Splenectomy in Thalassaemia: Why, When and How
Splenectomy is indicated in the transfusion-dependent thalassaemia patient when increased blood transfusion requirement that prevents adequate control with iron chelation therapy, hypersplenism, symptomatic splenomegaly. Annual transfusion volume exceeding 225 to 250 mL/kg/year with packed red blood cells may indicate the presence of hypersplenism. If a decision to perform surgery is made, partial or full splenectomy is the option. However, a partial splenectomy is more complicated than a full splenectomy and should be considered only in infants to preserve some splenic function. Full splenectomy can be performed by open or laparoscopic technique. However, open procedure is necessary in cases of marked splenomegaly. Patients must receive adequate immunization against Streptococcus pneumoniae, Haemophilus influenzae type B, and Neisseria meningitides prior to surgery. An evaluation for gallstones should be performed prior to surgery. Major adverse effects of splenectomy are sepsis, thrombophilia, pulmonary hypertension and iron overload. Splenectomy should be avoided in children younger than five years because of a greater risk of fulminant post-splenectomy sepsis. After splenectomy, patients should receive oral penicillin prophylaxis and be instructed to seek urgent medical attention for a fever over 101º Fahrenheit. Alternative antibiotics for patients unable to take penicillin include amoxicillin, trimethoprim-sulfamethoxazole and erythromycin. Patients need to be made aware of the potential for travel-related infections such as babesiosis and malaria, as well as the risk inherent in travel to an area where medical care is not readily accessible. Postoperative thrombocytosis is common, with platelet counts often reaching 1,000,000 -2,000,000/mm3, all guidelines recommend thromboprophylaxis perioperatively in patients with thrombocytosis. Special consideration should be given to the use of low-dose aspirin (80 mg/kg/d) for patients with high platelet counts, or the use of anticoagulation for patients with a history of previous thrombosis or other risk factors. Thromboembolic complications are frequent in thalassaemia in splenectomised patients. Following splenectomy Iron will be redirected and accumulated in the liver, heart, and other organs and effective chelation protocols should be introduced. So before considering splenectomy in this situation, the patient should be placed on an adequate transfusion program for several months and then re-evaluated.
Correspondence: Professor Shahnoor Islam, Department of Paediatric Surgery, Dhaka Medical College Hospital, Dhaka-1000, Bangladesh. e-mail: firstname.lastname@example.org
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