Focal splenic lesions in myeloproliferative disease: association with fatal outcome.

Abstract

A nodular tumor of the spleen in patients with myeloproliferative disease (MPD) is a very rare form of splenic involvement. The aim of the study was to describe the clinical data, sonographic patterns, and prognosis of nodular splenic infiltration in patients with MPD. During a 20-year period, nodular splenic lesions were found in 10 out of 183 patients with MPD. Retrospectively, splenic size, echomorphology of the lesions, clinical data, sonographic follow-up, and survival were analyzed. In 9 out of 10 patients the lesions were hyperechoic--in one patient hypoechoic. In 3 patients the lesions were solitary. Seven patients had multiple nodular lesions. Low platelet count was seen in 8 patients; blast crisis was seen in 7 patients. The mean survival time was 2.9 months after detection of the splenic lesions. In one patient, autopsy confirmed the diagnosis of myelosarcoma of the spleen. The appearance of nodular splenic lesions in MPD is associated with blast crisis and a short survival. Definite histologic or cytologic findings associated with splenic nodules in MPD have not been identified yet. Myelosarcoma of the spleen is the most probable diagnosis suggested.