NEW
Font size
WorksheetsHemoglobinopathies
Total questions: 23
Worksheet time: 12mins
Production of alpha globin chains is regulated by genes found in which chromosome?
11
16
7
6
True or false: In the synthesis of all globin chains, there is one copy of each globin gene per chromatid, for a total of two genes per diploid nucleus.
True
False
Which genetic mutation is the most common type of genetic mutation occurring in hemoglobinopathies
deletion
insertion
fusion
point mutation
The American physician and clinical cardiologist who first observed and describe sickle cell anemia in 1910
James Herrick
Victor Emmel
Vernon Hahn
Linus Pauling
In HbS homozygotes, the sickling process begins at oxygen saturation levels below:
60
70
85
95
True or false: In HbS, glutamic acid is replaced by lysine at the 6th position of the beta chain
True
False
Which clinical manifestation is the hallmark of sickle cell disease?
splenic sequestration
vasoocclusive crisis
acute chest syndrome
chronic hemolysis
remnants of RBC nuclei
Howell-Jolly bodies
Pappenheimer bodies
Heinz bodies
None of the choices
The presence of which RBC morphology is the hallmark of sickle cell disease
drepanocytes and acanthocytes
codocytes and drepanocytes
schistocytes and stomatocytes
spherocytes and codocytes
The most common screening test for HbS
capillary electrophoresis
tandem mass spectrometry
hemoglobin solubility test
high performance liquid chromatography
The only curative therapy for sickle cell disease
hematopoietic stem cell transplantation
blood exchange transfusion
hydroxycarbamide
L-glutamine
The most common consequence of transfusion therapy in sickle cell disease
transfusion reactions
hepatitis B infection
iron overload
none of the choices
This protein is responsible for iron overload in non-transfusion-dependent beta thalassemia
hepcidin
erythroferrone
ferritin
transferrin
Which iron-chelating agent is administered orally once daily?
deferasirox
deferoxamine
deferiprone
none of the choices
Which is the only curative therapy for thalassemia major?
hematopoietic stem cell transplantation
transfusion therapy
iron chelation
hydroxyurea
Which is the most common type of mutation in beta thalassemia?
point mutations
deletion
fusion
extension
Which is the most common type of mutation in alpha thalassemia?
point mutations
deletion
fusion
extension
Tetramers of beta globin chains
Hb Barts
Hb H
Both
Neither
Which is the predominant hemoglobin seen in a fetus with hydrops fetalis?
Portland
Hb F
Hb H
Hb Bart
True or false: In beta thalassemia, RBCs are typically microcytic and hypochromic
True
False
Diagnosis
Hb H disease
Sickle cell anemia
Hb C
Beta thalassemia
Cooley's anemia
Alpha thalassemia intermedia
Hb H disease
Beta thalassemia major
Alpha thalassemia major
Which disorder is incompatible with life?
Alpha thalassemia major
Beta thalassemia major
Hb SS
All of the choices
