)

hb is a negative charged molecule

Cellulose Acetate Electrophoresis (alkaline ph)

Cellulose Acetate Electrophoresis (alkaline ph) sample

red cell hemolysate

Cellulose Acetate Electrophoresis (alkaline ph) HB S positive should be confirm with?

Sickle Cell test

confirmatory for HB

h l electrophoresis

for differentiation of FA hb

Neutral Ph electrophoresis

used ro confirm variant Hb and further differentiates

citrate agar electrophoresis

method of choice when examining newborns for Hb S and C

citrate agar electrophoresis

citrate agar electrophoresis Hb S and C move towards

ANODE

citrate agar electrophoresis Hb Aand F move towards

CATHODE

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