Splenomegaly

If anybody can hellp and provide me the information:

My doctor in India is behind us to remove spleen from the body. My son is 15 yrs old and has spleen of about 7 cms as checked by doctor. He is receiving 2 units of PRBC blood transfusion every 18 - 19 days to maintain HB of about 9.5 10 pre-transufion.  His weight is about 51 kgs. His yearly requirement of blood is 225 ml per kg. Doctor is advicing us that if yearly blood requiremnt is more than 200 ml per kg so spleen should be removee. He does not have any problem.

Let me know if any body has information about it. As far as i am concern i am not going to allow doctor to remove spleen till some problem is there or there is a heavy requirment of blood.

Andy: if you have time and information please reply, Thanks.

These are the current guidelines on Splenectomy for thal majors, from the Standards of Care Guidelines for thalassemia.

4.5 Splenectomy
The use of splenectomy in thalassemia has declined in recent years.
This is partly due to a decreased prevalence of hypersplenism
in adequately transfused patients. There is also an increased
appreciation of the adverse effects of splenectomy on blood
coagulation. In general, splenectomy should be avoided unless
absolutely indicated.
Splenectomy is indicated in the transfusion-dependent patient
when hypersplenism increases blood transfusion requirement
and prevents adequate control of body iron with chelation
therapy. An enlarged spleen—without an associated increase
in transfusion requirement—is not necessarily an indication
for surgery. Patients with hypersplenism may have moderate
to enormous splenomegaly, and some degree of neutropenia or
thrombocytopenia may be present.
Annual transfusion volume exceeding 225 to 250 mL/kg per year
with packed red blood cells (hematocrit 75 percent) may indicate
the presence of hypersplenism. The volume calculation should
be corrected if the average hematocrit is less than 75 percent.
The possible development of alloantibody should also be ruled
out. 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 9.5 and 10 may reverse hypersplenism.
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.
The indications for splenectomy in hemoglobin H–Constant
Spring patients are different than in beta-thalassemia disorders

His spleen is not severely enlarged. If his iron is under control, there is little justification for splenectomy at this time.

Thanks a lot Andy. I understood, and from your brief it is clear that my son does not require spleen to be removed. Doctors in india do not have much knowledge in thalassemia. And they are also behind making money.