Case report: mantle cell lymphoma, prolymphocytoid variant, with leukostasis syndrome


A 76-year-old man presented with leukostasis syndrome, including oculodynia, blurred vision, and visual field defects, due to mantle cell lymphoma, prolymphocytoid variant, with marked leukocytosis, 1227 × 109/l. He had splenomegaly but no lymphadenopathy or hepatomegaly. The tumor cells were CD5+, CD19+, CD20+, FMC-7+, and kappa light chain restricted. Immunohistochemistry showed expression of p53 and of cyclin D1. Fluorescent in situ hybridization demonstrated t(11;14) with translocation between CYCLIN D1 and the immunoglobulin heavy-chain genes. The patient received leukapheresis and aggressive chemotherapy, but the leukocyte count remained above 100 × 109/l. The patient's condition rapidly deteriorated with lymphomatous infiltration of his lungs and soft tissues, and he expired 6 months after diagnosis. While it is known that mantle cell lymphoma may have a leukemic phase, the degree of leukocytosis in this case exceeds that previously reported in the literature and resulted in a clinical syndrome of leukostasis.

DOI: 10.1038/modpathol.3800139

Cite this paper

@article{Smith2004CaseRM, title={Case report: mantle cell lymphoma, prolymphocytoid variant, with leukostasis syndrome}, author={Marc D Smith and Timothy Singleton and Savitha Balaraman and Ishmael A Jaiyesimi and Barbara O'Malley and Abdul M Al-Saadi and Joan C Mattson}, journal={Modern Pathology}, year={2004}, volume={17}, pages={879-883} }