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Thalassemia and Hemoglobinopathy

Total questions: 20

Worksheet time: 10mins

Name
Class
Date
1.

What is the amino acid substitution in patients with sickle cell anemia?

a)

adenine for thymine

b)

lysine for valine

c)

valine for glutamic acid

d)

glutamic acid for valine

2.

Which of the following factors contributes to the pathophysiology of sickling?

a)

increased iron concentration

b)

hypochromia

c)

fava beans

d)

dehydration

3.

Which of the following shows crystals like those seen here

a)

Hgb CC disease

b)

Hgb DD disease

c)

Hgb EE disease

d)

Hgb SC disease

4.

The crystals seen here are associated with:

a)

Hgb CC disease

b)

Hgb SS disease

c)

Hgb SC disease

d)

Hgb EE disease

5.

All but one of the following may be seen in the PB smear when a sickle cell crisis has occurred

a)

Target cells

b)

NRBCs

c)

Polychromasia

d)

Moderate elliptocytes

6.

List two sets of lab data that can distinguish IDA from beta thalassemia trait.

a)

Serum iron and RBC

b)

Hgb and Hct

c)

WBC and RDW

d)

RBC indices and Platelets

7.

What is the majority hemoglobin in beta thalassemia major

a)

Hgb A

b)

Hgb A2

c)

Hgb F

d)

Hgb H

8.

Which of the following alpha thalassemia is inconsistent with life?

a)

Alpha thal trait

b)

Alpha thal minor

c)

Hgb H

d)

Hgb Bart's

9.

Which of the following hemoglobins contains β4 tetramers?

a)

Hgb Bart's

b)

Hgb Gower

c)

Hgb H

d)

Hgb Portland

10.

Which of the following hemoglobins contains γ4 tetramers?

a)

Hgb Bart's

b)

Hgb Gower

c)

Hgb H

d)

Hgb Portland

11.

A hemoglobin electrophoresis showed 49% Hb S, 42% Hb C, Hb F 6%, and

Hb A2 - 3%. These results are consistent with a diagnosis of:

a)

sickle cell anemia

b)

sickle cell trait

c)

Hgb SC disease

d)

Hgb C trait

12.

A hemoglobin electrophoresis showed 40% Hb S, 57% Hb A, Hb F 1%, and

Hb A2 - 2%. These results are consistent with a diagnosis of:

a)

sickle cell anemia

b)

sickle cell trait

c)

Hgb SC disease

d)

Hgb C trait

13.

Cells containing large amounts of Hb S sickle when which of the following conditions occur?

a)

high oxygen tension, and acidosis

b)

hypoxia and alkalosis

c)

temperatures

<37 C and alkalosis

d)

temperatures

>37 C and hypoxia

14.

What disease does the following code for: β+ β

a)

beta thal minor

b)

beta thal intermedia

c)

beta thal major

d)

Cooley's anemia

15.

Which nationality is MOST likely to be affected by thalassemia?

a)

Chinese

b)

South American Indians

c)

Southeast Asians

d)

Europeans

16.

Which of the following lab results would be expected in a patient with alpha thalassemia?

a)

MCH = 32 pg

b)

MCV = 70 fl

c)

stomatocytes

d)

Increased Hgb A

17.

Which thalassemiapresents with an abnormal hgb that precipitates in red cells and will have a golf ball appearance after incubation with brilliant cresyl blue

a)

Hgb F

b)

Hgb H

c)

Hgb Bart's

d)

Beta thal major

18.

Why is hydrops fetalis incompatible with life?

a)

Life cannot exist without HbA

b)

Lack of embryoinc hemoglobins precludes fetal development

c)

All three normal adult hemoglobins contain alpha chains

d)

Fetal hgb is essential to sustain life after birth

19.

What disease does the following code for: αα/- -

a)

Aphal thal trait

b)

Alpha thal minor

c)

Hgb H

d)

Hgb Bart's

20.

What disease does the following code for: α-/- -

a)

Aphal thal trait

b)

Alpha thal minor

c)

Hgb H

d)

Hgb Bart's