The records of 26 patients with systemic mast cell disease (SMCD) treated during the past decade at the National Institutes of Health were reviewed to determine the role of splenectomy in the management of SMCD. Seventeen (65%) patients had indolent SMCD, manifested primarily by urticaria pigmentosa and mast cell infiltration of the skin, bone marrow, or gastrointestinal tract. None of these patients underwent splenectomy. These patients required only symptomatic therapy. Nine (35%) patients, including those with associated hematologic disorders and those with a lymphoma-like illness termed lymphadenopathic mastocytosis with eosinophilia, had aggressive SMCD. Five of nine patients with aggressive SMCD underwent splenectomy. Of the five patients with splenectomy, three were alive at the time of this report, whereas none of the four who did not have a splenectomy was still alive. Length of survival without splenectomy was 26 months. With splenectomy, length of survival at the time of this report was 34 months. Patients without splenectomy died of bleeding caused by severe thrombocytopenia. Patients with splenectomy appeared better able to tolerate chemotherapy. We thus conclude that while splenectomy is of no value in the management of indolent mastocytosis, it should be considered in patients with aggressive SMCD.
View details for Web of Science ID A1990CH21600014
View details for PubMedID 2404352