Thalassemia

Rajesh Kumar Sharma

Lecture
Himalayan College of Nursing
 Thalassemia
It is a hereditary group of hemolytic anemia's,
is characterized by defective synthesis in
polypeptide chains of the protein component
of hemoglobin. Consequently, RBC synthesis is
also impaired.
Causes:- Homozygous or heterozygous
inharitance
 Pathophysiology
Total or partial deficiency of beta polypeptide
chain production impairs hemoglobin synthesis
and results in continual production of fetal
hemoglobin. Normally, immunoglobulin
synthesis switches at birth time. This conversion
does not happen in thalassemic infants. Their red
cells are hypochromic and microcytic.
 Sign & symptoms
Sever anemia @ 6 months of life
Bone abnormalities
Pallor
Yellowish skin
Splenomegaly or hepatomegaly
Frequent infection
Bleeding tendencies
Small body & large head
Possibly mental retardation
 Complications
Pathological fracture
Cardiac arrhythmias
Heart failure
 Diagnosis
CBC
Microcytosis
Long bones X-ray
Fetal Hb level
 Treatment
Folic acid supplement
Transfusion of Packed cells
Splenectomy & bone marrow transplantation
Nursing considerations