Why splenomegaly in thalassemia

Splenectomy should be avoided unless there is an inability to maintain iron balance with optimal chelation, or if there are clinically significant complications such as pancytopenia and marked enlargement. Often, hypersplenism develops because of a low pre-transfusion hemoglobin.

Increasing the pre-transfusion hemoglobin to between 9. If a decision to perform surgery is made, partial or full splenectomy is the option. Partial splenectomy is a complicated surgery utilized to preserve some splenic function.

It should be reserved for infants requiring splenectomy. Full splenectomy can usually be performed by laparoscopic technique. However, open procedure is necessary in cases of marked splenomegaly. If your spleen grows too big, your doctor might suggest surgery to remove it. In most cases, you can't prevent thalassemia. If you have thalassemia, or if you carry a thalassemia gene, consider talking with a genetic counselor for guidance if you want to have children.

There is a form of assisted reproductive technology diagnosis, which screens an embryo in its early stages for genetic mutations combined with in vitro fertilization.

This might help parents who have thalassemia or who are carriers of a defective hemoglobin gene have healthy babies. The procedure involves retrieving mature eggs and fertilizing them with sperm in a dish in a laboratory. The embryos are tested for the defective genes, and only those without genetic defects are implanted into the uterus. Mayo Clinic does not endorse companies or products. Advertising revenue supports our not-for-profit mission.

This content does not have an English version. This content does not have an Arabic version. Overview Thalassemia thal-uh-SEE-me-uh is an inherited blood disorder that causes your body to have less hemoglobin than normal. Request an Appointment at Mayo Clinic. Share on: Facebook Twitter. Show references Jameson JL, et al. Disorders of hemoglobin. In: Harrison's Principles of Internal Medicine.

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