Facebook Twitter YouTube

18393

Role of Splenectomy In Thalassemia Patients Undergoing Hematopoietic STEM CELL Transplantation - CANCELED

Saturday, October 20, 2012
Napoleon Ballroom (Hilton Riverside)
Massimo Villa, MD1, Pietro Sodani, MD2, Andrea Roveda, MD2, Francesco Rulli, MD1, Katia Paciaroni, MD2, Cecilia Alfieri, MD2, Gioia De Angelis, MD2, Cristiano Gallucci, MD2, Mario Patricolo3, Michele Grande, MD1, Chiara Giurioli, MD1, Gianfranco Tucci, MD1 and Guido Lucarelli, MD2, (1)Department of Surgery, University Hospital of Tor Vergata, Rome, Italy, (2)Mediterranean Institute of Hematology, University Hospital of Tor Vergata, Rome, Italy, (3)Department of Surgery, Division of Pediatric Surgery, Medcare Hospital and Medical Centres, Dubai, United Arab Emirates

Purpose: Beta thalassemia is a genetic disorder resulting in absent beta globin chain synthesis. Currently, the only cure is allogeneic stem cell transplantation (SCT) which corrects the defect in the hematopoietic system by using healthy stem cells, which must be immunologically acceptable.  Patients can be categorized into 3 classes of risk for bone marrow transplantation (the greatest is class 3), on the basis of hepatomegaly, portal fibrosis, and quality of chelation before transplantation. Chelation was characterized as regular when deferoxamine treatment was initiated within 18 months of the first transfusion. Massive splenomegaly is frequently found in patients in a higher class risk group. Splenectomy is indicated if there is significant abdominal discomfort, splenic infarction, or symptomatic hypersplenism, but could impact engraftment. We reviewed our experience with splenectomy prior to SCT in patients with thalassemia.

Methods: Seventeen patients underwent splenectomy from May 2005 to April 2011; all were prepared for surgery by preoperative blood transfusion to achieve a haemoglobin level of over 9.5 g/dL. All received appropriate immunizations.  The mean and median ages were 8 years, respectively (4 - 16). Mean operative time was 75 minutes (range 60 – 100). Average postoperative hospitalization time was 5.7 days (range 5-7). Minimum spleen weight was 495 grams and maximum 2397 grams.

Results: The median time (range) to reach granulocyte counts of 0.5 and 1.0 × 109/L among splenectomised patients was 18 (14-41) days. The corresponding time points for patients without splenectomy were 25 (20-28) days. Platelet counts of at least 20 × 109/L were reached at 23 (15-71) days. Among splenectomised patients, there was one death from transplantation-related causes (chronic lung graft versus host disease). Two patients had EBV reactivation with a high viral load which resolved after specific treatment. One patient developed a lung fungal infection which resolved with antifungal therapy. Two months after transplant one patient developed disseminated lung fungal infection which resolved with specific antifungal therapy.   Only one patient at day +2 after surgery showed an increased level of amylase and lipase, but he was given promptly i.v. sandostatine treatment and those above mentioned enzymes normalized in five days.

Conclusion: The predominant indications for splenectomy were recurrent acute splenic sequestration and increased transfusion demand in thalassaemics patients.Here we reported that splenectomy prior to an allogeneic SCT in thalassemia patients is associated with faster engraftment without a significantly increased risk of death from peri-transplant infections. While a larger study is warranted, it appears that pre- transplant splenectomy for thalassemia major is not associated with an averse impact of ever free survival and overall survival.