Thalassemia

Background Of Thalassemia

Definition of Thalassemia
Thalassaemia is a blood disorder that is characterized by the condition of red blood cells
easily damaged or the age shorter than normal blood cells (120 days). Consequently
thalassaemia sufferers will experience symptoms of anemia including dizziness, pale face,
often limp body, hard sleep, lost appetite, and recurrent infections.
 Thalassemia is derived
Thomas Benton Cooley
from the Greek word
was an inventor of
talassa meaning sea. The
thalassaemia disease in
meaning of the sea is the
1925. Thomas Benton
Mediterranean Sea, since
Cooley encountered
it was first mentioned in
children suffering from
the area around the
anemia with enlarged
Mediterranean Sea.
History of Thalassemia spleen after one year old.
Thalassemia is found not
Furthermore, this anemia
only around the
is called splenic anemia
Mediterranean Sea, but
or erythroroblastosis or
also in southeast Asia,
mediteranean anemia or
often referred to as the
Cooley anemia according
Thalassemia belt (WHO,
to the founder
1983) before it was first
(Weatherall,1965).
discovered in 1925.
 KEMENKES 2007

Papua B... 2.20%

Ma 1.90%

Nusa Tenggara B... 2.60%

3.00%

Goron... 3.10%

Sumatra Sel... 5.40%

Jak 12.30%

13.40%

0 2 4 6 8 10 12 14 16
Prevalence Overseas Universitas
Pendidikan Indonesia (UPI) 2014
Irak Saudi Arabia Singapore Hongkong Srilangka India

27%
30%

4%

5%

27%
7%
Mechanism Of Disease Thalassemia

Thalassemia

beta
thalassemia if form of genetic
it occurs in disorders
beta globin

alpha
thalassemia if
it occurs in
alpha globin
 General Specific
Symtomps Symtomps Yellowish skin
Anoreksia
Pale
Slow Growth and
puberty
Bone problem
Weakness
The Urine will
Less Focus become dark
Spleen enlargement
 Method and Examination of Thalassemia

I. Primary hematology
• Method tests
: Automatic
• Equipment : (Hematology Analyzer) Sysmex KX-21 XP-300
• Procedure :

Collecting and Selecting

Inputting Analyzing
preparing whole blood
samples sample No. samples
mode
  Blood Smear Examination  Reticulocyte Examination
Method : Method : Supravital Staining (microscopic)
Microscopic: Microscope
Equipment Equipment : Microscope
Procedure : Procedure :
Preparation of blood smear Put blood and stain
BCB in a ratio of
1:1, homogenize
and leave for 15
minutes

Count the number of

Make a blood smear,
reticulocytes in 1000
let it dry
erythocyte cells

Staining of Preparat Check using a

• A blood smear, inundated with microscope
wright dye, then leave for 1
minute
• Then, add a buffer with a pH of
6.4. Allow 15-20 minutes
• Rinse with water and dry
• Check using a microscope
II. Type of thalassemia
 HB Elektrophoresis
Method : Capillary Electrophoresis
Equipment : Minicap Capilarrys Flex-Piercing
Procedure :

Select the
Insert the sample and
hemoglobin
Prepare a device electrophoresis
control into the
rolating sampler
program in the menu

After all hemoglobin

Close the minicap
electrophoresis
door and the analysis
sequences are
will run
complete, remove all
automatically
samples and controls
 Laboratory Result

Case Parameter Concetration

Thalassemia Hb : 7,0 Male : 14-18 g/dL
Female : 12-16 g/dL
Retyculosite : > 1,5 0,5-1,5%
Hematocrit : <21 Male : 42-52%
Female : 37-47%
Blood Smear:
• Anysocytosis and
poilkilositosis
( Fragmentosite and
tear drop cell )
• Hypchromia
microsite
• Target cell
• NRBC
Case Parameter Concetration
Thalassemia Hb Elektroforesis

Minor HbA HbA2 HbF

Major HbA2 HbF

 MANAGEMENT CARE
Minor Intermediate Mayor
• None • Blood Transfusion • Blood Transfusion
(not often Or (routine)
Routine) • Medication (routine)
• Medication (not • Bone Marrow
often or routine) Transplantation (to
stopping routine
Blood transfusion)

To prevent thalassemia is to do Thalassemia screening through blood test for a future

brides.the aim is to detect whether the prospective father and mother have thallasemia
trait or not so as to prevent the occurrence of thalassemia.
 Conclusion
Thalassemia is genetic disease that makes sufferers have impaired haemoglobin (Hb)
synthesis due to lack or fornation of Hb-forming proteins, this causes erythrocytes to
break easily, making sufferers have very low Hb levels so that sufferers are required to
get blood transfusions for life.

Thalassemia can be found throughout the world, especially countries that include
the thalassemia belt ( southeast asia, sub Saharan Africa and Mediterranean) until now
thalassemia has not been cured even bone marrow grafts can only avoid the necessity
of routine blood transfusionsbut not able to prevent offspring from not having this
disease.
The best way to prevent this disease is screening thalassemia with blood tests in
adolescents or brides to be the goal is to detect whether the prospective father and
mother have genes carrying thalassemia traits or not so as to prevent the occurrence
of thalassemia.