Laparoscopic subtotal splenectomy in hereditary spherocytosis : to preserve the upper or the lower pole of the spleen?

BACKGROUND: Clinical manifestations of hereditary spherocytosis can be controlled by splenectomy. The use of this procedure has been restricted due to concerns regarding exposure of patients to a lifelong risk of overwhelming infections. Subtotal splenectomy, which removes 85-90% of the enlarged spleen, is a logical alternative. In the first cases performed by laparoscopy we have chosen to preserve the upper pole. However, this technique showed some disadvantages, especially concerning the correct intraoperative evaluation of the splenic remnant volume. Therefore, we developed a new variant of the procedure by preserving the lower pole of the spleen. METHODS: Based on the authors' experience in laparoscopy (176 laparoscopic splenectomies), 10 laparoscopic subtotal splenectomies were performed in patients with hereditary microspherocytosis, preserving either the upper or the lower splenic pole. RESULTS: Patient age ranged between 5 and 35 years. The mean volume of the remnant spleen was 41.4 cm3. There were no complications, and no transfusions were needed. Follow-up for 1-30 months was available. CONCLUSIONS: Subtotal splenectomy appears to control hemolysis while maintaining splenic function. The laparoscopic approach is safe and effective and should be considered the procedure of choice in hereditary microspherocytosis. Laparoscopic subtotal splenectomy presents an advantage over open subtotal splenectomy, resulting in decreased blood loss, shorter hospital stay, no conversions, fewer operative and postoperative complications, and excellent remission rates. On the basis of our experience, the preservation of the lower pole of the spleen seems to be a first-line option for the optimal evaluation of the residual splenic mass.

[Laparoscopic subtotal splenectomy in the treatment of hereditary spherocytosis]. / Splenectomia subtotala laparoscopica în tratamentul microsferocitozei ereditare.

Clinical manifestations of hereditary spherocytosis, the most common red blood cell membrane disorder, can be controlled by splenectomy. However, concerns regarding exposure of patients to a life long risk for overwhelming infections have restricted its use, especially în children. Subtotal splenectomy, as long as 80% to 90% of the enlarged spleen is removed, is a logical alternative. Subtotal splenectomy was effective în decreasing the hemolytic rate, while maintaining the phagocytic and immune function of the spleen. This surgical procedure should be considered în transfusion-dependent infants and children whit hereditary spherocytosis and în older patients whit erythrocyte membrane defects. Based on our experience în laparoscopy (120 laparoscopic splenectomies) and open subtotal splenectomy (5 cases) we performed 2 laparoscopic subtotal splenectomies in patients with hereditary microspherocytosis with good short term results. We have had no problem with blood loss and no transfusions were needed. The procedure can be performed safely and easily with all the traditional advantages of a minimally invasive approach. In order to evaluate the long term clinical benefit a minimal follow-up of 5 years is needed.

Epstein-Barr virus (EBV). VI. Incidence of EBV antibodies in patients with malignant lymphoproliferative diseases.

Serum samples from 52 patients with malignant lymphoproliferative diseases and 12 clinically healthy subjects were tested by indirect immunofluorescence (IF) reaction for the presence of specific EB antibodies anti-VCA and active EBV infection. The tests revealed EB antibodies anti-VCA in 32 patients with lymphoproliferative diseases and in 2 clinically healthy subjects and active EBV infection in 18/32 and in 0/2 EB anti-VCA positive patients and clinically healthy subjects, respectively.