Idiopathic myelofibrosis is a chronic myeloproliferative disorder that may evolve to myeloid hepatosplenomegaly, which is also called myeloid metaplasia, involving several organs. Occasionally the spleen reaches giant proportions and must be withdrawn. However, this procedure is followed by greater morbidity and mortality. Partial splenectomies, preserving the splenic pedicle, have been proposed in order to reduce postoperative complications. After an early postoperative improvement, some patients develop with symptomatic recurrence of their disease. The present communication shows two alternatives for the treatment of complicated myeloid splenomegaly: partial splenectomy, preserving the upper splenic pole supplied only by the splenogastric vessels and total splenectomy followed by autotransplants of splenic tissue. We performed the subtotal splenectomy in five patients. Their ten-year postoperative follow-ups and improvement in health suggest that this operation should be considered for the treatment of huge spleens due to myelofibrosis with myeloid splenomegaly. Another patient was submitted to total splenectomy and autologous splenic transplantation on the greater omentum. The three-year follow-up was uneventful related to the splenic remnants. However, this last patient required intense hematological control of his severe disease. In conclusion, whether an operation is indicated to complement the hematological therapy of myeloid splenomegaly, a splenic conservative procedure must be performed. Subtotal splenectomy or total splenectomy and autotransplants of splenic tissue are worth considering in such cases.
Myeloid splenomegaly; myeloid metaplasia; idiopathic myelofibrosis; spleen; subtotal splenectomy; splenic transplantation
