Long-term Results of Splenectomy in Transfusion-dependent Thalassemia.


Akca T., Ozdemir G. N., Aycicek A., Ozkaya G.

Journal of pediatric hematology/oncology, cilt.45, sa.3, ss.143-148, 2023 (SCI-Expanded) identifier identifier identifier

  • Yayın Türü: Makale / Tam Makale
  • Cilt numarası: 45 Sayı: 3
  • Basım Tarihi: 2023
  • Doi Numarası: 10.1097/mph.0000000000002468
  • Dergi Adı: Journal of pediatric hematology/oncology
  • Derginin Tarandığı İndeksler: Science Citation Index Expanded (SCI-EXPANDED), Scopus, CAB Abstracts, EMBASE, MEDLINE
  • Sayfa Sayıları: ss.143-148
  • Anahtar Kelimeler: splenectomy, children, thalassemia, transfusion-dependent, long-term, LAPAROSCOPIC SPLENECTOMY, MAGNETIC-RESONANCE, CHELATION-THERAPY, PREVALENCE, STRATEGIES, SEPSIS
  • Bursa Uludağ Üniversitesi Adresli: Evet

Özet

Splenectomy is indicated in transfusion-dependent thalassemia (TDT) only in certain situations. This study aimed to present the effectiveness, complications, and long-term follow-up results of splenectomy in children with TDT. We performed a 30-year single-institution analysis of cases of splenectomy for TDT between 1987 and 2017 and their follow-up until 2021. A total of 39 children (female/male: 24/15) were included. The mean age at splenectomy was 11.2 +/- 3.2 years, and their mean follow-up duration after splenectomy was 21.5 +/- 6.4 years. Response was defined according to the patient's annual transfusion requirement in the first year postsplenectomy and on the last follow-up year. Complete response was not seen in any of the cases; partial response was observed in 32.3% and no response in 67.6%. Thrombocytosis was seen in 87% of the patients. The platelet counts of 7 (17.9%) patients were >1000 (10(9)/L), and aspirin prophylaxis was given to 22 (56.4%) patients. Complications were thrombosis in 2 (5.1%) patients, infections in 11 (28.2%) patients, and pulmonary hypertension in 4 (10.2%) patients. Our study showed that after splenectomy, the need for transfusion only partially decreased in a small number of TDT patients. We think splenectomy can be delayed with appropriate chelation therapy up to higher annual transfusion requirement values.