thalasemia complications

1. THALASSEMIA
COMPLICATIONS
Submitted by: omama tariq (mcf1800994)

2. THALASSEMIA COMPLICATIONS
 Complications can be grouped as
 (1) transfusion-transmitted infections,
 (2) transfusional iron overload,
 (3) toxicities of iron chelation therapy, and
 (4) bacterial infections
 Cardiac complications
 Liver complications
 Endocrine complications
 Bone complications
 Other complications
 Dental Complications
 Growth retardation
 Leg ulcers

3. CARDIAC COMPLICATIONS
 Cardiac dysfunction(both systolic and diastolic dysfuctions)
 Ferritin greater than 2500 mg/L or a liver iron concentration > 15 mg Fe/g dry
weight is associated with high risk of cardiac death in thalassemia
 ferritin and liver iron may not correlate with cardiac iron load, cardiac iron can be
specifically and reproducibly measured using cardiac MRI T2*.
 Monitoring: 6 monthly cardiac physical examination , cardiac function yearly
starting at age of 10 years, if history suggestive of arrhythmias ECG, 24-hour Holter
monitoring should be done.
 Chelation therapy to reduce high iron load lowers the likelihood of developing
cardiac dysfunction

4. LIVER COMPLICATIONS
 includes- transfusion-related viral hepatitis
(Hepatitis B, C), iron overload, drug toxicity, and
biliary disease due to gallstones.
 Liver enzymes should be monitored routinely.
 Liver iron concentration should be monitored
routinely and chelation therapy initiated and
adjusted to reduce complications of iron
overload.
 In the presence of elevated liver iron, liver
fibrosis, and cirrhosis may be accelerated by
alcohol, liver toxic drugs, and untreated viral
hepatitis, Limit such exposure.

5. ENDOCRINE COMPLICATIONS
 Iron overload is responsible for dysfunction of many of endocrine glands like thyroid,
parathyroid pituitary gland, gonads and pancreas.
 These include-
 short stature (34%),
 delayed puberty, hypogonadotropic hypogonadism (35– 55%),
 hypothyroidism (10%),
 hypoparathyroidism (4%), and
 diabetes mellitus (5.6 – 20%)
 ENDOCRINE COMPLICATIONS -INTERVENTIONS
 Short stature-
 The diagnosis of growth hormone deficiency, other hormonal or nutritional deficiencies or
deferoxamine toxicity should be considered
 Growth hormone stimulation testing should be done and, if indicated, growth hormone therapy
started

6. BONE COMPLICATIONS
 Bone disorders are common and multifactorial in patients with thalassemia .
 Related to inadequate transfusion, iron-overload, over-chelation, and other endocrine
factors contribute to the development of osteopenia and osteoporosis
 Interventions:
 Adequate blood transfusions
 Adequate chelation therapy should be maintained
 No Over-chelation
 Hormone replacement therapy
 Calcitonin , bisphosphonates
 Diet rich in calcium and vitamin D

7. HYPERSPLENISM- SPLENECTOMY
 Indictions of splenectomy
 An increase in the yearly requirement of
packed cells more than 1.5 times the basal
requirement, i.e. packed cell 200 to 220 cc per
kg/ year.
 Massive splenic enlargement posing risk of
splenic rupture, or when it is associated with
left upper quadrant pain
 Presence of leukopenia or
thrombocytopenia due to hyperspleenism

8. CAUSES OF DEATH
 Congestive heart failure
 Arrhythmia
 Sepsis secondary to increased susceptability to infection post spleenectomy
 Multiorgan failure due to hemochromatosis