Splenectomy is a relatively safe therapeutic option for children with noncancerous hematologic diseases such as thalassemia, hereditary spherocytosis, and immune thrombocytopenia who have had proper preoperative vaccination and follow-up care, according to findings published in the British Journal of Haematology. Results also suggest that although infectious and thrombotic complications are rare, they are more prevalent among children with thalassemia, therefore central venous catheter insertions should be avoided in this patient group.

A team of investigators in Israel conducted a historical perspective study to highlight short- and long-term infectious and thrombotic complications associated with splenectomy among pediatric patients with nonmalignant hematological diseases.

Using data from 103 patients (56% male) who underwent splenectomy in a tertiary hematologic clinic, the investigators found that 33 patients had transfusion-dependent thalassemia, 7 patients had non-transfusion-dependent thalassemia, 4 patients had sickle-thalassemia, 41 patients had hereditary spherocytosis, and 18 patients had immune thrombocytopenia. The mean age at time of splenectomy was 11.6 years.

Follow-up time was 1657 follow-up years; 30% of patients had more than 20 years of postsplenectomy follow-up, and less than 10% of patients had more than 30 years of follow-up (median age at analysis, 33 years).


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No typical cases of overwhelming postsplenectomy infection were reported during follow-up; 47 severe infections were reported in 20 patients, the majority of which occurred in patients with central venous catheter insertions. Of all reported infections, 65% occurred in patients with thalassemia with central venous catheter insertions, and 25% occurred in patients with thalassemia without catheter insertions.

Of the 8 thrombotic events that were reported, 7 were in patients with transfusion-dependent thalassemia who had a central venous catheter at the time of thrombosis.

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The investigators reported that the use of central venous catheters was linked to 4- and 6-fold increases in infectious and thrombotic complications, respectively, and that such insertions should be avoided in patients with thalassemia.

“We conclude that by fully considering the diagnosis-related risk-benefit issues while implementing proper preventative measures, splenectomy should be a safe therapeutic procedure in children with haematological disorders, including thalassaemia, [hereditary spherocytosis, and immune thrombocytopenia].

Reference

Yacobovich J, Barzilai-Birenboim S, Steinberg-Shemer O, Stark P, Pazgal I, Tamary H. Splenectomy in childhood for non-malignant haematologic disorders – long-term follow-up shows minimal adverse effects [published online April 28, 2020]. Br J Haematol. doi: 10.1111/bjh.16657